|
1
|
Colak MA, Joshi S, Freeman AJ, Gariepy CE, Rasmussen SK, Nathan JD. Incidence, Management, and Survival of Pancreatic Malignancies in Children: A Population-Based SEER Study. J Pediatr Surg 2025; 60:162197. [PMID: 39919339 DOI: 10.1016/j.jpedsurg.2025.162197] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/04/2024] [Revised: 01/06/2025] [Accepted: 01/22/2025] [Indexed: 02/09/2025]
Abstract
BACKGROUND Pancreatic neoplasms are uncommon in children. We assessed the incidence, treatment, and survival of children with pancreatic malignancies, and determined factors associated with mortality using a large database. METHODS The Surveillance, Epidemiology, and End Results 22 database (excluding IL and MA) was queried to identify pediatric patients diagnosed with pancreatic malignancies between 2000 and 2020. Incidence, demographics, treatment modalities, and survival data were compared between tumor groups. RESULTS Pancreatic malignancies were identified in 300 patients (69 % female). Patients were classified by histology into solid pseudopapillary carcinoma (SPC; n = 144, 48 %), neuroendocrine (NET; n = 77, 25.7 %), epithelial (EP; n = 27, 9 %), pancreatoblastoma (PB; n = 30, 10 %), and non-epithelial/unknown (NE/U; n = 22, 7.3 %). Overall median age at diagnosis was 14, whereas median age for pancreatoblastoma was 4 (p < 0.001). Majority of SPCs (86.8 %) were diagnosed in females (p < 0.001). At diagnosis, 95 (56.2 %), 40 (23.7 %), and 34 (20.1 %) patients had localized, regional, and metastatic disease, respectively. Overall, 225 (86.2 %) patients underwent operation. Five-year overall survival rates at 5-years for each tumor group were significantly different (p < 0.001) at 100 %, 84.2 %, 69 %, 67.2 %, and 59 % for SPC, NET, NE/U, PB, and EP types, respectively. Age-adjusted incidence of pancreatic malignancies was 0.0424 per 100,000. Annual percent change in incidence from 2000 to 2020 was 9.9 [CI: (6.8,13.1), p < 0.001). Regional and distant disease, not undergoing operation, and certain tumor types were associated with increased mortality. CONCLUSION Pancreatic malignancy incidence increased over the last 20 years. Tumor type (pancreatoblastoma, neuroendocrine, and epithelial), advanced disease stage, and not undergoing surgery were independently associated with increased mortality. TYPE OF STUDY Retrospective cohort study. LEVEL OF EVIDENCE III.
Collapse
Affiliation(s)
- Muhammed Ali Colak
- Department of Abdominal Transplant and Hepatopancreatobiliary Surgery, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH, 43205, USA
| | - Shivam Joshi
- Center for Biostatistics, The Ohio State University Wexner Medical Center, 410 W 10th Ave, Columbus, OH, 43210, USA
| | - A Jay Freeman
- Division of Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH, 43205, USA; Department of Pediatrics, The Ohio State University College of Medicine, 1645 Neil Ave, Columbus, OH, 43210, USA
| | - Cheryl E Gariepy
- Division of Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH, 43205, USA; Department of Pediatrics, The Ohio State University College of Medicine, 1645 Neil Ave, Columbus, OH, 43210, USA
| | - Sara K Rasmussen
- Department of Abdominal Transplant and Hepatopancreatobiliary Surgery, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH, 43205, USA; Department of Surgery, The Ohio State University College of Medicine, 1645 Neil Ave, Columbus, OH, 43210, USA
| | - Jaimie D Nathan
- Department of Abdominal Transplant and Hepatopancreatobiliary Surgery, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH, 43205, USA; Department of Surgery, The Ohio State University College of Medicine, 1645 Neil Ave, Columbus, OH, 43210, USA.
| |
Collapse
|
|
2
|
Mansfield SA, De Corti F, Aldrink JH, Pire A, Crocoli A, Dall'Igna P, Matthyssens L, Virgone C. Rare tumors II: Adrenocortical tumors, neuroendocrine tumors of the appendix, pheochromocytoma and paraganglioma, pancreatoblastoma and solid pseudopapillary tumors of the pancreas. Pediatr Blood Cancer 2025; 72 Suppl 2:e31207. [PMID: 39034593 DOI: 10.1002/pbc.31207] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/25/2024] [Revised: 06/28/2024] [Accepted: 07/02/2024] [Indexed: 07/23/2024]
Abstract
Very rare tumors are usually divided into two groups: One includes tumors that are rare among both children and adults; the other one encompasses tumors that frequently occur in adults but are rarely observed in children. In this review, we focus on adrenocortical tumors, neuroendocrine tumors of the appendix, pheochromocytoma and paraganglioma, pancreatoblastoma and solid pseudopapillary tumors of the pancreas, with special attention to the role of surgery as main curative intervention or as part of the multimodal treatment.
Collapse
Affiliation(s)
- Sara A Mansfield
- Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio, USA
| | | | - Jennifer H Aldrink
- Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio, USA
| | - Aurore Pire
- Department of Pediatric Surgery, AP-HP Necker Enfants Malades Hospital, Paris, France
| | - Alessandro Crocoli
- Department of Surgery, General and Thoracic Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
| | - Patrizia Dall'Igna
- Pediatric Surgery, Department of Precision and Regenerative Medicine and Jonic Area, Pediatric Hospital Giovanni XXIII, University of Bari, Bari, Italy
| | - Lucas Matthyssens
- Department of Gastrointestinal and Paediatric Surgery, Princess Elisabeth Children's Hospital, Ghent University Hospital, Ghent University, Ghent, Belgium
| | - Calogero Virgone
- Pediatric Surgery, University Hospital of Padova, Padua, Italy
- Department of Women's and Children's Health, University of Padova, Padua, Italy
| |
Collapse
|
|
3
|
Lampridis S. Unraveling the landscape of pediatric pancreatic tumors: Insights from Japan. World J Gastrointest Oncol 2025; 17:101477. [PMID: 39817131 PMCID: PMC11664617 DOI: 10.4251/wjgo.v17.i1.101477] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/15/2024] [Revised: 10/03/2024] [Accepted: 10/18/2024] [Indexed: 12/12/2024] Open
Abstract
Pediatric pancreatic tumors, though rare, pose significant diagnostic and management challenges. The recent, 22-year nationwide survey on pediatric pancreatic tumors in Japan by Makita et al offers valuable insights into this uncommon entity, revealing striking geographical variations and questioning current treatment paradigms. This editorial commentary analyzes the study's key findings, including the predominance of solid pseudopapillary neoplasms and their younger age of onset, which contrast sharply with Western data. It explores the implications for clinical practice and research, emphasizing the need for population-specific approaches to diagnosis and treatment. The revealed limited institutional experience and surgical management patterns prompt a reevaluation of optimal care delivery for these complex cases, suggesting benefits of centralizing healthcare services. Furthermore, the commentary advocates for international collaborative studies to elucidate the genetic, environmental, and lifestyle factors influencing the development and progression of pediatric pancreatic tumors across diverse populations. It also outlines future directions, calling for advancements in precision medicine and innovative care delivery models to improve global patient outcomes. Unraveling Makita et al's findings within the broader landscape of pediatric oncology can stimulate further research and clinical advancements in managing pancreatic and other rare tumors in children.
Collapse
Affiliation(s)
- Savvas Lampridis
- Faculty of Medicine, Imperial College London, London SW7 2AZ, United Kingdom
- Department of Surgical Oncology, 424 General Military Training Hospital, Thessaloniki 56429, Greece
| |
Collapse
|
|
4
|
Makita S, Uchida H, Kano M, Kawakubo N, Miyake H, Yoneda A, Tajiri T, Fukumoto K. Nationwide questionnaire survey on pediatric pancreatic tumors in Japan. World J Gastrointest Oncol 2024; 16:4166-4176. [DOI: 10.4251/wjgo.v16.i10.4166] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/27/2024] [Revised: 08/23/2024] [Accepted: 09/06/2024] [Indexed: 09/26/2024] Open
Abstract
BACKGROUND Pediatric pancreatic tumors are rare and account for < 0.1% of all childhood cancers. The primary treatment for pancreatic tumors is surgical resection. However, because of the lack of knowledge regarding pediatric pancreatic tumors, no comprehensive treatment plans for pediatric pancreatic tumors have been developed.
AIM To compared the clinical features, treatment methods, and prognosis of pediatric pancreatic tumors in Japan with those in other countries.
METHODS Questionnaires were sent to 213 pediatric surgical units in Japan. Pancreatic tumors that were not surgically treated were excluded from the survey. The primary survey investigated the number of patients aged 0-18 years who underwent pancreatic tumor surgery and the type of tumors managed during the 22-year study period (from January 1, 2000 to December 31, 2021) by post card. The secondary survey assessed the clinical images, treatment methods, and tumor outcomes via email.
RESULTS The primary survey enrolled 228 patients. In the secondary survey, 213 patients were eventually enrolled. The most common type of pancreatic tumor was solid pseudopapillary neoplasm (SPN) [n = 164 (77.0%)], followed by pancreatoblastoma [n = 16 (7.5%)], pancreatic endocrine tumor [n = 14 (6.6%)], non-epithelial tumor [n = 9 (4.2%)], pancreatic tumor [n = 7 (3.3%)], and metastatic pancreatic tumor [n = 3 (1.4%)]. Overall, 123 (57.7%) patients underwent distal pancreatectomy, of whom 49 underwent laparoscopic surgery. Forty-four (20.7%) patients underwent enucleation, of whom eight underwent laparoscopic surgery. Thirty-two (15.0%) patients underwent pancreaticoduodenectomy, of whom one underwent laparoscopic surgery. All patients with SPN, including those with distant metastases and recurrent disease, survived.
CONCLUSION SPN was more common in Japan than in other countries. Regardless of the histological type, resection is the most effective treatment for pediatric pancreatic tumors.
Collapse
Affiliation(s)
- Satoshi Makita
- Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan
| | - Hiroo Uchida
- Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan
| | - Motohiro Kano
- Department of Pediatric Surgery, National Center for Child Health and Development, Tokyo 157-8535, Japan
| | - Naonori Kawakubo
- Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, Fukuoka 819-0395, Japan
| | - Hiromu Miyake
- Department of Pediatric Surgery, Shizuoka Children’s Hospital, Shizuoka 420-8660, Japan
| | - Akihiro Yoneda
- Department of Pediatric Surgery, National Center for Child Health and Development, Tokyo 157-8535, Japan
| | - Tatsuro Tajiri
- Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, Fukuoka 819-0395, Japan
| | - Koji Fukumoto
- Department of Pediatric Surgery, Shizuoka Children’s Hospital, Shizuoka 420-8660, Japan
| |
Collapse
|
|
5
|
Li T, Cheng Y, Han Y, Yang L, Wei Q, Yan X, Shi Y, Zhang X, Yang M, Liu Y. Paediatric pancreatic acinar cell carcinoma with a novel SEC31A-BRAF fusion gene. Virchows Arch 2024; 485:743-747. [PMID: 38822175 DOI: 10.1007/s00428-024-03830-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/31/2024] [Revised: 05/02/2024] [Accepted: 05/19/2024] [Indexed: 06/02/2024]
Abstract
Paediatric pancreatic acinar cell carcinoma (PACC) presents a diagnostic challenge, often confused with pancreatoblastoma (PB) due to its rarity. It is crucial to differentiate between PB and PACC, given their distinct therapeutic strategies and prognoses. Histologically, the absence of squamoid nests and scarcity of tumor mesenchyme support PACC. Conversely, the identification of a BRAF alteration leans towards PACC. Here, we present the case of an 8-year-old girl with a well-defined mass in the pancreas. The tumor exhibited a SEC31A-BRAF fusion gene and amplification of 18p, showcasing unequivocal acinar differentiation and a minor degree of neuroendocrine differentiation. Additionally, the tumor displayed scant fibrous stroma, and an absence of squamoid nests, further supporting PACC. Notably, this is the first reported instance of a solid tumor featuring a SEC31A-BRAF gene fusion. The discovery of this novel fusion gene expands our understanding of BRAF fusion partner profiles, particularly in the context of paediatric PACC.
Collapse
Affiliation(s)
- Tingting Li
- Department of Pathology, The Affiliated Children's Hospital of Xi'an Jiaotong University, Xi'an, 710003, Shaanxi, China
| | - Yin Cheng
- Departments of Pathology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China
| | - Yixuan Han
- Second School of Clinical Medicine, Xi'an Medical University, Xi'an, 710003, Shaanxi, China
| | - Li Yang
- Department of Pathology, Xijing Hospital, The Fourth Military Medical University, Xi'an, 710032, Shaanxi, China
| | - Qiang Wei
- Department of General Surgery, The Affiliated Children's Hospital of Xi'an Jiaotong University, Xi'an, 710003, Shaanxi, China
| | - Xiaosong Yan
- Department of Pathology, The Affiliated Children's Hospital of Xi'an Jiaotong University, Xi'an, 710003, Shaanxi, China
| | - Yue Shi
- Department of Pathology, The Affiliated Children's Hospital of Xi'an Jiaotong University, Xi'an, 710003, Shaanxi, China
| | - Xin Zhang
- Department of Pathology, The Affiliated Children's Hospital of Xi'an Jiaotong University, Xi'an, 710003, Shaanxi, China
| | - Meng Yang
- Department of Medical Imaging, The Affiliated Children's Hospital of Xi'an Jiaotong University, Xi'an, 710003, Shaanxi, China
| | - Yanfei Liu
- Department of Pathology, The Affiliated Children's Hospital of Xi'an Jiaotong University, Xi'an, 710003, Shaanxi, China.
| |
Collapse
|
|
6
|
Ho IG, Ihn K, Le SM, Shin S, Han SJ. Surgical management of pancreatic neoplasms in children: a single-institution experience over 15 years. Front Pediatr 2024; 12:1468276. [PMID: 39359740 PMCID: PMC11445754 DOI: 10.3389/fped.2024.1468276] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/21/2024] [Accepted: 09/05/2024] [Indexed: 10/04/2024] Open
Abstract
Introduction Pancreatic neoplasms are rare among children and very few studies have reported on surgical outcomes for pediatric pancreatic neoplasms. Therefore, we aimed to describe patient and tumor characteristics and report on the surgical outcomes of pediatric pancreatic neoplasm. Methods In this retrospective single-center study, we reviewed and analyzed the data of patients who underwent surgery for pediatric pancreatic neoplasms at Severance Children's Hospital between January 2007 and December 2022. Clinical data including demographics, surgical procedures, and postoperative and long-term outcomes were evaluated. Results A total of 28 patients underwent surgical treatment for pancreatic neoplasms with a median age of 11.7 years (range: 0.4-17.8). The most common histological diagnosis among benign tumors was solid pseudopapillary neoplasm (SPN), which occurred in 20 patients (71.4%). This was followed by a mucinous cyst, nesidioblastosis, pseudocyst, duplication cyst, and benign cyst, each occurring in one patient (3.5%). Regarding malignant tumors, pancreatoblastoma, solid pseudopapillary carcinoma, and malignant pheochromocytoma were noted in one patient each (3.5%). Tumor locations included the head in 4 patients (14.2%), the body in 7 (25%), and the tail in 16 (57.1%), and was diffuse in 1 (3.5%). The most common surgical resection range was distal pancreatectomy, found in 22 patients (78.5%), followed by pylorus-preserving pancreaticoduodenectomy, found in 2 (7.2%); duodenum-preserving pancreatic resection, central pancreatectomy, tumor enucleation, and near-total pancreatectomy were performed in one patient each (3.5%). Overall, 4 patients developed grade B or C postoperative pancreatic fistulas, and 1 experienced postoperative mortality due to uncontrollable bleeding. The mean follow-up period was 6.1 years (range: 1-15.6 years), during which no significant impact on growth after surgery was detected. Among the 20 patients with SPN, tumor rupture occurred in 4 (20%), among whom 2 experienced tumor recurrences. Conclusions Histological diagnosis of benign tumors was predominant in this case series and various extents of surgical resection were performed. Surgical treatment for pediatric pancreatic neoplasms appears to be safe and effective. However, considering the long-term prognosis of these patients, it is essential to determine the appropriate extent of surgical resection based on the location of the tumor.
Collapse
Affiliation(s)
- In Geol Ho
- Division of Pediatric Surgery, Severance Children's Hospital, Department of Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Kyong Ihn
- Division of Pediatric Surgery, Severance Children's Hospital, Department of Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Sung Min Le
- Division of Pediatric Surgery, Severance Children's Hospital, Department of Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Soyong Shin
- Division of Pediatric Surgery, Severance Children's Hospital, Department of Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Seok Joo Han
- Division of Pediatric Surgery, Severance Children's Hospital, Department of Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea
| |
Collapse
|
|
7
|
Fuchs J, Loos M, Kinny-Köster B, Hackert T, Schneider M, Mehrabi A, Berchtold C, Al-Saeedi M, Müller BP, Strobel O, Feißt M, Kessler M, Günther P, Büchler MW. Pancreatic Surgery in Children: Complex, Safe, and Effective. Ann Surg 2024; 280:332-339. [PMID: 38386903 PMCID: PMC11224565 DOI: 10.1097/sla.0000000000006125] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/24/2024]
Abstract
OBJECTIVE The aim of this study was to assess indications for and report outcomes of pancreatic surgery in pediatric patients. BACKGROUND Indications for pancreatic surgery in children are rare and data on surgical outcomes after pediatric pancreatic surgery are scarce. METHODS All children who underwent pancreatic surgery at a tertiary hospital specializing in pancreatic surgery between 2003 and 2022 were identified from a prospectively maintained database. Indications, surgical procedures, and perioperative as well as long-term outcomes were analyzed. RESULTS In total, 73 children with a mean age of 12.8 years (range: 4 mo to 18 y) underwent pancreatic surgery during the observation period. Indications included chronic pancreatitis (n=35), pancreatic tumors (n=27), and pancreatic trauma (n=11). Distal pancreatectomy was the most frequently performed procedure (n=23), followed by pancreatoduodenectomy (n=19), duodenum-preserving pancreatic head resection (n=10), segmental pancreatic resection (n=7), total pancreatectomy (n=3), and others (n=11). Postoperative morbidity occurred in 25 patients (34.2%), including 7 cases (9.6%) with major complications (Clavien-Dindo≥III). There was no postoperative (90-d) mortality. The 5-year overall survival was 90.5%. The 5-year event-free survival of patients with chronic pancreatitis was 85.7%, and 69.0% for patients with pancreatic tumors. CONCLUSION This is the largest single-center study on pediatric pancreatic surgery in a Western population. Pediatric pancreatic surgery can be performed safely. Centralization in pancreatic centers with high expertise in surgery of adult and pediatric patients is important as it both affords the benefits of pancreatic surgery experience and ensures that surgical management is adapted to the specific needs of children.
Collapse
Affiliation(s)
- Juri Fuchs
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Martin Loos
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Benedict Kinny-Köster
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Thilo Hackert
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Martin Schneider
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Arianeb Mehrabi
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Christoph Berchtold
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Mohammed Al-Saeedi
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Beat P. Müller
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Oliver Strobel
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Manuel Feißt
- Institute of Medical Biometry, University of Heidelberg, Heidelberg, Germany
| | - Markus Kessler
- Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Patrick Günther
- Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Markus W. Büchler
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| |
Collapse
|
|
8
|
Leng Q, Lv W, Yang H, Li X, Wang W, Cheng K, Chang C, Cao D. Case report: Remarkable response to later-line surufatinib in an adult patient with liver metastatic of pancreatoblastoma. Front Pharmacol 2024; 15:1361628. [PMID: 38948477 PMCID: PMC11211453 DOI: 10.3389/fphar.2024.1361628] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/26/2023] [Accepted: 05/24/2024] [Indexed: 07/02/2024] Open
Abstract
Pancreatoblastoma (PB), a neoplasm derived from pancreatic follicular cells, primarily affects the pediatric population. Although infrequent in adults, it is associated with a considerably worse prognosis. Approximately one-third of patients are diagnosed with metastatic disease, with liver metastases being the most prevalent. Diagnosis relies on histopathological alterations including squamous vesicles, positive staining for CK8/CK18/CK19, and nuclear displacement of β-catenin. Additionally, liver metastases demonstrate substantial enhancement during the arterial phase of a contrast-enhanced computed tomography (CT) scan. Surgical resection serves as the principal therapeutic approach for addressing primary lesions and liver metastatic PB. In instances where surgical intervention is not viable, patients may derive benefits from systemic therapy and radiotherapy. This particular case report presents the clinical details of a 27-year-old female patient diagnosed with PB, who subsequently developed multiple liver metastases following a pancreaticoduodenectomy. Genomic examinations revealed the presence of ERBB2 amplification, RAD54L deletion, low TMB-L, and MSS in the patient. Despite the patient undergoing chemotherapy and Her-2 targeted therapy in conjunction with immunotherapy, no reduction in lesion size was observed until the administration of surufatinib. Subsequently, a notable outcome ensued, where the metastatic lesions were effectively excised via surgical intervention. Surufatinib has demonstrated a progression-free survival (PFS) of no less than 14 months, and the patient's survival has endured for a duration of 33 months. This indicates the potential efficacy of surufatinib as a viable therapeutic alternative for adult patients afflicted with PB.
Collapse
Affiliation(s)
- Qingqing Leng
- Division of Abdominal Tumor Multimodality Treatment, Cancer Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Wanrui Lv
- Department of Oncology, Meishan Municipal People’s Hospital, Meishan, Sichuan, China
| | - Heqi Yang
- Division of Abdominal Tumor Multimodality Treatment, Cancer Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Xiaofen Li
- Division of Abdominal Tumor Multimodality Treatment, Cancer Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Weiya Wang
- Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Ke Cheng
- Division of Abdominal Tumor Multimodality Treatment, Cancer Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Chen Chang
- Division of Abdominal Tumor Multimodality Treatment, Cancer Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Dan Cao
- Division of Abdominal Tumor Multimodality Treatment, Cancer Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| |
Collapse
|
|
9
|
Dumitrascu T. Preservation of Pancreatic Function Should Not Be Disregarded When Performing Pancreatectomies for Pancreatoblastoma in Children. Pediatr Rep 2024; 16:385-398. [PMID: 38804376 PMCID: PMC11130799 DOI: 10.3390/pediatric16020033] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/25/2024] [Revised: 05/05/2024] [Accepted: 05/10/2024] [Indexed: 05/29/2024] Open
Abstract
Complete surgical resection in the context of a multimodal approach has been associated with excellent long-term survival in children diagnosed with pancreatoblastoma (PB). Traditionally, curative intent surgery for PB implies standard pancreatic resections such as pancreaticoduodenectomies and distal pancreatectomies with splenectomies, surgical procedures that may lead to significant long-term pancreatic functional deficiencies. Postoperative pancreatic functional deficiencies are particularly interesting to children because they may interfere with their development, considering their long life expectancy and the significant role of pancreatic functions in their nutritional status and growth. Thus, organ-sparing pancreatectomies, such as spleen-preserving distal pancreatectomies and central pancreatectomies, are emerging in specific tumoral pathologies in children. However, data about organ-sparing pancreatectomies' potential role in curative-intent PB surgery in children are scarce. Based on the literature data, the current review aims to present the early and late outcomes of pancreatectomies in children (including long-term deficiencies and their potential impact on the development and quality of life), particularly for PB, and further explore the potential role of organ-sparing pancreatectomies for PB. Organ-sparing pancreatectomies are associated with better long-term pancreatic functional outcomes, particularly central pancreatectomies, and have a reduced impact on children's development and quality of life without jeopardizing their oncological safety. The long-term preservation of pancreatic functions should not be disregarded when performing pancreatectomies for PB in children. A subset of patients with PB might benefit from organ-sparing pancreatectomies, particularly from central pancreatectomies, with the same oncological results as standard pancreatectomies but with significantly less impact on long-term functional outcomes.
Collapse
Affiliation(s)
- Traian Dumitrascu
- Division of Surgical Oncology, Fundeni Clinical Institute, Department of General Surgery, Carol Davila University of Medicine and Pharmacy, Fundeni Street no. 258, 022328 Bucharest, Romania
| |
Collapse
|
|
10
|
Qi X, Zhou B, Liang F, Wang X. Prognostic factors of pancreatic tumors in children and adolescents: a population study based on the surveillance, epidemiology, and end results database. BMC Gastroenterol 2024; 24:108. [PMID: 38486208 PMCID: PMC10941481 DOI: 10.1186/s12876-024-03194-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/06/2023] [Accepted: 03/04/2024] [Indexed: 03/17/2024] Open
Abstract
PURPOSE Pancreatic tumors in children are uncommon, and data is scarce. The purpose of this study is to examine the prognostic factors of pediatric pancreatic tumors in a population-based cohort. METHODS The Surveillance, Epidemiology, and End Results (SEER) database was used to identify all pediatric patients with pancreatic tumors diagnosed between 1975 and 2018. The overall survival (OS) rates were determined using a Kaplan-Meier analysis. The log-rank test was used for univariate survival analysis. Cox proportional-hazards regression was used to determine the variables related to OS. RESULTS We identified 195 children with pancreatic tumors, with a median age at diagnosis of 16 years. Tumors were classified as neuroendocrine tumors (33.8%), solid pseudopapillary tumors (SPTs) (32.3%), pancreatoblastoma (11.3%), and others (22.6%). Of the patients, 30.3% had distant metastases, and 69.7% had surgery. Pancreatoblastomas were more common in younger children, whereas solid pseudopapillary tumors were more common in female patients. Overall 1-year, 3-year, and 5-year survival rates for all patients were 90.3%, 79.2%, and 77.7%, respectively. The Cox proportional hazard regression revealed that SEER stage and surgery were significant independent predictors of overall survival. CONCLUSIONS Pancreatic tumors are rare in children, and overall survival is grim except for SPTs. SEER stage and surgery were determined to be the most relevant determinants of OS in our study.
Collapse
Affiliation(s)
- Xianzhong Qi
- Department of Pathology, First People's Hospital of Linping District, Hangzhou, Zhejiang, China
| | - Bi Zhou
- Department of Pediatrics, Suzhou Hospital of Anhui Medical University, Suzhou, Anhui, China
| | - Fuhua Liang
- Department of Pediatric Surgery, Nanning Women and Children's Hospital, Nanning, Guangxi, China
| | - Xinxin Wang
- Department of Radiation Oncology, The Third Hospital of Nanchang, 330025, Nanchang, Jiangxi, China.
| |
Collapse
|
|
11
|
Jentzsch C, Fuchs J, Agaimy A, Vokuhl C, Escherich G, Blattmann C, Warmann SW, Schmidt A, Schäfer J, Brecht IB, Schneider DT, Abele M. Solid pseudopapillary neoplasms of the pancreas in childhood and adolescence-an analysis of the German Registry for Rare Pediatric Tumors (STEP). Eur J Pediatr 2023; 182:5341-5352. [PMID: 37733117 PMCID: PMC10746605 DOI: 10.1007/s00431-023-05203-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/01/2023] [Revised: 09/09/2023] [Accepted: 09/12/2023] [Indexed: 09/22/2023]
Abstract
Solid pseudopapillary neoplasms (SPNs) are the most common entity among pediatric pancreatic tumors. Still, these are rare tumors with an annual incidence of 0.1-0.2/1,000,000, and little is known about their optimal treatment. This analysis aimed to increase knowledge about the occurrence and treatment strategies of SPN in childhood. Data regarding diagnostics, treatment, and outcome of children aged 0-18 years with SPN recorded in the German Registry for Rare Pediatric Tumors (STEP) were analyzed. Thirty-eight patients were identified with a median age of 14.5 years at diagnosis (range: 8-18) and a female preponderance (81.6%). The most frequent location of the tumor was the pancreatic tail. In histopathological and immunohistochemical examination, pseudopapillary, solid, and cystic lesions as well as expression of beta-catenin, progesterone receptors, and cyclin D1 were the most common findings. All patients underwent surgical resection. Most patients underwent open resection, predominantly tail resection for tumors in the tail region and pylorus-preserving pancreaticoduodenectomy for tumors in the head region. The main postoperative sequela was exogenous pancreatic insufficiency (23.7%), especially with SPN in the pancreatic head. No recurrence occurred during follow-up, although two patients underwent resection with microscopic residue. CONCLUSION SPN of the pancreas in childhood are low-grade malignancies with usually favorable treatment outcomes. However, therapy can lead to relevant long-term sequelae. To prevent recurrence, complete surgical resection is recommended, sparing as much healthy pancreatic tissue as possible. Interdisciplinary collaboration between specialists is essential to optimize treatment. Molecular genetic analysis of these tumors could improve understanding of their genesis. WHAT IS KNOWN • Solid pseudopapillary neoplasms (SPNs) of the pancreas are very rare tumors in childhood. • Little is known about tumorigenesis, and there are no specific guidelines for treatment and follow-up in pediatric patients. WHAT IS NEW • Characteristics, treatment, and outcome were comprehensively assessed in a large cohort of pediatric patients with SPN. • We propose recommendations for diagnosis, treatment, and follow-up of children with SPN, based on our analysis and considering published experience.
Collapse
Affiliation(s)
- Christian Jentzsch
- Pediatric Hematology/Oncology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
| | - Jörg Fuchs
- Pediatric Surgery and Pediatric Urology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
| | - Abbas Agaimy
- Institute of Pathology, University Hospital Erlangen, Erlangen, Germany
| | - Christian Vokuhl
- Section of Pediatric Pathology, Department of Pathology, University Hospital Bonn, Bonn, Germany
| | - Gabriele Escherich
- Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
| | - Claudia Blattmann
- Department of Pediatric Oncology, Hematology and Immunology, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany
| | - Steven W Warmann
- Pediatric Surgery and Pediatric Urology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
| | - Andreas Schmidt
- Pediatric Surgery and Pediatric Urology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
| | - Jürgen Schäfer
- Section of Pediatric Radiology, Department of Diagnostic and Interventional Radiology, University Hospital Tuebingen, Tuebingen, Germany
| | - Ines B Brecht
- Pediatric Hematology/Oncology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
| | - Dominik T Schneider
- Clinic of Pediatrics, Klinikum Dortmund, University Witten/Herdecke, Dortmund, Germany
| | - Michael Abele
- Pediatric Hematology/Oncology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany.
| |
Collapse
|
|
12
|
Elks W, Li JR, Zajack A, Horner L, Ikeda A, St Hill C, Scheidler M, Chang S. Pancreaticoduodenectomy in 11-year-old male with a non-functional pancreatic neuroendocrine tumor. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2022. [DOI: 10.1016/j.epsc.2022.102516] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
|
|
13
|
Jayaraman D, John A, Bose JC, Venkitaraman B, Shalini S, Murali A. Successful Outcome of Metastatic Pancreatoblastoma in an Adolescent: A Case Report and Review of the Literature. J Indian Assoc Pediatr Surg 2022; 27:747-750. [PMID: 36714488 PMCID: PMC9878534 DOI: 10.4103/jiaps.jiaps_4_22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/11/2022] [Revised: 03/13/2022] [Accepted: 04/30/2022] [Indexed: 11/12/2022] Open
Abstract
Pancreatoblastoma, an orphan disease, is the most common malignant epithelial neoplasm of the pancreas in children. With vague clinical features, diagnosis is made by radiological suggestions and histopathology. The presence of metastatic disease and inoperable/incomplete excision remains the poor prognostic markers. We present a rare instance of an adolescent who has survived metastatic pancreatoblastoma after neoadjuvant chemoreduction/complete surgical excision.
Collapse
Affiliation(s)
- Dhaarani Jayaraman
- Department of Pediatrics, Division of Pediatric Hemato-Oncology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
| | - Arockia John
- Department of Pediatrics, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
| | - Jagadesh Chandra Bose
- Department of Surgical Oncology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
| | - Balasubramanian Venkitaraman
- Department of Surgical Oncology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
| | - Sai Shalini
- Department of Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
| | - Arunan Murali
- Department of Radio Diagnosis, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
| |
Collapse
|
|
14
|
Reissig TM, Uhrig S, Jost PJ, Luchini C, Vicentini C, Liffers ST, Allgäuer M, Adsay V, Scarpa A, Lawlor RT, Fröhling S, Stenzinger A, Klöppel G, Schildhaus HU, Siveke JT. MCL1 as putative target in pancreatoblastoma. Virchows Arch 2022; 481:265-272. [PMID: 35668118 PMCID: PMC9343273 DOI: 10.1007/s00428-022-03349-w] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2021] [Revised: 04/26/2022] [Accepted: 05/23/2022] [Indexed: 12/05/2022]
Abstract
Pancreatoblastoma (PB) is a rare tumor of the pancreas. In case of metastases, the treatment options are sparse and targeted approaches are not developed. We here evaluate MCL1 amplification as a putative target in PB.Thirteen samples from adult (10/13) and pediatric patients (3/13) were collected. Three of these samples had been previously subjected to whole-exome sequencing (2 cases) or whole-genome sequencing (1 case) within a precision oncology program (NCT/DKTK MASTER), and this analysis had shown copy number gains of MCL1 gene. We established a fluorescence in situ hybridization (FISH) test to assess the copy number alterations of MCL1 gene in 13 formalin-fixed paraffin-embedded PBs, including the 3 cases assessed by genome sequencing. FISH analysis showed the amplification of MCL1 in 2 cases (both were adult PB), one of which was a case with the highest copy number gain at genomic analysis. In both cases, the average gene copy number per cell was ≥ 5.7 and the MCL1/1p12 ratio was ≥ 2.4. Our data support MCL1 as a putative target in PB. Patients with MCL1-amplified PB might benefit from MCL1 inhibition. Sequencing data is useful to screen for amplification; however, the established FISH for MCL1 can help to determine the level and cellular heterogeneity of MCL1 amplification more accurately.
Collapse
Affiliation(s)
- Timm M Reissig
- Bridge Institute of Experimental Tumor Therapy, West German Cancer Center, University Hospital Essen, University Duisburg-Essen, Hufelandstrasse 55, 45147, Essen, Germany
- Division of Solid Tumor Translational Oncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Heidelberg, Germany
- Department of Medical Oncology, West German Cancer Center, University Hospital Essen, Essen, Germany
- German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany
| | - Sebastian Uhrig
- Computational Oncology Group, Molecular Diagnostics Program, NCT Heidelberg and DKFZ, Heidelberg, Germany
- Division of Applied Bioinformatics, German Cancer Research Center (DKFZ) and National Center for Tumor Diseases (NCT) Heidelberg, Heidelberg, Germany
- German Cancer Consortium (DKTK), Heidelberg, Germany
| | - Philipp J Jost
- Department of Medicine III, Klinikum rechts der Isar, TUM School of Medicine, Technical University of Munich, Munich, Germany
- Division of Clinical Oncology, Department of Medicine, Medical University of Graz, Graz, Austria
| | - Claudio Luchini
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
- ARC-NET Research Centre, University of Verona, Verona, Italy
| | - Caterina Vicentini
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Sven-Thorsten Liffers
- Bridge Institute of Experimental Tumor Therapy, West German Cancer Center, University Hospital Essen, University Duisburg-Essen, Hufelandstrasse 55, 45147, Essen, Germany
- Division of Solid Tumor Translational Oncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Heidelberg, Germany
- German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany
| | - Michael Allgäuer
- Institute of Pathology, University Hospital Heidelberg, Heidelberg, Germany
| | - Volkan Adsay
- Department of Pathology and Research Center for Translational Medicine (KUTTAM), Koç University, Istanbul, Turkey
| | - Aldo Scarpa
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
- ARC-NET Research Centre, University of Verona, Verona, Italy
| | | | - Stefan Fröhling
- German Cancer Consortium (DKTK), Heidelberg, Germany
- Division of Translational Medical Oncology, National Center for Tumor Diseases (NCT) Heidelberg and German Cancer Research Center (DKFZ), Heidelberg, Germany
| | - Albrecht Stenzinger
- German Cancer Consortium (DKTK), Heidelberg, Germany
- Institute of Pathology, University Hospital Heidelberg, Heidelberg, Germany
| | - Günter Klöppel
- Department of Pathology, Technical University of Munich, Munich, Germany
| | - Hans-Ulrich Schildhaus
- German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany.
- Institute of Pathology, West German Cancer Center, University Hospital Essen, University Duisburg-Essen, Hufelandstrasse 55, 45147, Essen, Germany.
| | - Jens T Siveke
- Bridge Institute of Experimental Tumor Therapy, West German Cancer Center, University Hospital Essen, University Duisburg-Essen, Hufelandstrasse 55, 45147, Essen, Germany.
- Division of Solid Tumor Translational Oncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Heidelberg, Germany.
- Department of Medical Oncology, West German Cancer Center, University Hospital Essen, Essen, Germany.
- German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany.
| |
Collapse
|
|
15
|
Liu T, Zhao T, Shi C, Chen L. Pancreatoblastoma in children: Clinical management and literature review. Transl Oncol 2022; 18:101359. [PMID: 35180620 PMCID: PMC8857517 DOI: 10.1016/j.tranon.2022.101359] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2021] [Revised: 01/15/2022] [Accepted: 01/25/2022] [Indexed: 11/25/2022] Open
Abstract
PURPOSE The aim of this study is to analyze the clinical and pathological features of pancreatoblastoma (PB) and to obtain better management for patients with relapsed or metastatic disease. METHODS Four cases treated in our institution and 59 cases reported previously in the literature from the PubMed biomedical database (2000-2020) were reviewed and analyzed. RESULTS Four cases with PB presented with abdominal pain and palpable abdominal masses, with the tumor size ranging from 5.2 to 18 cm in diameter. The invasion of the splenic vein and superior mesenteric artery, duodenum, and lymph nodes were risk factors for PB. Three cases were treated with combination therapy and showed favorable outcomes, while one case was treated with chemotherapy alone due to tumor progression and died of the disease. Squamous corpuscles were revealed in the tumor samples and considered a defining component for histological diagnosis. CONCLUSIONS Multidisciplinary diagnosis plays an important role in clinical management. The risk factors should be considered in the therapeutic stratification of PB before surgery.
Collapse
Affiliation(s)
- Tingting Liu
- Department of Pediatric Hematology/Oncology, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Tong Zhao
- Department of Pathology, The Second People's Hospital of Baoshan, Baoshan, Yunnan, China
| | - Cuicui Shi
- Department of Gastroenterology, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Lei Chen
- Department of Pathology, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Kongjiang Road 1665, Shanghai, China.
| |
Collapse
|
|
16
|
Patterson KN, Trout AT, Shenoy A, Abu-El-Haija M, Nathan JD. Solid pancreatic masses in children: A review of current evidence and clinical challenges. Front Pediatr 2022; 10:966943. [PMID: 36507125 PMCID: PMC9732489 DOI: 10.3389/fped.2022.966943] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/11/2022] [Accepted: 11/03/2022] [Indexed: 11/26/2022] Open
Abstract
Pancreatic tumors in children are infrequently encountered in clinical practice. Their non-specific clinical presentation and overlapping imaging characteristics often make an accurate preoperative diagnosis difficult. Tumors are categorized as epithelial or non-epithelial, with epithelial tumors further classified as tumors of the exocrine or endocrine pancreas. Although both are tumors of the exocrine pancreas, solid pseudopapillary neoplasm is the most prevalent solid pancreatic tumor in children, while pancreatoblastoma is the most common malignant tumor. Insulinoma is the most common pediatric pancreatic tumor of the endocrine pancreas. Malignant tumors require a complete, often radical, surgical resection. However, pancreatic parenchyma-sparing surgical procedures are utilized for benign tumors and low-grade malignancy to preserve gland function. This review will discuss the epidemiology, pathophysiology, clinical and diagnostic characteristics, and management options associated with both common and rare solid pancreatic masses in children. We will also discuss current challenges encountered in their evaluation and treatment.
Collapse
Affiliation(s)
- Kelli N Patterson
- Center for Surgical Outcomes Research, Abigail Wexner Research Institute, Nationwide Children's Hospital, Columbus, OH, United States
| | - Andrew T Trout
- Department of Radiology, Cincinnati Children's Hospital Medical Center, Departments of Radiology and Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States
| | - Archana Shenoy
- Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, Department of Pathology, The Ohio State University College of Medicine, Columbus, OH, United States
| | - Maisam Abu-El-Haija
- Division of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States
| | - Jaimie D Nathan
- Department of Abdominal Transplant and Hepatopancreatobiliary Surgery, Nationwide Children's Hospital, Department of Surgery, The Ohio State University College of Medicine, Columbus, OH, United States
| |
Collapse
|
|
17
|
Treatment Strategies for Children With Relapsed Pancreatoblastoma: A Literature Review. J Pediatr Hematol Oncol 2021; 43:288-293. [PMID: 33323880 DOI: 10.1097/mph.0000000000002033] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/21/2020] [Accepted: 11/05/2020] [Indexed: 11/26/2022]
Abstract
Pancreatoblastoma (PB) is a tumor typically seen in childhood. Despite its rarity, there are some internationally agreed recommendations for its first-line treatment, but very little is known about the management of relapse. We reviewed the literature on the treatment and outcome of children with progressing/recurrent PB, and the role of high-dose chemotherapy (HD-CT) or liver transplantation in difficult cases. A first analysis concerned 15 patients: liver metastases were the most frequent cause of first-line treatment failure. Eight patients underwent surgery, only 3 were irradiated. Various second-line chemotherapy regimens were adopted, with evidence of response in 8 children. The most often-used combinations included etoposide, cyclophosphamide/ifosfamide, and cisplatin/carboplatin. Overall, 7 patients are alive with a median follow-up of 24 months (range, 3 to 88 mo). In a separate analysis, considering patients in first-line or second-line treatment, we found 5 of 6 patients alive after HD-CT and 3 of 3 after liver transplantation. Our review shows that the outcome for patients with recurrent PB is not always dismal, especially when surgery is possible. Different chemotherapy combinations can be used, and HD-CT or liver transplantation may be considered in selected cases.
Collapse
|
|
18
|
Prud'homme N, Galante GJ, Kaur H, Brundler MA, Beaudry P, Strother D. Comment on: Pancreaticoduodenectomy for the treatment of pancreatic neoplasms in children: A Pediatric Surgical Oncology Research Collaborative study. Pediatr Blood Cancer 2021; 68:e29074. [PMID: 33890416 DOI: 10.1002/pbc.29074] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2021] [Accepted: 04/09/2021] [Indexed: 12/22/2022]
Affiliation(s)
- Nicolas Prud'homme
- Departments of Oncology and Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
| | - Gary J Galante
- Department of Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
| | - Harmeet Kaur
- Department of Radiology, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
| | - Marie-Anne Brundler
- Department of Pathology and Laboratory Medicine and Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
| | - Paul Beaudry
- Department of Surgery, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
| | - Douglas Strother
- Departments of Oncology and Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
| |
Collapse
|
|
19
|
Bolasco G, Capriati T, Grimaldi C, Monti L, De Pasquale MD, Patera IP, Spada M, Maggiore G, Diamanti A. Long-term outcome of pancreatic function following oncological surgery in children: Institutional experience and review of the literature. World J Clin Cases 2021; 9:7340-7349. [PMID: 34616800 PMCID: PMC8464449 DOI: 10.12998/wjcc.v9.i25.7340] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/23/2021] [Revised: 04/13/2021] [Accepted: 07/05/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Pancreatic neoplasms are uncommon in children and in most cases they are benign or have low malignant potential. Pancreatoblastoma and solid pseudopapillary tumor are the most frequent types in early and late childhood, respectively. Complete resection, although burdened by severe complications, is the only curative treatment for these diseases. Pancreatic surgery may result in impaired exocrine and endocrine pancreatic function. However, limited data are available on the long-term pediatric pancreatic function following surgical resection.
AIM To investigate endocrine and exocrine pancreatic function and growth after oncological pancreatic surgery in a pediatric series.
METHODS A retrospective analysis of all pediatric patients who underwent surgery for pancreatic neoplasm in our Institution from January 31, 2002 to the present was performed. Endocrine and exocrine insufficiency, auxological and fat-soluble vitamin status (A, D, E and clotting tests) were assessed at diagnosis and at every follow-up visit. Exocrine insufficiency was defined as steatorrhea with fecal elastase-1 < 200 µg/g stool, while endocrine insufficiency was identified as onset of Diabetes or Impaired Glucose Tolerance. Growth was evaluated based on body mass index (BMI) z-score trend.
RESULTS Sixteen patients (12 girls and 4 boys, mean age 10.7 ± 5.3 years), were included. Nine patients (56%) had a neoplasm in the pancreatic head, 4 in the body/tail, 2 in the tail and 1 in the body. Histological findings were as follows: Solid pseudopapillary tumor in 10 patients (62.5%), insulinoma in 2 patients, neuroendocrine tumor in 2 patients and acinar cell carcinoma in 2 patients. The most frequent surgery was pancreaticoduodenectomy (50%). Exocrine failure occurred in 4 patients (25%) and endocrine failure in 2 patients (12.5%). Exocrine insufficiency occurred early (within 6 mo after surgery) and endocrine insufficiency later (8 and 10 years after surgery). Mean BMI z-score was 0.36 ± 1.1 at diagnosis and 0.27 ± 0.95 at the last assessment. Vitamin D was insufficient (< 30 ng/mL) in 8 of the 16 patients during the follow-up period. Vitamins A, E and clotting test were into the normal ranges in all patients.
CONCLUSION Careful and long-term monitoring should follow any pancreatic surgery, to recognize and promptly treat exocrine and endocrine pancreatic insufficiency, which can occur after surgery.
Collapse
Affiliation(s)
- Giulia Bolasco
- Hepatology, Gastroenterology and Nutrition Unit, “Bambino Gesù” Children’s Hospital, IRCCS, Rome 00165, Italy
| | - Teresa Capriati
- Hepatology, Gastroenterology and Nutrition Unit, “Bambino Gesù” Children’s Hospital, IRCCS, Rome 00165, Italy
| | - Chiara Grimaldi
- Division of Hepatobiliopancreatic Surgery, Liver and Kidney Transplantation, "Bambino Gesù" Children's Hospital, IRCCS, Rome 00165, Italy
| | - Lidia Monti
- Radiology Department, "Bambino Gesù" Children’s Hospital, IRCCS, Rome 00165, Italy
| | | | | | - Marco Spada
- Division of Hepatobiliopancreatic Surgery, Liver and Kidney Transplantation, "Bambino Gesù" Children's Hospital, IRCCS, Rome 00165, Italy
| | - Giuseppe Maggiore
- Hepatology, Gastroenterology and Nutrition Unit, “Bambino Gesù” Children’s Hospital, IRCCS, Rome 00165, Italy
| | - Antonella Diamanti
- Hepatology, Gastroenterology and Nutrition Unit, “Bambino Gesù” Children’s Hospital, IRCCS, Rome 00165, Italy
| |
Collapse
|
|
20
|
Paredes O, Kawaguchi Y, Ruiz E, Payet E, Berrospi F. Surgery of pancreas tumors in pediatric and adolescent patients: a single institution experience in South America. Pediatr Surg Int 2021; 37:1041-1047. [PMID: 33742268 DOI: 10.1007/s00383-021-04877-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 03/02/2021] [Indexed: 11/28/2022]
Abstract
PURPOSE Pancreas tumors are extremely rare in pediatric and adolescent patients. Surgical resection is the mainstay of treatment; however, the data are limited with respect to morbidity and mortality. We aimed to evaluate short- and long-term outcomes of pediatric and adolescent patients who underwent surgical resection of pancreatic tumors. METHODS Patients [Formula: see text] 18-year-olds who underwent resection of pancreas tumor at the National Institute of Neoplastic Diseases INEN during 2000-2020 were included. RESULTS Thirty-four patients were diagnosed; 28 patients were female and 6 were male. The median age was 13.4-years-old. Histological diagnosis was solid pseudopapillary neoplasm (SPN) (n = 29, 85.3%), pancreatoblastoma (n = 3), neuroendocrine carcinoma (n = 1), and insulinoma (n = 1). No patient experienced postoperative mortality and 15 (44.1%) patients developed postoperative complications including pancreatic fistula as the most frequent. Under a median follow-up period of 33.8 (0.5-138) months, four (11.8%) patients died. Of the 29 patients with SPN, the 3- and-5-year OS rates were 100% and 83.1%, respectively. CONCLUSIONS SPN was the most frequent cause of surgical treatment for pediatric and adolescent patients in the high-volume cancer center in Peru and was associated with favorable survival. Pancreaticoduodenectomy was safely performed in this patient group with acceptable morbidity and zero mortality.
Collapse
Affiliation(s)
- Oscar Paredes
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Holbein Street 107, Lima, Perú.
| | - Yoshikuni Kawaguchi
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Eloy Ruiz
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Holbein Street 107, Lima, Perú
| | - Eduardo Payet
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Holbein Street 107, Lima, Perú
| | - Francisco Berrospi
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Holbein Street 107, Lima, Perú
| |
Collapse
|
|
21
|
Pancreatic resection in the pediatric, adolescent and young adult population: nationwide analysis on complications. HPB (Oxford) 2021; 23:1175-1184. [PMID: 33281080 DOI: 10.1016/j.hpb.2020.10.029] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/03/2020] [Revised: 10/09/2020] [Accepted: 10/14/2020] [Indexed: 12/12/2022]
Abstract
BACKGROUND The aim of this study was to determine pancreatic surgery specific short- and long-term complications of pediatric, adolescent and young adult (PAYA) patients who underwent pancreatic resection, as compared to a comparator cohort of adults. METHODS A nationwide retrospective cohort study was performed in PAYA patients who underwent pancreatic resection between 2007 and 2016. PAYA was defined as all patients <40 years at time of surgery. Pancreatic surgery-specific complications were assessed according to international definitions and textbook outcome was determined. RESULTS A total of 230 patients were included in the PAYA cohort (112 distal pancreatectomies, 99 pancreatoduodenectomies), and 2526 patients in the comparator cohort. For pancreatoduodenectomy, severe morbidity (29.3% vs. 28.6%; P = 0.881), in-hospital mortality (1% vs. 4%; P = 0.179) and textbook outcome (62% vs. 58%; P = 0.572) were comparable between the PAYA and the comparator cohort. These outcomes were also similar for distal pancreatectomy. After pancreatoduodenectomy, new-onset diabetes mellitus (8% vs. 16%) and exocrine pancreatic insufficiency (27% vs. 73%) were lower in the PAYA cohort when compared to adult literature. CONCLUSION Pancreatic surgery-specific complications were comparable with patients ≥40 years. Development of endocrine and exocrine insufficiency in PAYA patients who underwent pancreatoduodenectomy, however, was substantially lower compared to adult literature.
Collapse
|
|
22
|
Bien E, Roganovic J, Krawczyk MA, Godzinski J, Orbach D, Cecchetto G, Barthlen W, Defachelles AS, Ferrari A, Weldon CB, Brecht IB, Schneider DT, Bisogno G, Kolenova A, Ben-Ami T, Martinova K, Virgone C, Stachowicz-Stencel T, Kachanov D, Reguerre Y. Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations. Pediatr Blood Cancer 2021; 68 Suppl 4:e29112. [PMID: 34174157 DOI: 10.1002/pbc.29112] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/22/2021] [Revised: 04/20/2021] [Accepted: 04/25/2021] [Indexed: 12/17/2022]
Abstract
Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm that affects typically young children. Signs related to advanced upper-abdominal tumor accompanied by elevated serum α-fetoprotein levels in a young child suggest PBL, however histopathological confirmation is mandatory. The mainstay of the treatment is a complete surgical resection. Unresectable and/or metastatic PBL may become amenable to complete delayed surgery after neoadjuvant chemotherapy. This manuscript presents the international consensus recommendations for the diagnosis and treatment of children with PBL, established by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the EU-funded PARTNER (Paediatric Rare Tumors Network - European Registry) project.
Collapse
Affiliation(s)
- Ewa Bien
- Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland
| | - Jelena Roganovic
- Department of Pediatrics, Clinical Hospital Center Rijeka, University of Rijeka, Rijeka, Croatia
| | - Malgorzata A Krawczyk
- Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland
| | - Jan Godzinski
- Department of Pediatric Surgery, Marciniak Hospital, Wroclaw, Poland.,Department of Pediatric Traumatology and Emergency Medicine, Wroclaw Medical University, Wroclaw, Poland
| | - Daniel Orbach
- SIREDO Oncology Center (Care, Innovation and Research for Children and AYA with Cancer), PSL Research University, Institut Curie, Paris, France
| | - Giovanni Cecchetto
- Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | - Winfred Barthlen
- Pediatric Surgery, Universitaetsmedizin Greifswald, Greifswald, Germany
| | | | - Andrea Ferrari
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Christopher B Weldon
- Departments of Surgery, Oncology and Anesthesiology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard University, Boston, Massachusetts, USA
| | - Ines B Brecht
- Pediatric Hematology and Oncology, Children's Hospital, Eberhard-Karls-Universitaet Tübingen, Tübingen, Germany
| | | | - Gianni Bisogno
- Hematology-Oncology Division, Department of Pediatrics, Padova University Hospital, Padua, Italy
| | | | - Tal Ben-Ami
- Pediatric Hematology Unit, Kaplan Medical Center, Rehovot, Israel
| | - Kata Martinova
- Department of Hematology and Oncology, University Clinic for Children`s Diseases, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje, Republic of North Macedonia
| | - Calogero Virgone
- Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | | | - Denis Kachanov
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia
| | - Yves Reguerre
- Department of Pediatric Hematology and Oncology, Félix Guyon University Hospital, St Denis, Réunion Island, France
| |
Collapse
|
|
23
|
Abstract
Abstract
Introduction
Pancreatic tumors are reported rarely in childhood and represent an extremely rare entity in Pediatric Oncology. One of the least common types of pediatric pancreatic tumor is acinar cell carcinoma (ACC). We aim to present a rare case of ACC and the difficulties we faced during diagnosis and treatment.
Patient and Methods
An 8-year old girl presented with jaundice. Workup revealed a tumor originating from the head of the pancreas with multiple metastatic lesions in her liver. Evaluation of tumor markers revealed elevated levels of AFP. Pathology report was indicative of acinar cell carcinoma of the pancreas.
Results
After consulting the EXPeRT group (European Cooperative Study Group for Pediatric Rare Tumors), chemotherapy was initiated. Partial response was observed after the first 4 courses with decrease of AFP levels. While planning her surgery, AFP elevated and a second-line course of chemotherapy was administered. Our patient underwent Whipple’s Duodenopancreatectomy with partial metastasectomy. Although the postoperative period was uneventful, AFP continued to rise even after postoperative chemotherapy was administered. There were signs of metastatic disease progression. Our patient received a third-line regimen with no improvement. She received local radiotherapy and a next-line chemotherapy course. Local relapse and metastatic disease progression placed our patient in palliative care. She passed away nine months after the initial diagnosis.
Conclusions
Acinar cell carcinoma of the pancreas is a rare type of pediatric cancer with very challenging diagnosis and treatment. Cooperation at the European level and multicenter management of those rare cases is vital for the optimum outcome.
Collapse
|
|
24
|
Chagpar R, Naples R, Siperstein AE. Pancreatic Neuroendocrine Tumors. PEDIATRIC GASTROINTESTINAL AND LIVER DISEASE 2021:938-948.e4. [DOI: 10.1016/b978-0-323-67293-1.00084-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2025]
|
|
25
|
Cheng H, Yang S, Ren Q, Yang W, Han W, Chang X, Zhu Z, Qin H, Wang H. Pancreatectomies for pediatric pancreatic tumors: A single institute experience from 2007 to 2018. J Pediatr Surg 2020; 55:1722-1726. [PMID: 31575410 DOI: 10.1016/j.jpedsurg.2019.08.051] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/20/2019] [Revised: 08/18/2019] [Accepted: 08/31/2019] [Indexed: 02/07/2023]
Abstract
BACKGROUND To investigate the safety, feasibility, and complications of pancreatectomies for pediatric pancreatic tumors. METHODS The medical records of pancreatectomy patients from January 2007 to January 2018 were retrospectively analyzed for perioperative factors and complications. Patients were divided into pancreatic head (n = 43), body (n = 18) and tail (n = 43) groups. RESULTS Seventy-two girls and 32 boys (median age 10 years at diagnosis, range: 0-15 years) were enrolled and had solid pseudopapillary tumors (n = 73), pancreatoblastoma (n = 19), neuroendocrine tumors (n = 9), and others. Primary surgical procedures included pylorus-preserving pancreaticoduodenectomy (n = 10) and distal pancreatectomy with splenectomy (n = 4), and organ-sparing resection procedures included duodenum-preserving pancreas head resection (n = 25), middle segmental pancreatic resection (n = 15), spleen-preserving distal pancreatectomy (n = 37) and local enucleation (n = 13), with a median blood loss of 20 cm3 (range: 10-300 cm3). Short-term complications included pancreatic fistula (35.6%), bile leakage (2.9%), intraabdominal infection (21.2%), delayed gastric emptying (23.1%), and postpancreatectomy hemorrhage (5.8%). After a median follow-up of 38 months (range: 3-143 months), 94 patients (90.4%) were alive without tumor recurrence, 2 were alive after tumor recurrence, 1 pancreatoblastoma patient died from tumor recurrence, and 7 were lost to follow-up. Only 14 patients (14/96, 14.6%) had long-term complications at the outpatient follow-up. CONCLUSIONS Surgical resection was the main treatment for pancreatic tumors. Organ-sparing resection procedures led to good long-term results for pediatric pancreatic tumors, even if these procedures could cause a relatively high incidence of short-term complications (especially pancreatic fistula and postpancreatectomy hemorrhage). LEVEL OF EVIDENCE Level IV.
Collapse
Affiliation(s)
- Haiyan Cheng
- Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China
| | - Shen Yang
- Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China
| | - Qinghua Ren
- Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China
| | - Wei Yang
- Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China
| | - Wei Han
- Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China
| | - Xiaofeng Chang
- Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China
| | - Zhiyun Zhu
- Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China
| | - Hong Qin
- Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
| | - Huanmin Wang
- Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
| |
Collapse
|
|
26
|
Yepuri N, Naous R, Meier AH, Cooney RN, Kittur D, Are C, Jain A, Dhir M. A systematic review and meta-analysis of predictors of recurrence in patients with Solid Pseudopapillary Tumors of the Pancreas. HPB (Oxford) 2020; 22:12-19. [PMID: 31350105 DOI: 10.1016/j.hpb.2019.06.005] [Citation(s) in RCA: 21] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/10/2018] [Revised: 06/02/2019] [Accepted: 06/09/2019] [Indexed: 12/12/2022]
Abstract
BACKGROUND The recurrence rates and predictors of recurrence in patients with Solid Pseudopapillary tumors (SPT) are unclear, which makes it challenging to determine the duration of follow-up. The aim of the current study was to perform a systematic review and meta-analysis to determine the recurrence rates and pathologic factors associated with recurrence in patients with SPT. METHODS A PubMed, Scopus, and Web of Science search was conducted to identify studies of SPT published during the last 15 years: (09/2002-09/2017). Studies reporting on patients with SPT and follow-up of >5 years were included. The search strategy was conducted per 2009 PRISMA guidelines. RESULTS A total of 103 studies reporting on 2599 non-metastatic SPT patients were identified. Sixty-nine patients (2.6%) developed recurrence during follow-up. Pooled estimates from studies with a sample size >20 (N = 33) noted an overall recurrence rate of 2% (95% CI 1-2%). Male gender (OR 1.960), positive lymph nodes (OR 11.9), R1 margins (OR 11.1), and LVI (OR 5.5), were associated with a significantly (all p < 0.05) increased risk of recurrence. CONCLUSION Current meta-analysis suggests that only 2% of patients with SPT experience recurrence after resection. These data will guide the treating physicians and patients regarding recurrence rates and help identify patients at increased risk of recurrence during follow-up.
Collapse
Affiliation(s)
- Natesh Yepuri
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Rana Naous
- Department of Pathology, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Andreas H Meier
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Robert N Cooney
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Dilip Kittur
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Chandrakanth Are
- Department of Surgery, Division of Surgical Oncology, University of Nebraska Medical Center, Omaha, NE, 68198, USA
| | - Ajay Jain
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Mashaal Dhir
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA.
| |
Collapse
|
|
27
|
Grigoletto V, Tagarelli A, Atzeni C, Cecchetto G, Indolfi P, De Pasquale MD, De Leonardis F, Coppadoro B, Sorbara S, Chiaravalli S, Ferrari A, Bisogno G. Pleuropulmonary blastoma: a report from the TREP (Tumori Rari in Età Pediatrica) Project. TUMORI JOURNAL 2019; 106:126-132. [PMID: 32270754 DOI: 10.1177/0300891619871344] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
INTRODUCTION Pleuropulmonary blastoma (PPB) is a rare, aggressive mesenchymal tumor of childhood. The Italian Tumori Rari in Età Pediatrica (TREP) Registry was the first in Europe dedicated to prospective data collection on rare pediatric tumors. We analyzed data from an Italian series of patients with PPB, focusing on the role of the TREP Project. METHODS We considered patients aged 0-14 with histologically confirmed diagnosis, registered in population-based cancer registries (before 2000) or the TREP Registry (2000 to 2014), and analyzed data on clinical characteristics, treatment, and outcome. Event-free survival (EFS) and overall survival (OS) were estimated. Relevant prognostic factors were identified performing a univariate analysis. RESULTS Thirty-seven cases were included (7 type I, 13 type II, 17 type III). The average diagnosis rate rose from 1.10 to 1.73 cases/year after the TREP Project started. All patients underwent surgery, 33 received chemotherapy, and 9 had radiotherapy. The median follow-up was 8.7 years. For type I, II, and III, respectively, the 5-year OS was 85.7% (33.4-97.9), 52.7% (23.4-75.5), and 57.8% (31.1-77.3); the 5-year EFS was 85.7% (33.4-97.9), 52.7% (23.4-75.5), and 52.9% (27.6-73.0). Favorable prognostic factors for EFS were Intergroup Rhabdomyosarcoma Study (IRS) stage I (p = 0.03) and T1 tumor (p = 0.05). A total of 78.3% of patients who had chemotherapy after 2000 received a standardized treatment. CONCLUSIONS The TREP Registry showed an excellent capacity for registering cases of PPB. Patients received homogeneous treatment after the TREP Project started. Long-term outcomes were excellent for type I and unsatisfactory for type II and III. Tumor invasiveness and IRS stage were of prognostic value.
Collapse
Affiliation(s)
- Veronica Grigoletto
- Division of Pediatric Hematology and Oncology, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | - Arianna Tagarelli
- Division of Pediatric Hematology and Oncology, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | - Catia Atzeni
- Division of Pediatric Hematology and Oncology, Ospedale Microcitemico, ASL Cagliari, Cagliari, Italy
| | - Giovanni Cecchetto
- Division of Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | - Paolo Indolfi
- Pediatric Oncology Service, Department of Pediatrics, Second University, Naples, Italy
| | | | - Francesco De Leonardis
- Division of Pediatric Hematology and Oncology, Department of Pediatrics, University of Bari Faculty of Medicine and Surgery, Bari, Italy
| | - Beatrice Coppadoro
- Division of Pediatric Hematology and Oncology, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | - Silvia Sorbara
- Division of Pediatric Hematology and Oncology, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | | | - Andrea Ferrari
- Pediatric Oncology Unit, IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - Gianni Bisogno
- Division of Pediatric Hematology and Oncology, Department of Women's and Children's Health, University of Padua, Padua, Italy
| |
Collapse
|
|
28
|
|
|
29
|
Crocoli A, Grimaldi C, Virgone C, De Pasquale MD, Cecchetto G, Cesaro S, Bisogno G, Cecinati V, Narciso A, Alberti D, Ferrari A, Dall'Igna P, Spada M, Inserra A. Outcome after surgery for solid pseudopapillary pancreatic tumors in children: Report from the TREP project-Italian Rare Tumors Study Group. Pediatr Blood Cancer 2019; 66:e27519. [PMID: 30362240 DOI: 10.1002/pbc.27519] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/05/2018] [Revised: 09/12/2018] [Accepted: 10/01/2018] [Indexed: 12/11/2022]
Abstract
BACKGROUND Solid pseudopapillary pancreatic tumors (SPPT) are an extremely rare entity in pediatric patients. Even if the role of radical surgical resection as primary treatment is well established, data about follow-up after pancreatic resection in children are scant. METHODS A retrospective review of data from the Italian Pediatric Rare Tumor Registry (TREP) was performed. Short-term (<30 days) and long-term complications of different surgical resections, as well as long-term follow-up were evaluated. RESULTS From January 2000 to present, 43 patients (male:female = 8:35) were enrolled. The median age at diagnosis was 13.2 years (range, 7-18). Nine children had an incidental diagnosis, whereas 26 complained of abdominal pain and 4 of palpable mass. Tumors arose either from the head of pancreas (n = 14) or from body/tail (n = 29): only one patient presented with metastatic disease. Resection was complete in all patients (cephalic duodenopancreatectomy vs distal resection). At follow-up (median, 8.4 years; range, 0-17 years), one recurrence occurred in a patient with intraoperative rupture. All patients are alive. Three pancreatic fistulas occurred in the body/tail group, whereas four complications occurred in the head group (one ileal ischemia, two stenosis of the pancreatic duct, and one chylous fistula). CONCLUSION Surgery is the best therapeutic option for these tumors; hence, complete resection is mandatory. Extensive resections, including cephalic duodenopancreatectomy, are safe when performed in specialized centers. Long-term follow-up should be aimed to detect tumor recurrence and to evaluate residual pancreatic function.
Collapse
Affiliation(s)
- Alessandro Crocoli
- Department of Pediatric Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
| | - Chiara Grimaldi
- Department of Pediatric Surgery and Transplantation, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
| | - Calogero Virgone
- Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | | | - Giovanni Cecchetto
- Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | - Simone Cesaro
- Pediatric Hematology-Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy
| | - Gianni Bisogno
- Hematology Oncology Division, Department of Women's and Children's Health, University of Padova, Padova, Italy
| | - Valerio Cecinati
- Pediatric Hematology and Oncology Unit, Department of Hematology, Transfusion Medicine and Biotechnology, Pescara, Italy
| | - Alessandra Narciso
- Department of Pediatric Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
| | - Daniele Alberti
- Pediatric Surgery Department, ASST Spedali Civili di Brescia, Brescia, Italy
| | - Andrea Ferrari
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - Patrizia Dall'Igna
- Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | - Marco Spada
- Department of Pediatric Surgery and Transplantation, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
| | - Alessandro Inserra
- Department of Pediatric Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
| |
Collapse
|
|
30
|
Mylonas KS, Doulamis IP, Tsilimigras DI, Nasioudis D, Schizas D, Masiakos PT, Kelleher CM. Solid pseudopapillary and malignant pancreatic tumors in childhood: A systematic review and evidence quality assessment. Pediatr Blood Cancer 2018; 65:e27114. [PMID: 29697193 DOI: 10.1002/pbc.27114] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/09/2017] [Revised: 02/22/2018] [Accepted: 03/18/2018] [Indexed: 12/15/2022]
Abstract
A systematic review of 32 studies reporting on 489 children with pancreatic tumors was performed. The most prevalent histologic subtype was solid pseudopapillary tumor (SPT) (61.3%). Pancreaticoduodenectomy was the most commonly performed operation (48%). Neoadjuvant chemo/radiotherapy was utilized in 3.9, 36.2, 25, and 27.8% of patients with SPTs, pancreatoblastomas (PBLs), neuroendocrine tumors, and exocrine carcinomas, respectively. Adjuvant chemotherapy (75.6%) and radiation (34%) were most commonly utilized in PBLs. All-cause mortality was highest in exocrine carcinomas (50%). Overall, 98.8% of patients with SPTs survived. PBL exhibited the highest recurrence rate (14.7%) within a mean of 23.5 months.
Collapse
Affiliation(s)
- Konstantinos S Mylonas
- Department of Pediatric Surgery, Massachusetts General Hospital, Boston, Massachusetts.,Pediatrics Working Group, Society of Junior Doctors, Athens, Greece
| | - Ilias P Doulamis
- Surgery Working Group, Society of Junior Doctors, Athens, Greece
| | - Diamantis I Tsilimigras
- Surgery Working Group, Society of Junior Doctors, Athens, Greece.,First Department of Surgery, Laiko General Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
| | - Dimitrios Nasioudis
- Surgery Working Group, Society of Junior Doctors, Athens, Greece.,Department of Obstetrics and Gynecology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania
| | - Dimitrios Schizas
- Surgery Working Group, Society of Junior Doctors, Athens, Greece.,First Department of Surgery, Laiko General Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
| | - Peter T Masiakos
- Department of Pediatric Surgery, Massachusetts General Hospital, Boston, Massachusetts
| | - Cassandra M Kelleher
- Department of Pediatric Surgery, Massachusetts General Hospital, Boston, Massachusetts
| |
Collapse
|
|
31
|
Mylonas KS, Nasioudis D, Tsilimigras DI, Doulamis IP, Masiakos PT, Kelleher CM. A population-based analysis of a rare oncologic entity: Malignant pancreatic tumors in children. J Pediatr Surg 2018; 53:647-652. [PMID: 28693851 DOI: 10.1016/j.jpedsurg.2017.06.024] [Citation(s) in RCA: 27] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2017] [Revised: 05/19/2017] [Accepted: 06/23/2017] [Indexed: 12/30/2022]
Abstract
PURPOSE To examine the clinicopathological characteristics and prognosis of pediatric patients with malignant pancreatic tumors in a population-based cohort. METHODS The Surveillance, Epidemiology, and End Results (SEER) database was utilized to identify all pediatric patients with malignant pancreatic tumors, diagnosed between 1973 and 2013. Kaplan-Meier analysis was performed to determine median and five-year overall survival (OS) rates. Univariate survival analysis was executed using the log-rank test. Cox proportional hazards model was used to identify variables independently associated with mortality. RESULTS A total of 114 patients with pancreatic malignancies were identified. Median patient age was 16years and the majority of patients were white (64%) females (61.4%). The most prevalent histologic subtype was neuroendocrine tumors (35.1%), whereas pancreatoblastoma was more common during the first decade of life (P<0.001). Distant metastases were noted in 41.7% of the patients, while 33.3% and 25% had localized and regional disease respectively. Five-year OS rates were 77%, 66.4% and 64.8% for patients with pancreatoblastoma, neuroendocrine and epithelial tumors respectively. No death was observed in the solid pseudopapillary tumor group. Only history of having cancer-directed surgery (CDS) was significantly associated with lower overall mortality (HR: 5.1, 95% CI: 2.1, 12.4). CONCLUSION Pancreatic malignancies are rare in children. Their prognosis is variable and only CDS was independently associated with superior survival. EVIDENCE RATING/CLASSIFICATION Prognosis study, Level II.
Collapse
Affiliation(s)
- Konstantinos S Mylonas
- Department of Pediatric Surgery, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA; Surgery Working Group, Society of Junior Doctors, Menelaou 5 Street, 15123, Athens, Greece.
| | - Dimitrios Nasioudis
- Surgery Working Group, Society of Junior Doctors, Menelaou 5 Street, 15123, Athens, Greece; Department of Obstetrics and Gynecology, Weill Cornell Medicine, 1300 York Avenue, New York, NY 10065, USA.
| | - Diamantis I Tsilimigras
- Surgery Working Group, Society of Junior Doctors, Menelaou 5 Street, 15123, Athens, Greece; School of Medicine, National and Kapodistrian University of Athens, Mikras Asias 75, 11527, Athens, Greece.
| | - Ilias P Doulamis
- Surgery Working Group, Society of Junior Doctors, Menelaou 5 Street, 15123, Athens, Greece.
| | - Peter T Masiakos
- Department of Pediatric Surgery, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
| | - Cassandra M Kelleher
- Department of Pediatric Surgery, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
| |
Collapse
|
|
32
|
Scandavini C, Valente R, Rangelova E, Segersvärd R, Arnelo U, Permert J, Svensson PJ, Stenman J, Del Chiaro M. Pancreatectomies for pancreatic neoplasms in pediatric and adolescent age: A single institution experience. Pancreatology 2018; 18:204-207. [PMID: 29277262 DOI: 10.1016/j.pan.2017.12.009] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/16/2017] [Revised: 11/22/2017] [Accepted: 12/18/2017] [Indexed: 02/07/2023]
Abstract
BACKGROUND There are very few data in the current literature regarding the short- and long-term outcome of surgery for pediatric pancreatic tumors (PPT). No data are available on the impact of pancreatic surgery on the children's growth. METHODS This is a retrospective cohort study on a consecutive series of pediatric/adolescent patients who underwent pediatric surgery at Karolinska University Hospital from January 2005 to July 2017. RESULTS Overall 14 pancreatic operations were performed in 13 patients. The median age was 11.4 years (range 3-15). Six pancreaticoduodenectomies (42.8%), 5 distal pancreatectomies (35.7%), and 3 enucleations (21.5%) were performed. The final histology revealed a solid pseudopapillary tumor in 9 cases (69.2%), neuroblastoma in 1 (7.7%), ganglioneuroma in 1 (7.7%), pancreatoblastoma in 1 (7.7%), and insulinoma in 1 (7.7%). Overall, 3 patients developed post-operative complications (23%). There was no peri-operative mortality. All patients are alive after a median follow-up time of 80 months. Exocrine insufficiency was detected post-operatively in 4 patients (30.7%) Endocrine insufficiency requiring insulin treatment developed in one patient (7.7%). No significant impact on growth was detected in any of the patients after pancreatic resection. CONCLUSIONS In our series, surgery performed for PPTs seems to be safe and effective. The effect of pancreatic surgery on children's growth does not seem to be significant.
Collapse
Affiliation(s)
- Chiara Scandavini
- Pancreatic Surgery Unit, Division of Surgery (KLINTEC) - Karolinska Institutet at Center for Digestive Diseases Karolinska University Hospital, Stockholm, Sweden
| | - Roberto Valente
- Pancreatic Surgery Unit, Division of Surgery (KLINTEC) - Karolinska Institutet at Center for Digestive Diseases Karolinska University Hospital, Stockholm, Sweden; Digestive and Liver Diseases Unit - Sapienza University of Rome, Italy
| | - Elena Rangelova
- Pancreatic Surgery Unit, Division of Surgery (KLINTEC) - Karolinska Institutet at Center for Digestive Diseases Karolinska University Hospital, Stockholm, Sweden
| | - Ralf Segersvärd
- Pancreatic Surgery Unit, Division of Surgery (KLINTEC) - Karolinska Institutet at Center for Digestive Diseases Karolinska University Hospital, Stockholm, Sweden
| | - Urban Arnelo
- Pancreatic Surgery Unit, Division of Surgery (KLINTEC) - Karolinska Institutet at Center for Digestive Diseases Karolinska University Hospital, Stockholm, Sweden
| | - Johan Permert
- Pancreatic Surgery Unit, Division of Surgery (KLINTEC) - Karolinska Institutet at Center for Digestive Diseases Karolinska University Hospital, Stockholm, Sweden
| | - Pär-Johan Svensson
- Astrid Lindgren Children's Hospital, Karolinska University Hospital and Department for Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
| | - Jakob Stenman
- Astrid Lindgren Children's Hospital, Karolinska University Hospital and Department for Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
| | - Marco Del Chiaro
- Pancreatic Surgery Unit, Division of Surgery (KLINTEC) - Karolinska Institutet at Center for Digestive Diseases Karolinska University Hospital, Stockholm, Sweden.
| |
Collapse
|
|
33
|
Yao L, Xie ZB, Jin C, Jiang YJ, Li J, Yang F, Lin QJ, Fu DL. Radical resection and enucleation in Chinese adolescents with pancreatic tumors: A 15-year case series. Medicine (Baltimore) 2017; 96:e6438. [PMID: 28328854 PMCID: PMC5371491 DOI: 10.1097/md.0000000000006438] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/24/2016] [Revised: 02/22/2017] [Accepted: 02/25/2017] [Indexed: 12/24/2022] Open
Abstract
Pancreatic tumors rarely occur in adolescents, and the appropriateness of radical resection for these patients remains controversial.Medical records were retrospectively reviewed for patients younger than 19 years who underwent radical resection or limited resection (enucleation) between 2000 and 2015. Patient demographics, clinical characteristics, operative details, growth, and survival were analyzed.During the study period, 11 adolescents (mean age, 16.18 years; standard deviation, 1.99; interquartile range, 15.0-18.0) underwent radical resection (n = 7) or enucleation (n = 4) to treat solid pseudopapillary tumors (n = 5), pancreatic neuroendocrine tumors (n = 5), or pancreatic ductal adenocarcinoma (n = 1). None of the 7 patients who underwent radical resection experienced recurrence or serious complications, while 3 of 4 patients who underwent enucleation experienced recurrence (P = 0.02). Recurrence-free survival was slightly longer in patients who underwent radical resection, and this procedure did not appear to affect adolescent growth and development.Radical resection might be safe and effective for adolescents with pancreatic tumors.
Collapse
|
|
34
|
Ferrari A, Schneider DT, Bisogno G, Orbach D, Villarroel M, Giron V, Rodriguez-Galindo C, Sorbara S, Magni C, Chiaravalli S, Casanova M, Cecchetto G, Godzinski J, Bien E, Stachowicz-Stencel T, Brennan B, Reguerre Y, Sultan I, Brecht IB. The challenge of very rare childhood cancers in developed and developing countries. Expert Opin Orphan Drugs 2017. [DOI: 10.1080/21678707.2017.1298440] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Affiliation(s)
- Andrea Ferrari
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | | | - Gianni Bisogno
- Hematology-Oncology Division, Department of Pediatrics, Padova University Hospital, Padova, Italy
| | - Daniel Orbach
- Department of Pediatrics, Adolescent and Young Adult Oncology, Institut Curie, Paris, France
| | | | - Veronica Giron
- National Pediatric Oncology Unit/Unidad Nacional de Oncologia Pediatrica, Guatemala City, Guatemala
| | | | - Silvia Sorbara
- Hematology-Oncology Division, Department of Pediatrics, Padova University Hospital, Padova, Italy
| | - Chiara Magni
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Stefano Chiaravalli
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Michela Casanova
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Giovanni Cecchetto
- Pediatric Surgery, Department of Pediatrics, Padova University Hospital, Padova, Italy
| | - Jan Godzinski
- Department of Pediatric Surgery, Marciniak Hospital, Wroclaw, Poland
| | - Ewa Bien
- Department of Pediatrics, Medical University, Gdansk, Poland
| | | | - Bernadette Brennan
- Department of Pediatric Oncology, Royal Manchester Children’s Hospital, Manchester, UK
| | - Yves Reguerre
- Pediatric Hematology-Oncology Department, Centre Hospitalier Universitaire, Angers, France
| | - Iyad Sultan
- Department of Pediatric Oncology, King Hussein Cancer Center, Amman, Jordan
| | - Ines B. Brecht
- Pediatric Hematology and Oncology, University of Tuebingen, Tuebingen, Germany
| |
Collapse
|
|
35
|
Irtan S, Galmiche-Rolland L, Elie C, Orbach D, Sauvanet A, Elias D, Guérin F, Coze C, Faure-Conter C, Becmeur F, Demarche M, Galifer RB, Galloy MA, Podevin G, Aubert D, Piolat C, De Lagausie P, Sarnacki S. Recurrence of Solid Pseudopapillary Neoplasms of the Pancreas: Results of a Nationwide Study of Risk Factors and Treatment Modalities. Pediatr Blood Cancer 2016; 63:1515-21. [PMID: 27186826 DOI: 10.1002/pbc.25986] [Citation(s) in RCA: 33] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/24/2015] [Revised: 02/11/2016] [Accepted: 02/26/2016] [Indexed: 12/13/2022]
Abstract
BACKGROUND Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years. MATERIAL AND METHODS Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences. RESULTS Fifty-one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7-17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0-163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow-up of 65 months [0.3-221], the overall and event-free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33-94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03). CONCLUSIONS SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option.
Collapse
Affiliation(s)
- Sabine Irtan
- Department of Pediatric Surgery, Necker Enfants-Malades Hospital-Université Paris Descartes, Sorbonne Paris Cite, Paris, France
| | - Louise Galmiche-Rolland
- Department of Pathology, Necker Enfants-Malades Hospital-Université Paris Descartes, Sorbonne Paris Cite, Paris, France
| | - Caroline Elie
- Clinical Research Unit/Clinical Investigation Centre, Necker Enfants-Malades Hospital, Paris, France
| | - Daniel Orbach
- Department of Pediatrics, Adolescents, Young Adults, Institut Curie, Groupe Fracture, French Rare Pediatric Tumors Group, Paris, France
| | - Alain Sauvanet
- Department of Hepato-Pancreato-Biliary Surgery, Pôle des Maladies de l'Appareil Digestif (PMAD), AP-HP, Hôpital Beaujon, Clichy, France
| | - Dominique Elias
- Department of Surgical Oncology, Gustave Roussy Cancer Campus, Villejuif, France
| | - Florent Guérin
- Department of Pediatric Surgery, Hôpitaux Universitaires Paris-Sud, Bicêtre Hospital, Université Paris XI, Le Kremlin-Bicêtre, France
| | - Carole Coze
- Department of Pediatric Hematology-Oncology, Aix-Marseille Univ and APHM, Hôpital d' Enfants de La Timone, Marseille, France
| | | | - François Becmeur
- Department of Pediatric Surgery, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
| | - Martine Demarche
- Department of Pediatric Surgery, CHR de la Citadelle, Liège, Belgium
| | - René Benoît Galifer
- Department of Pediatric Surgery, Lapeyronie-Arnaud de Villeneuve Hospital, Montpellier, France
| | - Marie Agnès Galloy
- Department of Pediatric Surgery, Children's Hospital Nancy-Brabois University Hospital, Vandoeuvre-lès-Nancy, France
| | - Guillaume Podevin
- Department of Pediatric Surgery, University Hospital, Angers, France
| | - Didier Aubert
- Department of Pediatric Surgery, University Hospital, Besançon, France
| | - Christian Piolat
- Department of Pediatric Surgery, University Hospital, Grenoble, France
| | - Pascal De Lagausie
- Department of Pediatric Surgery, Aix-Marseille Univ and APHM, Hôpital d'Enfants de La Timone, Marseille, France
| | - Sabine Sarnacki
- Department of Pediatric Surgery, Necker Enfants-Malades Hospital-Université Paris Descartes, Sorbonne Paris Cite, Paris, France
| |
Collapse
|
|
36
|
Sacco Casamassima MG, Gause CD, Goldstein SD, Abdullah F, Meoded A, Lukish JR, Wolfgang CL, Cameron J, Hackam DJ, Hruban RH, Colombani PM. Pancreatic surgery for tumors in children and adolescents. Pediatr Surg Int 2016; 32:779-88. [PMID: 27364750 DOI: 10.1007/s00383-016-3925-y] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 06/22/2016] [Indexed: 12/11/2022]
Abstract
PURPOSE Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies. METHODS We conducted a retrospective review of patients ≤21 years with pancreatic neoplasms who underwent surgery at a single institution between 1995 and 2015. RESULTS Nineteen patients were identified with a median age at operation of 16.6 years (IQR 13.5-18.9). The most common histology was solid pseudopapillary neoplasm (SPN) (n = 13), followed by pancreatic neuroendocrine tumor (n = 3), serous cystadenoma (n = 2) and pancreatoblastoma (n = 1). Operative procedures included formal pancreatectomy (n = 17), enucleation (n = 1) and central pancreatectomy (n = 1). SPNs were noninvasive in all but one case with perineural, vascular and lymph node involvement. Seventeen patients (89.5 %) are currently alive and disease free at a median follow-up of 5.7 (IQR 3.7-10.9) years. Two patients died: one with metastatic insulinoma and another with SPN who developed peritoneal carcinomatosis secondary to a concurrent rectal adenocarcinoma. CONCLUSIONS Pediatric pancreatic tumors are a heterogeneous group of neoplastic lesions for which surgery can be curative. SPN is the most common histology, is characterized by low malignant potential and in selected cases can be safely and effectively treated with a tissue-sparing resection and minimally invasive approach.
Collapse
Affiliation(s)
| | - Colin D Gause
- Division of Pediatric Surgery, The Johns Hopkins School of Medicine, Baltimore, MD, USA
| | - Seth D Goldstein
- Division of Pediatric Surgery, The Johns Hopkins School of Medicine, Baltimore, MD, USA
| | - Fizan Abdullah
- Division of Pediatric Surgery, Department of Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA
| | - Avner Meoded
- Division of Pediatric Radiology, The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University, Baltimore, MD, USA
| | - Jeffrey R Lukish
- Division of Pediatric Surgery, The Johns Hopkins School of Medicine, Baltimore, MD, USA
| | - Christopher L Wolfgang
- Department of Surgery, The Sol Goldman Pancreatic Research Center, The Johns Hopkins School of Medicine, Baltimore, MD, USA
| | - John Cameron
- Department of Surgery, The Sol Goldman Pancreatic Research Center, The Johns Hopkins School of Medicine, Baltimore, MD, USA
| | - David J Hackam
- Division of Pediatric Surgery, The Johns Hopkins School of Medicine, Baltimore, MD, USA
| | - Ralph H Hruban
- Department of Pathology, The Sol Goldman Pancreatic Research Center, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
| | - Paul M Colombani
- Department of Surgery, Johns Hopkins All Children's Hospital, 601 5th Street South, Suite 501, Saint Petersburg, FL, 33701, USA.
| |
Collapse
|
|
37
|
A method of reconstruction after pancreaticoduodenectomy for pancreatic malignancies in very young children: Two cases reports. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2016. [DOI: 10.1016/j.epsc.2016.04.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
|
|
38
|
Lachter J, Balanson S, Ilivitzki A. Endoscopic ultrasound-guided fine-needle aspiration for diagnosis of Burkitt's lymphoma in the pancreas of a 3 year old. Dig Endosc 2016; 28:613. [PMID: 26947432 DOI: 10.1111/den.12648] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/08/2016] [Revised: 02/20/2016] [Accepted: 03/02/2016] [Indexed: 02/08/2023]
Affiliation(s)
- Jesse Lachter
- Department of Gastroenterology, Rambam Health Care Campus, Haifa, Israel.,The Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel
| | - Sarit Balanson
- The Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel
| | - Anat Ilivitzki
- Department of Radiology, Rambam Health Care Campus, Haifa, Israel.,The Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel
| |
Collapse
|
|
39
|
Mansfield SA, Mahida JB, Dillhoff M, Porter K, Conwell D, Ranalli M, Walker JP, Aldrink JH. Pancreaticoduodenectomy outcomes in the pediatric, adolescent, and young adult population. J Surg Res 2016; 204:232-6. [DOI: 10.1016/j.jss.2016.04.049] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2016] [Revised: 04/13/2016] [Accepted: 04/20/2016] [Indexed: 02/07/2023]
|
|
40
|
Morikawa M, Nakazawa T, Kato S, Fujimoto D, Koneri K, Murakami M, Hirono Y, Maeda H, Goi T, Katayama K, Imamura Y, Tanizawa A, Yamaguchi A. Acinar cell carcinoma of the pancreas in childhood. Int Cancer Conf J 2016; 5:140-145. [PMID: 31149442 DOI: 10.1007/s13691-016-0245-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/29/2015] [Accepted: 02/24/2016] [Indexed: 12/31/2022] Open
Abstract
A 12-year-old Japanese girl with pancreatic acinar cell carcinoma is presented. She was referred to our hospital with upper abdominal pain on exercise. Computed tomography scan showed a 17 × 17 × 12 cm heterogeneous mass in the right abdominal cavity centering around the pancreatic head to the anterior pararenal space. We performed pylorus-preserving pancreatoduodenectomy, because the tumor invaded the pancreatic head. Macroscopically, the tumor was a 19 × 18 cm, encapsulated mass derived from the pancreatic head without invasion to the surrounding organs, and consisted of solid and cystic portions. Histological examination showed tumor cells proliferating in an acinar pattern and invading the duodenal muscle layer. Immunohistochemically, tumor cells were positive for α1 trypsin and α1 chymotrypsin. From these histological findings, we diagnosed the lesion as an acinar cell carcinoma of the pancreas. We report this case of childhood acinar cell carcinoma, which is extremely rare, with a review of the literature.
Collapse
Affiliation(s)
- Mitsuhiro Morikawa
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| | - Toshiyuki Nakazawa
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| | - Shigeru Kato
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| | - Daisuke Fujimoto
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| | - Kenji Koneri
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| | - Makoto Murakami
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| | - Yasuo Hirono
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| | - Hiroyuki Maeda
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| | - Takanori Goi
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| | - Kanji Katayama
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| | - Yoshiaki Imamura
- 2Department of Pathology, Faculty of Medicine, University of Fukui, Fukui, Japan
| | - Akihiko Tanizawa
- 3Department of Human Resource Development for Cancer Medicine, Faculty of Medicine, University of Fukui, Fukui, Japan
| | - Akio Yamaguchi
- 1First Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Shimoaizuki, Eiheiji-cho, Matsuoka, Yoshida-gun, Fukui, 910-1193 Japan
| |
Collapse
|
|
41
|
Prabhu A, Bhagat M, Ramadwar M, Qureshi SS. Pancreatoblastoma masquerading as hepatoblastoma: A diagnostic dilemma. J Indian Assoc Pediatr Surg 2016; 21:84-6. [PMID: 27046982 PMCID: PMC4790136 DOI: 10.4103/0971-9261.176969] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Clinicoradiological and histopathological differentiation of pancreatoblastoma from hepatoblastoma can often be a challenge in clinical practice owing to their peculiar resemblance. We report a case of a 4-year-old boy with a right hypochondriac region mass, which was diagnosed as hepatoblastoma on the basis of imaging, raised tumor marker, and biopsy; however, pancreatic origin of the mass was ascertained on exploration and pancreatoblastoma was confirmed on histopathology.
Collapse
Affiliation(s)
- Aruna Prabhu
- Department of Pediatric Surgical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
| | - Monica Bhagat
- Department of Pediatric Surgical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
| | - Mukta Ramadwar
- Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India
| | - Sajid S Qureshi
- Department of Pediatric Surgical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
| |
Collapse
|
|
42
|
Natural compounds for pediatric cancer treatment. Naunyn Schmiedebergs Arch Pharmacol 2015; 389:131-49. [DOI: 10.1007/s00210-015-1191-5] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2015] [Accepted: 11/08/2015] [Indexed: 12/13/2022]
|
|
43
|
Pappo AS, Furman WL, Schultz KA, Ferrari A, Helman L, Krailo MD. Rare Tumors in Children: Progress Through Collaboration. J Clin Oncol 2015; 33:3047-54. [PMID: 26304909 DOI: 10.1200/jco.2014.59.3632] [Citation(s) in RCA: 31] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
Rare pediatric tumors account for approximately 10% of all childhood cancers, which in themselves are a rare entity. The diverse histologies and clinical behaviors of rare pediatric tumors pose challenges to the investigation of their biologic and clinical features. National and international cooperative groups such as the Rare Tumor Committee of the Children's Oncology Group, Rare Tumors in Pediatric Age Project, and European Cooperative Study Group for Pediatric Rare Tumors have developed several initiatives to advance knowledge about rare pediatric cancers. However, these programs have been only partially effective, necessitating the development of alternative mechanisms to study these challenging diseases. In this article, we review the current national and international collaborative strategies to study rare pediatric cancers and alternative methods under exploration to enhance those efforts, such as independent registries and disease-specific, National Cancer Institute-sponsored clinics.
Collapse
Affiliation(s)
- Alberto S Pappo
- Alberto S. Pappo and Wayne L. Furman, St Jude Children's Research Hospital, Memphis, TN; Kris A. Schultz, Children's Hospital of Minnesota, Minneapolis, MN; Andrea Ferrari, Instituto Nazionale Tumori, Milano, Italy; Lee Helman, National Cancer Institute Center for Cancer Research, Bethesda, MD; and Mark D. Krailo, Keck School of Medicine, Los Angeles, CA.
| | - Wayne L Furman
- Alberto S. Pappo and Wayne L. Furman, St Jude Children's Research Hospital, Memphis, TN; Kris A. Schultz, Children's Hospital of Minnesota, Minneapolis, MN; Andrea Ferrari, Instituto Nazionale Tumori, Milano, Italy; Lee Helman, National Cancer Institute Center for Cancer Research, Bethesda, MD; and Mark D. Krailo, Keck School of Medicine, Los Angeles, CA
| | - Kris A Schultz
- Alberto S. Pappo and Wayne L. Furman, St Jude Children's Research Hospital, Memphis, TN; Kris A. Schultz, Children's Hospital of Minnesota, Minneapolis, MN; Andrea Ferrari, Instituto Nazionale Tumori, Milano, Italy; Lee Helman, National Cancer Institute Center for Cancer Research, Bethesda, MD; and Mark D. Krailo, Keck School of Medicine, Los Angeles, CA
| | - Andrea Ferrari
- Alberto S. Pappo and Wayne L. Furman, St Jude Children's Research Hospital, Memphis, TN; Kris A. Schultz, Children's Hospital of Minnesota, Minneapolis, MN; Andrea Ferrari, Instituto Nazionale Tumori, Milano, Italy; Lee Helman, National Cancer Institute Center for Cancer Research, Bethesda, MD; and Mark D. Krailo, Keck School of Medicine, Los Angeles, CA
| | - Lee Helman
- Alberto S. Pappo and Wayne L. Furman, St Jude Children's Research Hospital, Memphis, TN; Kris A. Schultz, Children's Hospital of Minnesota, Minneapolis, MN; Andrea Ferrari, Instituto Nazionale Tumori, Milano, Italy; Lee Helman, National Cancer Institute Center for Cancer Research, Bethesda, MD; and Mark D. Krailo, Keck School of Medicine, Los Angeles, CA
| | - Mark D Krailo
- Alberto S. Pappo and Wayne L. Furman, St Jude Children's Research Hospital, Memphis, TN; Kris A. Schultz, Children's Hospital of Minnesota, Minneapolis, MN; Andrea Ferrari, Instituto Nazionale Tumori, Milano, Italy; Lee Helman, National Cancer Institute Center for Cancer Research, Bethesda, MD; and Mark D. Krailo, Keck School of Medicine, Los Angeles, CA
| |
Collapse
|
|
44
|
Response of Relapsed Pancreatoblastoma to a Combination of Vinorelbine and Oral Cyclophosphamide. J Pediatr Hematol Oncol 2015; 37:e378-80. [PMID: 26056794 DOI: 10.1097/mph.0000000000000375] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Pancreatoblastoma is a rare tumor of the pancreas in children, with favorable prognosis if completely resected. If unresectable, neoadjuvant chemotherapy with cisplatin-based regimens are commonly used with good response that allows for resection. For locally aggressive or metastatic disease, neoadjuvant chemotherapy has been reported. Treatment for relapsed or refractory cases is based on anecdotal experiences. We report 2 cases of relapsing pancreatoblastoma with clinical and radiologic response to vinorelbine and cyclophosphamide. Although cure was not achieved, this combination can be offered as an easily tolerated alternative to aggressive chemotherapy for relapsed cases in a palliative setting.
Collapse
|
|
45
|
La Rosa S, Sessa F, Capella C. Acinar Cell Carcinoma of the Pancreas: Overview of Clinicopathologic Features and Insights into the Molecular Pathology. Front Med (Lausanne) 2015; 2:41. [PMID: 26137463 PMCID: PMC4469112 DOI: 10.3389/fmed.2015.00041] [Citation(s) in RCA: 68] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/11/2015] [Accepted: 05/31/2015] [Indexed: 12/14/2022] Open
Abstract
Acinar cell carcinomas (ACCs) of the pancreas are rare pancreatic neoplasms accounting for about 1–2% of pancreatic tumors in adults and about 15% in pediatric subjects. They show different clinical symptoms at presentation, different morphological features, different outcomes, and different molecular alterations. This heterogeneous clinicopathological spectrum may give rise to difficulties in the clinical and pathological diagnosis with consequential therapeutic and prognostic implications. The molecular mechanisms involved in the onset and progression of ACCs are still not completely understood, although in recent years, several attempts have been made to clarify the molecular mechanisms involved in ACC biology. In this paper, we will review the main clinicopathological and molecular features of pancreatic ACCs of both adult and pediatric subjects to give the reader a comprehensive overview of this rare tumor type.
Collapse
Affiliation(s)
- Stefano La Rosa
- Department of Pathology, Ospedale di Circolo , Varese , Italy
| | - Fausto Sessa
- Department of Surgical and Morphological Sciences, University of Insubria , Varese , Italy
| | - Carlo Capella
- Department of Surgical and Morphological Sciences, University of Insubria , Varese , Italy
| |
Collapse
|
|
46
|
The Role of Registries and Tumor Banking in Rare Pediatric Tumors. CURRENT PEDIATRICS REPORTS 2015. [DOI: 10.1007/s40124-015-0077-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
|
|
47
|
Abstract
During recent years, minimally invasive surgery (MIS) has become the standard approach for various operations in infants and children. MIS in pediatric thoracic and abdominal tumors is a controversial approach in the surgical management of childhood cancer. Meanwhile, more and more oncological biopsies and resections are being performed laparoscopically or thoracoscopically. Despite its increasing role in pediatric tumor surgery, the different national and international multicenter trial groups have not yet implemented MIS within guidelines and recommendations in most of the current treatment protocols. An increasing number of retrospective reports describes a potential role of MIS in the management of different pediatric oncological entities. Over the time, there has been a diverse development of this approach with regard to the different neoplasms. Nevertheless, there is a lack of prospective randomized trails assessing MIS. This still represents a requirement for evidence-based medicine and judging the advantages and disadvantages of this approach. The purpose of this state-of-the-art article is to review the current literature to describe the application of MIS in pediatric solid tumors.
Collapse
Affiliation(s)
- Jörg Fuchs
- Department of Pediatric Surgery and Pediatric Urology, University Children's Hospital Tuebingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany,
| |
Collapse
|
|
48
|
Escobar MA, Bond BJ, Schopp J. Solid pseudopapillary tumour (Frantz's tumour) of the pancreas in childhood. BMJ Case Rep 2014; 2014:bcr-2013-200889. [PMID: 24488660 DOI: 10.1136/bcr-2013-200889] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/02/2023] Open
Abstract
An 11-year-old girl presented with acute pancreatitis and mass in the head of the pancreas. MRI revealed a heterogeneous right-upper quadrant retroperitoneal mass measuring 6.8×6.1×5.5 cm arising from the pancreatic head. Endoscopic ultrasound with fine-needle aspirate revealed a solid pseudopapillary tumour (SPT) of the pancreas. The patient underwent a pylorus-preserving Whipple procedure. Pathology confirmed SPT. First described by Frantz, SPT represents less than 3% of all exocrine tumours. It is especially rare in children and shows different clinical features compared with adults. In our patient, tumour cells were arranged at the periphery of fibrovascular cores, but they did not show definite gland formation, keratinisation or cytoplasmic pigment accumulation. A periodic acid-Schiff stain without diastase did not show appreciable glycogen within the tumour cells, classic for Frantz's tumour. The literature, diagnosis, management and pathogenesis on this rare entity in children are reviewed and discussed.
Collapse
Affiliation(s)
- Mauricio Antonio Escobar
- Department of Pediatric Surgical Services, Mary Bridge Children's Hospital & Health Center, Tacoma, Washington, USA
| | | | | |
Collapse
|
|
49
|
Ahmed TS, Chavhan GB, Navarro OM, Traubici J. Imaging features of pancreatic tumors in children: 13-year experience at a pediatric tertiary hospital. Pediatr Radiol 2013; 43:1435-43. [PMID: 23744182 DOI: 10.1007/s00247-013-2721-2] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2013] [Revised: 04/12/2013] [Accepted: 04/19/2013] [Indexed: 11/24/2022]
Abstract
BACKGROUND Pancreatic tumors are rare in children, resulting in limited information regarding their frequency and imaging appearances. OBJECTIVE To review all pancreatic tumors seen in children over a decade at a large tertiary pediatric institution and to describe multimodality imaging findings. MATERIALS AND METHODS We conducted a retrospective review of imaging studies performed in children with pancreatic tumors between January 2000 and December 2012, analyzing imaging features on available US, CT and MR examinations. We reviewed patient charts for clinical features, management and final diagnosis. RESULTS We included 23 children in this study. Of these, 12 had solid and papillary epithelial neoplasms (SPEN), 3 had neuroendocrine tumors, 3 had lymphoma, and 1 each had hemangioendothelioma, benign congenital cysts with adipose tissue, dendritic cell sarcoma, metastatic rhabdomyosarcoma, and lipoma. All children with SPEN were teenage girls. SPEN showed characteristic imaging features including well-defined margins with surrounding capsule, solid-cystic components and hemorrhage. CONCLUSION Pancreatic tumors are uncommon in children. SPEN is the most common tumor and is seen predominantly in teenage girls and shows characteristic imaging features.
Collapse
Affiliation(s)
- Tahiya S Ahmed
- Department of Medical Imaging, University of Toronto, 555 University Ave., Toronto, ON, M5G 1X8, Canada
| | | | | | | |
Collapse
|
|
50
|
Abstract
Neuroblastoma is the most common solid tumor in infancy. Arising from the neural crest these tumors are usually located along the sympathetic chain from the neck to the pelvis and in the adrenal medulla. We report the case of a 3-week-old boy presenting with recurrent episodes of colicky pain. After ultrasound examination, magnetic resonance imaging and laboratory data a pancreatic neuroblastoma was suspected. Tumor resection via distal pancreatectomy and histologic investigation confirmed the diagnosis. Surgery is the treatment of choice in children with pancreatic masses, and is usually well tolerated even in the neonatal period. Conclusive diagnosis can be frequently established only by tumor resection or biopsy. Pancreatic neuroblastoma is an extremely rare tumor with only a few cases described in literature.
Collapse
Affiliation(s)
- Simon Kargl
- Women's and Children's Hospital Linz, Linz, Austria.
| | | | | |
Collapse
|