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Sugita H, Amaya K, Kawata C, Nagaoka Y, Iwaki Y, Nishida Y, Hirose A, Tsukada T, Nakamura T, Hada M, Takeshita M, Yoshikawa A, Kaji M. Robotic Distal Pancreatectomy for Adult Pancreatoblastoma with Tumor Extension to the Main Pancreatic Duct: A Case Report. Surg Case Rep 2025; 11:25-0037. [PMID: 40292021 PMCID: PMC12022996 DOI: 10.70352/scrj.cr.25-0037] [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: 01/23/2025] [Accepted: 03/27/2025] [Indexed: 04/30/2025] Open
Abstract
INTRODUCTION Adult pancreatoblastoma (PB) is an extremely rare malignant pancreatic epithelial tumor. Although no standard treatment strategy has been established, complete resection is recommended for long-term survival. Here, we presented a case of an adult patient with PB who successfully underwent complete resection via robotic surgery. Furthermore, this is the first report of robotic surgery for PB, highlighting its novelty and potential clinical relevance. CASE PRESENTATION A 40-year-old man presented with epigastric pain, and image examination revealed a well-defined tumor in the pancreatic tail extending into the main pancreatic duct (MPD) and reaching the pancreatic neck. With no evidence of distant metastases, surgery was planned following 2 courses of gemcitabine and S-1 chemotherapy for tumor shrinkage. There were no significant changes in the tumor's extension after chemotherapy, but no new lesions appeared, and robotic distal pancreatectomy was performed. Intraoperative findings confirmed the tumor extension into the MPD just above the superior mesenteric vein (SMV). The pancreas was sharply divided at the right edge of the SMV, and a negative transection margin was obtained. The pancreatic stump was closed by suture. The postoperative course was uneventful, and the pathological diagnosis confirmed PB with MPD and inferior mesenteric vein invasion. CONCLUSIONS We successfully resected an adult case of PB with tumor extension into the MPD via robotic surgery. Robotic surgery enabled precise pancreatic transection at the right edge of the SMV, ensuring a negative pancreatic transection margin in this case. In addition, robotic surgery contributed to the safe and secure suture closure of the pancreatic stump.
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Affiliation(s)
- Hiroaki Sugita
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Koji Amaya
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Chihiro Kawata
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Yasuhiro Nagaoka
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Yoshitaka Iwaki
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Yoji Nishida
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Atsushi Hirose
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Tomoya Tsukada
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Takashi Nakamura
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Masahiro Hada
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Masaki Takeshita
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Akemi Yoshikawa
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
| | - Masahide Kaji
- Department of Surgery, Toyama Prefectural Central Hospital, Toyama, Toyama, Japan
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Yin H, Romero-Hernandez F, Ganjouei AA, Wang JJ, Brown A, Hirose K, Maker AV, Nakakura E, Corvera C, Kirkwood KS, Wilhelm A, Peng JS, Alseidi A, Adam MA. Adult Pancreatoblastoma: Clinical Insights and Outcomes Compared to Pancreatic Ductal Adenocarcinoma (PDAC). Curr Oncol 2024; 31:5008-5020. [PMID: 39329998 PMCID: PMC11430823 DOI: 10.3390/curroncol31090370] [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: 07/16/2024] [Revised: 08/20/2024] [Accepted: 08/25/2024] [Indexed: 09/28/2024] Open
Abstract
Pancreatoblastoma is perceived to be aggressive in adults; however, data are limited due to the rarity of the disease. We benchmarked clinico-pathologic characteristics, outcomes, and survival of adult patients with pancreatoblastoma to a comparable PDAC cohort using the National Cancer Database (NCDB). This study included 301,204 patients: 35 with pancreatoblastoma and 301,169 PDAC patients. Pancreatoblastoma patients were younger than PDAC patients (56 vs. 69 years, p < 0.001). More pancreatoblastoma patients were managed at academic institutions (63.0% vs. 40.7%, p = 0.047). The most frequent primary site was the head and the neck of the pancreas. There were no differences in tumor size (4.2 cm vs. 3.7 cm, p = 0.828), lymph node positivity (14.3% vs. 26.4%, p = 0.103), or metastasis at time of diagnosis (31.4% vs. 46.1%, p = 0.081). The majority of pancreatoblastoma patients underwent resection compared to a minority of PDAC patients (69.7% vs. 15.5%, p < 0.001). Time from diagnosis to surgery was longer for pancreatoblastoma patients (33 vs. 14 days, p = 0.030). Pancreaticoduodenectomy was the most common type of resection in the pancreatoblastoma and PDAC groups (47.8% vs. 67.7%, p = 0.124). Among resected patients, pancreatoblastoma patients were less likely to receive radiation (4.8% vs. 37.0%, p = 0.002), but the use of chemotherapy was similar to PDAC patients (60.9% vs. 70.7%). After matching, median overall survival was longer for pancreatoblastoma than PDAC (59.8 months vs. 15.2 months, p = 0.014).
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Affiliation(s)
- Han Yin
- School of Medicine, University of California, San Francisco, CA 94143, USA;
| | | | - Amir Ashraf Ganjouei
- Department of Surgery, University of California, San Francisco, CA 94143, USA; (F.R.-H.)
| | - Jaeyun Jane Wang
- Department of Surgery, University of California, San Francisco, CA 94143, USA; (F.R.-H.)
| | - Audrey Brown
- Department of Surgery, University of California, San Francisco, CA 94143, USA; (F.R.-H.)
| | - Kenzo Hirose
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Ajay V. Maker
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Eric Nakakura
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Carlos Corvera
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Kimberly S. Kirkwood
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Alexander Wilhelm
- Department of Visceral Surgery, Clarunis—University Center for Gastrointestinal and Liver Diseases, St. Clara Hospital and University Hospital Basel, 4058 Basel, Switzerland
| | - June S. Peng
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Adnan Alseidi
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Mohamed A. Adam
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
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Yuan J, Guo Y, Li Y. Diagnosis, treatment, and prognosis of adult pancreatoblastoma. Cancer Med 2024; 13:e70132. [PMID: 39162366 PMCID: PMC11334167 DOI: 10.1002/cam4.70132] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2024] [Revised: 07/24/2024] [Accepted: 08/09/2024] [Indexed: 08/21/2024] Open
Abstract
BACKGROUND Pancreatoblastoma (PB) is one of the rare malignant tumors that typically occurs in children. Cases of PB in adults are highly unusual. This disease often presents with subtle symptoms and lacks characteristic clinical manifestations, leading to diagnostic challenges. OBJECTIVE This study integrates the relevant literature on adult PB, conducting data analysis on clinical features, laboratory and imaging results, pathological characteristics, and treatments according to inclusion and exclusion criteria. Kaplan-Meier univariate analysis and Log-rank tests are employed to analyze survival data from adult PB follow-up, exploring factors influencing prognosis. RESULTS A total of 65 articles were included, encompassing 103 cases of adult PB. The average age of PB patients was 41.78 years (range 19-81 years), and the male-to-female ratio was 1.06:1. Patients frequently presented with abdominal pain as the initial symptom. Laboratory results lacked specificity and imaging findings often presented as large, well-defined masses. PB exhibited distinctive pathological features, including squamous corpuscles (n = 76, 89.41%) and acinar differentiation (n = 34, 40%), with frequent positive expression of Trypsin, Chymotrypsin, and AACT (Alpha-1-Antichymotrypsin). APC (Adenomatous Polyposis Coli) gene mutation was the most common molecular alteration in adult PB. During the follow-up period, 43.59% of patients died (range 3 days to 348 months). The primary factors affecting prognosis were the presence of metastasis (χ2 = 3.996, p = 0.046) and incomplete surgical resection (χ2 = 5.586, p = 0.018), with mean survival times of 48 months and 27 months, respectively. CONCLUSIONS PB in adults is an invasive tumor. The key to distinguishing PB from other pancreatic tumors lies in recognizing its unique pathological feature, the squamous corpuscles. Timely and complete surgical resection is the preferred treatment following diagnosis. Patients with incomplete resection or the presence of lymph nodes or (and) distant metastases have a poor prognosis.
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Affiliation(s)
- Jiaqian Yuan
- The First Clinical Medical CollegeZhejiang Chinese Medical UniversityHangzhouChina
| | - Yong Guo
- The First Clinical Medical CollegeZhejiang Chinese Medical UniversityHangzhouChina
- Department of Medical OncologyThe First Affiliated Hospital of Zhejiang Chinese Medical UniversityHangzhouChina
| | - Yan Li
- The First Clinical Medical CollegeZhejiang Chinese Medical UniversityHangzhouChina
- Department of Medical OncologyThe First Affiliated Hospital of Zhejiang Chinese Medical UniversityHangzhouChina
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Liu Y, El Jabbour T, Somma J, Nakanishi Y, Ligato S, Lee H, Fu ZY. Blastomas of the digestive system in adults: A review. World J Gastrointest Surg 2024; 16:1030-1042. [PMID: 38690053 PMCID: PMC11056657 DOI: 10.4240/wjgs.v16.i4.1030] [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: 01/01/2024] [Revised: 02/03/2024] [Accepted: 03/25/2024] [Indexed: 04/22/2024] Open
Abstract
Blastomas, characterized by a mixture of mesenchymal, epithelial, and undifferentiated blastematous components, are rare malignant neoplasms originating from precursor blast cells. This review focuses on digestive system blastomas in adult patients, including gastroblastoma, hepatoblastoma, and pancreatoblastoma. Gastroblastoma is a biphasic, epitheliomesenchymal tumor, with only sixteen cases reported to date. In addition to the characteristic histology, metastasis-associated lung adenocarcinoma transcript 1 - glioma-associated oncogene homolog 1 gene fusion is typical, although recently novel ewing sarcoma breakpoint region 1 - c-terminal binding protein 1 and patched 1 - glioma-associated oncogene homolog 2 fusions have been described. Hepatoblastoma is exceptionally rare in adults and can show a variety of histologic patterns which may cause diagnostic difficulty. Pancreatoblastoma, primarily a pediatric tumor, displays acinar differentiation and squamoid nests with other lines of differentiation also present, especially neuroendocrine. Diagnostic approaches for these blastomas include a combination of imaging modalities, histopathological examination, and molecular profiling. The treatment generally involves surgical resection, which may be supplemented by chemotherapy or radiotherapy in some cases. Prognoses vary with gastroblastoma generally showing favorable outcomes post-surgery whereas hepatoblastoma and pancreatoblastoma often have poorer outcomes, particularly in the setting of metastases. This review highlights the complexity of diagnosing and managing these rare adult blastomas as well as the need for ongoing research to better understand their pathogenesis and improve treatment strategies.
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Affiliation(s)
- Yu Liu
- Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States
| | - Tony El Jabbour
- Department of Pathology, Hartford HealthCare, Hartford, CT 06102, United States
| | - Jonathan Somma
- Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States
| | - Yukihiro Nakanishi
- Department of Pathology, Moffitt Cancer Center, Tampa, FL 33612, United States
| | - Saverio Ligato
- Department of Pathology, Hartford HealthCare, Hartford, CT 06102, United States
| | - Hwajeong Lee
- Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY 12208, United States
| | - Zhi-Yan Fu
- Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States
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Hussain A, Farrukh J. Adult Pancreatoblastoma: An Uncommon Pancreatic Malignancy. Cureus 2023; 15:e48063. [PMID: 38046486 PMCID: PMC10689026 DOI: 10.7759/cureus.48063] [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] [Accepted: 10/31/2023] [Indexed: 12/05/2023] Open
Abstract
In this report we present a rare case of pancreatoblastoma in an adult patient. Whilst they are amongst the most common malignant pancreatic tumours in children, presentations in adults are exceedingly rare, with a small number of reported cases. Its presentation is often non-specific in terms of clinical examination, and subsequent imaging can show similar findings to those seen in benign neoplasms. This report highlights the difficulty of achieving a diagnosis and subsequent treatment of such an uncommon disease. Biopsy and resultant histology are essential in diagnosis and surgical resection remains the preferred modality of treatment. However, the use of chemotherapy and its efficacy in adults remains unclear, and the prognosis documented in existing literature for adults is worse when compared to paediatric presentations. This case emphasises the need to consider pancreatoblastoma as a differential diagnosis when suspecting pancreatic or abdominal malignancies to achieve early detection and diagnosis, in order to provide optimal treatment and improve patient outcomes.
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Affiliation(s)
- Aroosh Hussain
- Radiology, Royal Stoke University Hospital, Stoke-On-Trent, GBR
| | - Jawaad Farrukh
- Radiology, Royal Stoke University Hospital, Stoke-On-Trent, GBR
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Machado I, López-Guerrero JA, Fernandez A, López R, García Casado Z, Ferrandez A, Llombart-Bosch A, Charville GW. Adult Pancreatoblastoma: Report of 3 new Cases With Genetic Diversity and Autopsy Findings. Int J Surg Pathol 2022:10668969221133351. [PMID: 36573045 DOI: 10.1177/10668969221133351] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
We report the histopathological, immunohistochemical (IHC), and molecular findings in 3 patients with adult pancreatoblastoma, including 2 with autopsy features. The tumors were located in the tail and body of the pancreas, and the 2 autopsy examinations revealed liver and lung metastases. Histopathologically the neoplasms were composed of solid epithelial elements with nested or trabecular growth patterns, fibrous stroma, and squamoid clusters. Keratin 19 was positive mainly in squamoid corpuscles, and trypsin or chymotrypsin was positive in the acinar component. Neuroendocrine differentiation was observed in all tumors, and nuclear β-catenin expression in 2 tumors. Despite nuclear β-catenin expression, CTNNB1 mutation was found only in tumor 2. APC mutation was detected in tumor 1, and SMAD4 as well as MEN1 mutations in tumor 3. This last tumor also revealed chromosomal instability with many chromosomal losses and gains. The follow-up showed regional or distant metastases in all patients. Two patients died of disease after 3 and 26 months of follow-up and 1 patient is alive with no evidence of disease 6 years and 2 months after surgery. Adult pancreatoblastoma can display genetic heterogeneity, diverse histological appearance, and overlapping IHC findings. As a result, the differential diagnosis with other adult pancreatic tumors, such as acinar cell carcinoma, neuroendocrine neoplasm, solid pseudopapillary neoplasm, and mixed tumors may be challenging, especially when dealing with limited tumor tissue. The identification of squamoid corpuscles is essential for diagnosis. Although molecular findings might provide useful information, the integration of clinical, radiological, and histopathological findings is essential in pancreatoblastoma diagnosis.
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Affiliation(s)
- Isidro Machado
- Department of Pathology, Instituto Valenciano de Oncología, Valencia, Spain
- Patologika Laboratory, Hospital QuirónSalud, Valencia, Spain
- Department of Pathology, University of Valencia, Valencia, Spain
| | | | | | - Raquel López
- Department of Molecular Biology, Instituto Valenciano de Oncología, Valencia, Spain
| | - Zaida García Casado
- Department of Molecular Biology, Instituto Valenciano de Oncología, Valencia, Spain
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Omiyale AO. Adult pancreatoblastoma: Current concepts in pathology. World J Gastroenterol 2021; 27:4172-4181. [PMID: 34326617 PMCID: PMC8311526 DOI: 10.3748/wjg.v27.i26.4172] [Citation(s) in RCA: 19] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/30/2021] [Revised: 03/23/2021] [Accepted: 06/22/2021] [Indexed: 02/06/2023] Open
Abstract
Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas, which may pose diagnostic difficulties. Because of its rarity, little is known about its clinical and pathologic features. This article reviews the clinical and pathologic features of pancreatoblastoma in adults including differential diagnosis, treatment, and follow-up. Although pancreatoblastoma commonly occurs in childhood, there have now been more than 70 adult pancreatoblastomas described in the literature. There is a slight male predominance. There are no symptoms unique to pancreatoblastomas and adult patients are frequently symptomatic. The most common presenting symptom is abdominal pain. Grossly, the tumours are often large and well-circumscribed. Microscopically, pancreatoblastomas are composed of neoplastic cells with predominantly acinar differentiation and characteristic squamoid nests. These tumours are positive for trypsin, chymotrypsin, lipase, and BCL10. Loss of heterozygosity on chromosome 11p is the most common molecular alteration in pancreatoblastomas. Adult pancreatoblastomas are aggressive tumours with frequent local invasion, recurrence, and distant metastasis. Treatment consists of surgical resection. Chemotherapy and radiotherapy may have a role in the treatment of recurrent, residual, unresectable, and metastatic disease. It is important to distinguish pancreatoblastomas from morphological mimics such as acinar cell carcinomas, solid pseudopapillary neoplasms, and pancreatic neuroendocrine neoplasms.
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Affiliation(s)
- Ayo O Omiyale
- Department of Cellular Pathology, Imperial College Healthcare NHS Trust, London W6 8RF, United Kingdom
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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.
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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
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Elghawy O, Wang JS, Whitehair RM, Grosh W, Kindwall-Keller TL. Successful treatment of metastatic pancreatoblastoma in an adult with autologous hematopoietic cell transplant. Pancreatology 2021; 21:188-191. [PMID: 33199137 DOI: 10.1016/j.pan.2020.10.049] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/30/2020] [Revised: 10/18/2020] [Accepted: 10/27/2020] [Indexed: 02/07/2023]
Abstract
BACKGROUND Pancreatoblastoma is a rare pediatric malignant neoplasm characterized by its histological resemblance to fetal pancreatic tissue and poor clinical outcomes. Preoperative diagnosis of the neoplasm is difficult due to its rarity, variable clinical presentation, and its lack of distinct laboratory markers. Current mainstay of treatment is surgical resection of the tumor, although a standard of care has not yet been established. METHODS Data were collected on one patient admitted to the University of Virginia Hospital System. Radiology, hematopoietic cell transplant, and biopsy data were collected according to the best clinical practice. RESULTS Herein, we describe the case of an adult patient with pancreatoblastoma treated with high-dose chemotherapy and autologous peripheral blood hematopoietic cell transplantation. To the authors' knowledge, this is the first documented successful treatment of pancreatoblastoma using autologous hematopoietic cell transplantation in the United States, and the first successful treatment in an adult patient worldwide. CONCLUSIONS While it is difficult to draw conclusions based on a single case, we would like to highlight the success of this treatment modality in the management of our patient with a 51-month remission and open further discussion into exploring the use of autologous hematopoietic cell transplantation for pancreatoblastoma. Our patient is currently living 57 months after diagnosis despite the average survival rate being less than 18 months.
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Affiliation(s)
- Omar Elghawy
- School of Medicine, University of Virginia, Charlottesville, VA, USA
| | - John S Wang
- School of Medicine, University of Virginia, Charlottesville, VA, USA
| | - Rachel M Whitehair
- University of Virginia, Department of Pathology, Charlottesville, VA, USA
| | - William Grosh
- University of Virginia, Division of Hematology and Oncology, Charlottesville, VA, USA
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10
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Zhang X, Ni SJ, Wang XH, Huang D, Tang W. Adult pancreatoblastoma: clinical features and Imaging findings. Sci Rep 2020; 10:11285. [PMID: 32647222 PMCID: PMC7347875 DOI: 10.1038/s41598-020-68083-2] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2019] [Accepted: 06/16/2020] [Indexed: 12/17/2022] Open
Abstract
The objective of this study was to illustrate the clinical, CT, MRI, and 18F-FDG PET/CT features of adult pancreatoblastoma, an extremely rare disease. In this study, the clinical and imaging features of seven adult patients with pathologically confirmed pancreatoblastoma were retrospectively analyzed. The following parameters were evaluated: size, location, shape, margination, solid-cystic ratio, CT attenuation values or signal intensity and contrast enhancement pattern. We also analyzed whether abnormal FDG uptake occurred during 18F-FDG PET/CT imaging. All seven patients were male (mean age 45 years; range 22–65 years). Six tumors were irregular in shape, exogenous, and grew outward from the pancreatic parenchyma, similar to branches growing from a tree trunk (85.7%). The tumor margins were clear in five patients (71.4%), and three tumors (42.9%) were encapsulated. Six tumors (71.4%) were solid, with homogeneous enhancement observed on contrast-enhanced CT and MRI. Dynamic-enhanced CT and MRI showed progressive enhancement for all tumors. On 18F-FDG PET/CT, one tumor exhibited abnormal FDG uptake, and two tumors exhibited no abnormal uptake (66.7%). In conclusion, adult pancreatoblastoma most commonly occurs in male patients, and it usually appears as an exophytic, irregular, and hypovascular mass with well-defined margins and progressive enhancement on CT and MRI. This type of tumor always grows out of the parenchyma of the pancreas, similar to branches growing outward from a tree trunk.
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Affiliation(s)
- Xi Zhang
- Department of Radiology, Fudan University Shanghai Cancer Center, 270 Dongan Road, Shanghai, 200032, China. .,Department of Oncology, Shanghai Medical College of Fudan University, Shanghai, China.
| | - Shu-Juan Ni
- Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, China.,Department of Oncology, Shanghai Medical College of Fudan University, Shanghai, China
| | - Xiao-Hong Wang
- Department of Radiology, Fudan University Shanghai Cancer Center, 270 Dongan Road, Shanghai, 200032, China.,Department of Oncology, Shanghai Medical College of Fudan University, Shanghai, China
| | - Dan Huang
- Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, China.,Department of Oncology, Shanghai Medical College of Fudan University, Shanghai, China
| | - Wei Tang
- Department of Radiology, Fudan University Shanghai Cancer Center, 270 Dongan Road, Shanghai, 200032, China.,Department of Oncology, Shanghai Medical College of Fudan University, Shanghai, China
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12
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Adult Pancreatoblastoma With Liver Metastasis Diagnosed by 18F-FDG PET/CT and 2 Years' Postoperative Follow-up. Clin Nucl Med 2020; 45:e24-e28. [PMID: 31274558 DOI: 10.1097/rlu.0000000000002684] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Pancreatoblastoma is an extremely rare malignant tumor of the pancreas. Most patients are infants and children; however, a few cases have been reported in adults. Herein, we present the case of a 49-year-old man with pancreatoblastoma and liver metastasis who underwent preoperative multimodal imaging, in which F-FDG PET/CT showed a markedly increased F-FDG uptake in the metastatic lesion and a slightly increased uptake in the primary lesion. Subsequently, the patient underwent complete resection of the primary and metastatic lesions. No recurrences or metastases were found in 2 years of follow-up.
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Reid MD, Bhattarai S, Graham RP, Pehlivanoglu B, Sigel CS, Shi J, Saqi A, Shirazi M, Xue Y, Basturk O, Adsay V. Pancreatoblastoma: Cytologic and histologic analysis of 12 adult cases reveals helpful criteria in their diagnosis and distinction from common mimics. Cancer Cytopathol 2019; 127:708-719. [PMID: 31581358 PMCID: PMC7484954 DOI: 10.1002/cncy.22187] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2019] [Accepted: 08/28/2019] [Indexed: 12/16/2022]
Abstract
BACKGROUND Pancreatoblastoma (PBL) is a rare malignant pancreatic tumor seen predominantly in childhood, and its cytologic diagnosis remains challenging. METHODS Twelve fine-needle-aspirations from 11 adults were analyzed. RESULTS In total, 6 men and 5 women (median age, 45 years; age range, 32-60 years) had tumors measuring a median 5.6 cm (range, 2.5-12 cm) located in the pancreatic head (n = 7) or tail (n = 4), including 3 with familial adenomatous polyposis (FAP)/FAP-related syndromes and 4 with metastasis at diagnosis. The median follow-up was 39.8 months (range, 0.8-348 months), and 5 patients died of disease. The original cytology diagnoses were: PBL (n = 2), neuroendocrine neoplasm (n = 2), poorly differentiated neuroendocrine carcinoma (n = 2), well differentiated neuroendocrine tumor (n = 1), poorly differentiated carcinoma (n = 2), "positive for malignancy" (n = 1), acinar cell carcinoma (n = 1), and epithelioid neoplasm with endocrine and acinar differentiation versus PBL (n = 1). Universal cytopathologic findings included hypercellularity; 3-dimensional clusters; and single, monotonous, blast-like cells that were from 1.5 to 2.0 times the size of red blood cells with high nuclear-to-cytoplasmic ratio, fine chromatin, small, distinct nucleoli, and a resemblance to well differentiated neuroendocrine tumor and poorly differentiated neuroendocrine carcinoma. Branching pseudopapillae (n = 7) and grooved nuclei (n = 3) raised the differential diagnosis of solid-pseudopapillary neoplasm, but with more atypia. Uncommon features included pleomorphism (n = 4) and numerous mitoses (n = 1). Squamoid morules were seen on smears (n = 5) or cell blocks (n = 6) in 70% of patients and were characterized by epithelioid cells with elongated, streaming nuclei, fine chromatin, absent nucleoli, and positive nuclear β-catenin (n = 6 of 8). The median Ki-67 index was 21% (range, 2%-70%), and neuroendocrine marker expression was common (100%), but acinar markers were variable (63%). CONCLUSIONS A combination of cytologic findings in PBL, including a predominant population of primitive blast-like cells, subtle squamoid morules, frequent neuroendocrine and variable acinar phenotype, should facilitate accurate cytologic diagnosis and distinction from common mimics.
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Affiliation(s)
- Michelle D. Reid
- Department of Pathology, Emory University Hospital, Atlanta, Georgia
| | | | | | | | - Carlie S. Sigel
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York
| | - Jiaqi Shi
- Department of Pathology, University of Michigan, Ann Harbor, Michigan
| | - Anjali Saqi
- Department of Pathology, Columbia University Medical Center, New York, New York
| | - Maryam Shirazi
- Department of Pathology, Columbia University Medical Center, New York, New York
| | - Yue Xue
- Department of Pathology, Emory University Hospital, Atlanta, Georgia
| | - Olca Basturk
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York
| | - Volkan Adsay
- Department of Pathology, Koç University Hospital, Istanbul, Turkey
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Terino M, Plotkin E, Karagozian R. Pancreatoblastoma: an Atypical Presentation and a Literature Review. J Gastrointest Cancer 2019; 49:361-364. [PMID: 28194556 DOI: 10.1007/s12029-017-9925-x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Affiliation(s)
- Marisa Terino
- Department of Internal Medicine, University of Connecticut Health Center, 263 Farmington Ave, Farmington, CT, 06030, USA.
| | - Eileen Plotkin
- Department of Gastroenterology and Hepatology, University of Connecticut Health Center, 263 Farmington Ave, Farmington, CT, 06030, USA
| | - Raffi Karagozian
- Department of Gastroenterology and Hepatology, St. Francis Hospital and Medical Center, 114 Woodland St, Hartford, CT, 06105, USA
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Complete Surgical Resection and Aggressive Treatment for Liver Metastasis May Be Beneficial to Adult Patients With Pancreatoblastoma: A Case Report and Review of Literature. Int Surg 2019. [DOI: 10.9738/intsurg-d-19-00006.1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
Introduction:
Pancreatoblastoma (PB) is a rare pancreatic neoplasm that occurs most in pediatric patients. Here, we report a rare case of adult PB with liver metastasis and review the literature in order to assist clinicians in the management of the disease.
Case Presentation:
A 27-year-old female patient suffered from postprandial fullness, anorexia, and weight loss in the past 3 months. An abdominal ultrasound and contrast-enhanced computed tomography scans confirmed right abdominal mass with compression of major liver vessels, as well as the P-duct and biliary ducts and causing mild dilatation. Pancreatoduodenectomy was performed and pathologic findings showed typical squamoid corpuscles, which confirmed the diagnosis of PB. The patient was alive and disease-free for 1 year and 10 months until a new metastatic lesion was found. Radiofrequency ablation was arranged as a curative treatment, and no viable tumor or sign of recurrence was found until this paper was submitted. Based on a review of previous case reports, we found adult PB patients with only liver metastasis presented with a smaller tumor size (P = 0.031), more frequent pancreatic head origin (P = 0.043), and decreased 1-year mortality (P = 0.009) compared with patients with other distal metastases. Therefore, we assumed that PB with liver metastasis might present favorable outcome by complete surgical resection or other curative treatment.
Conclusion:
PB patients with liver metastasis are more likely to show a pancreatic head origin, smaller tumor size, and more favorable outcomes compared with other sites of metastasis. PB should be treated aggressively with surgical resection or other curative treatment as opposed to chemotherapy alone.
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Brunetti O, Aprile G, Marchetti P, Vasile E, Casadei Gardini A, Scartozzi M, Barni S, Delfanti S, De Vita F, Di Costanzo F, Milella M, Cella CA, Berardi R, Cataldo I, Scarpa A, Basile D, Mazzuca F, Graziano G, Argentiero A, Santini D, Reni M, Cascinu S, Silvestris N. Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors: A Retrospective Multicenter Analysis. Pancreas 2018; 47:759-771. [PMID: 29771769 DOI: 10.1097/mpa.0000000000001063] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
OBJECTIVES Two issues were put forth by clinicians in the management of the advanced stages of rare variants of pancreatic ductal adenocarcinoma and other exocrine histotypes with peculiar clinical and pathological features: Do chemotherapy regimens recommended in pancreatic ductal adenocarcinoma patients have a clinical activity in rare pancreatic tumors? Or should other chemotherapy combinations be considered in this subset of patients? METHODS We conducted a multicenter retrospective study that collected data from 2005 to 2016 at 14 Italian cancer centers with the aim to evaluate tumor response and time to progression for first- and second-line and overall survival. RESULTS Of approximately 4300 exocrine pancreatic cancer patients, 79 advanced cases affected by rare histological types were identified, with pancreatic acinar cell cancer (n = 23), pancreatic adenosquamous cancer (n = 16), and mucinous cystic neoplasm with an associated invasive mucinous cystadenocarcinoma (n = 15) most represented. Survival analyses for each subgroup in relation with the different chemotherapy regimens showed the lack of statistical significance correlations. CONCLUSIONS Because of the lack of clinical trials in patients affected by these rare pancreatic histotypes, only their molecular classification would help clinicians in future therapeutic choice.
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MESH Headings
- Adenocarcinoma, Mucinous/drug therapy
- Adenocarcinoma, Mucinous/pathology
- Aged
- Antineoplastic Combined Chemotherapy Protocols/therapeutic use
- Carcinoma, Acinar Cell/drug therapy
- Carcinoma, Acinar Cell/pathology
- Carcinoma, Adenosquamous/drug therapy
- Carcinoma, Adenosquamous/pathology
- Carcinoma, Pancreatic Ductal/drug therapy
- Carcinoma, Pancreatic Ductal/pathology
- Cystadenocarcinoma, Mucinous/drug therapy
- Cystadenocarcinoma, Mucinous/pathology
- Humans
- Kaplan-Meier Estimate
- Middle Aged
- Pancreatic Neoplasms/drug therapy
- Pancreatic Neoplasms/pathology
- Prognosis
- Retrospective Studies
- Treatment Outcome
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Affiliation(s)
| | | | - Paolo Marchetti
- Medical Oncology Unit, Sant'Andrea Hospital, University of Rome La Sapienza, Rome
| | - Enrico Vasile
- Medical Oncology Unit, Azienda Ospedaliero-Universitaria Pisana, Pisa
| | - Andrea Casadei Gardini
- Department of MedicalOncology, Istituto Scientifico Romagnolo per lo Studio e Cura dei Tumori (IRST) IRCCS, Meldola
| | | | - Sandro Barni
- Medical Oncology Unit, ASST Bergamo Ovest, Treviglio
| | - Sara Delfanti
- Medical Oncology Unit, Fondazione IRCCS Policlinico San Matteo, Pavia
| | | | | | - Michele Milella
- Medical Oncology 1, IRCCS Regina Elena National Cancer Institute, Rome
| | | | - Rossana Berardi
- Medical Oncology Unit, Università Politecnica Marche - Ospedali Riuniti Ancona, Ancona
| | - Ivana Cataldo
- Department of Pathology and Diagnostics, University of Verona, ARCNET, Verona
| | - Aldo Scarpa
- Department of Pathology and Diagnostics, University of Verona, ARCNET, Verona
| | - Debora Basile
- Department of Medical Oncology, University and General Hospital, Udine
| | - Federica Mazzuca
- Medical Oncology Unit, Sant'Andrea Hospital, University of Rome La Sapienza, Rome
| | - Giusi Graziano
- Scientific Direction, Cancer Institute "Giovanni Paolo II," Bari
| | | | | | - Michele Reni
- Department of Medical Oncology, IRCCS San Raffaele Scientific Institute, Milan
| | - Stefano Cascinu
- Modena Cancer Center, University of Modena and Reggio Emilia, Azienda Ospedaliera-Universitaria di Modena, Modena, Italy
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17
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Chen M, Zhang H, Hu Y, Liu K, Deng Y, Yu Y, Wu Y, Qi A, Li Y, Wen G. Adult pancreatoblastoma: A case report and clinicopathological review of the literature. Clin Imaging 2018; 50:324-329. [PMID: 29753278 DOI: 10.1016/j.clinimag.2018.05.001] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2018] [Revised: 04/27/2018] [Accepted: 05/01/2018] [Indexed: 02/06/2023]
Abstract
PURPOSE Our purpose was to report a case of adult pancreatoblastoma, and review the literature in order to assist clinicians in the management of the disease. MATERIALS AND METHODS The demographic, clinical, and imaging findings of 41 patients with pathologically proven pancreatoblastoma from 1986 to 2017 identified in PubMed were reviewed. The key words used for searching PubMed were: "pancreatoblastoma", "pancreatic tumor", and "adult pancreatoblastoma." We also reported the details of a case of adult pancreatoblastoma treated at our institution. RESULTS We identified 41 cases of adult pancreatoblastomas, and the mean age at diagnosis was 41.4 ± 17.4 years. Pancreatoblastomas occurred in the pancreatic head in 48.4% of patients, and in 39.0% of cases the tumor was >8 cm in diameter at diagnosis. Patient age and tumor size were similar between males and females (P = 0.59; P = 0.32, respectively). Metastases was present in 17 of the 41 adult patients (41.5%). No significant difference in age, sex, tumor size, and tumor location was found between patients with and without metastases (P = 0.57, 0.58, 0.64, 0.39, respectively). CONCLUSION Preoperative diagnosis of adult pancreatoblastoma is difficult because of the heterogeneous, variable cellular differentiation and atypical clinical and imaging features. A pancreatoblastoma should be considered when tumors in the pancreas are solid and cystic.
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Affiliation(s)
- Menglin Chen
- Medical Imaging Teaching and Research Office, Southern Medical University, Guangzhou 510515, Guangdong, China
| | - Haijie Zhang
- Medical Imaging Teaching and Research Office, Southern Medical University, Guangzhou 510515, Guangdong, China
| | - Yixin Hu
- Medical Imaging Teaching and Research Office, Southern Medical University, Guangzhou 510515, Guangdong, China
| | - Kai Liu
- Medical Imaging Teaching and Research Office, Southern Medical University, Guangzhou 510515, Guangdong, China
| | - Yanjia Deng
- Medical Imaging Teaching and Research Office, Southern Medical University, Guangzhou 510515, Guangdong, China
| | - Yuanmeng Yu
- Department of Medical Imaging, Jinling Hospital, Nanjing University Medical School, Nanjing 210002, Jiangsu, China
| | - Yulan Wu
- Medical Imaging Teaching and Research Office, Southern Medical University, Guangzhou 510515, Guangdong, China
| | - Anqi Qi
- Medical Imaging Teaching and Research Office, Southern Medical University, Guangzhou 510515, Guangdong, China
| | - Yingjia Li
- Department of Ultrasound, Nanfang Hospital, Southern Medical University, Guangzhou 510515, Guangdong, China.
| | - Ge Wen
- Medical Imaging Teaching and Research Office, Southern Medical University, Guangzhou 510515, Guangdong, China.
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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.
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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.
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Pancreatoblastoma: an unusual diagnosis in an adult patient. Clin J Gastroenterol 2017; 11:161-166. [PMID: 29285688 DOI: 10.1007/s12328-017-0812-6] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/06/2017] [Accepted: 12/19/2017] [Indexed: 12/20/2022]
Abstract
The authors describe a 31-year-old man admitted due to progressive weight loss, diarrhea and massive hepatomegaly. Laboratory data showed anemia (haemoglobin 11.7 g/dl), abnormal liver tests (total bilirubin 1.4 g/dl, aspartate aminotransferase 70 U/l, alanine aminotransferase 37 U/l and alkaline phosphatase 520 U/l). Abdominal ultrasound (US) displayed a large heterogeneous liver with a segment IV 25 mm nodule. Magnetic resonance revealed a 4 cm pancreatic tail mass and several liver nodules consistent with metastasis. The patient underwent an endoscopic ultrasound (EUS) with fine needle aspiration (FNA) from the pancreatic mass and liver metastasis with cytological evaluation consistent with a pancreatoblastoma, later confirmed through a percutaneous US-guided liver biopsy. During the inpatient period, liver function deteriorated and acute kidney injury developed. Severe progressive cachexia was observed. The patient was discharged on renal replacement therapy and palliative care. Death occurred 3 months after diagnosis. Pancreatoblastoma is an uncommon pancreatic malignant epithelial cancer of the pancreas, typically occurring in the paediatric population. Adult pancreatoblastoma is extremely rare, with about 40 cases reported in the literature and generally presenting a more aggressive biologic and clinical behaviour. Surgical resection is the treatment of choice, but most cases are detected in advanced stages. This case underlines the ability to establish a pancreatoblastoma cytology-based diagnosis with EUS-FNA, and confirms the associated poor outcome.
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Pancreatic Calcifications and Calcified Pancreatic Masses: Pattern Recognition Approach on CT. AJR Am J Roentgenol 2017; 209:77-87. [DOI: 10.2214/ajr.17.17862] [Citation(s) in RCA: 29] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
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Abstract
Nonductal pancreatic neoplasms, including solid pseudopapillary neoplasms, acinar cell carcinomas, and pancreatoblastomas, are uncommon. These entities share overlapping gross, microscopic, and immunohistochemical features, such as well-demarcated solid neoplasms, monotonous cellular tumor cells with little intervening stroma, and abnormal beta-catenin expression. Each tumor also has unique clinicopathologic characteristics with diverse clinical behavior. To differentiate nonductal pancreatic neoplasms, identification of histologic findings, such as pseudopapillae, acinar cell features, and squamoid corpuscles, is important. Immunostainings for acinar cell or neuroendocrine markers are helpful for differential diagnosis. This article describes the clinicopathologic and immunohistochemical features of nonductal pancreatic cancers.
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