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1
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Wu M, Lin J, Liu Z, Huang Z, Wang R. CT, MRI, and 18F-FDG PET/CT imaging features of seven cases of adult pancreatoblastoma. BMC Med Imaging 2022; 22:228. [PMID: 36581821 PMCID: PMC9798564 DOI: 10.1186/s12880-022-00958-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2022] [Accepted: 12/22/2022] [Indexed: 12/31/2022] Open
Abstract
OBJECTIVE This study mainly analysed the imaging data for seven cases of adult pancreatoblastoma (PB) and summarized additional imaging features of this disease based on a literature review, aiming to improve the understanding and diagnosis rate of this disease. MATERIALS AND METHODS The imaging data for seven adult patients pathologically diagnosed with adult PB were retrospectively analysed. Among the seven patients, six underwent computed tomography (CT) scans, two patients underwent abdominal magnetic resonance imaging (MRI), and five patients underwent 18F-FDG PET/CT. RESULTS The tumours were located in the head of the pancreas in three cases, in the tail of the pancreas in two cases, and in the gastric antrum and neck of the pancreas in one case. Six tumours showed blurred edges, and an incomplete envelope was observed in only two cases when enhanced, which showed extruded growth and cyst-solid masses; one tumour was a solid mass with ossification. Showing mild or significant enhancement in the arterial phase (AP) for six cases. In the MRI sequence, isointensity was found on suppressed T1-weighted imaging, and hyperintensity was noted on suppressed T2-weighted imaging in two cases, with significant enhancement. Pancreatic duct dilatation was found in four cases. Tumour 18F-FDG PET/CT imaging exhibited high uptake in five cases. CONCLUSION Adult PB involves a single tumour and commonly manifests as cystic-solid masses with blurred edges. Capsules are rare, ossification is an important feature, tumours can also present in ectopic pancreatic tissues, with mild or strengthening in the AP, and 18F-FDG uptake is high. These features are relatively specific characteristics in adult PB.
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Affiliation(s)
- Mengnan Wu
- Department of Radiology, Heyuan People's Hospital, General Hospital Guangdong, Heyuan, 517000, China
| | - Jiongbin Lin
- Department of Radiology, Nanfang Hospital, Southern Medical University, Guangzhou, 510515, China
| | - Zhuangsheng Liu
- Department of Radiology, The Sixth Affiliated Hospital, South China University of Technology, Nanhai People's Hospital, Foshan, 528200, Guangdong, China
| | - Zhiming Huang
- Department of Radiology, Heyuan People's Hospital, General Hospital Guangdong, Heyuan, 517000, China.
| | - Ruoning Wang
- Minimally Invasive Centre, Tumour Hospital, Sun Yat-Sen University, Guangzhou, 510060, China.
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2
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Sheng M, Zhang R, Ma X, Zhou H. CT manifestations of childhood pancreatoblastoma. WORLD JOURNAL OF PEDIATRIC SURGERY 2022; 5:e000398. [DOI: 10.1136/wjps-2021-000398] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/26/2021] [Accepted: 04/27/2022] [Indexed: 11/03/2022] Open
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3
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Morales GES, Payan HL, Valladares RAM, Rosado ID, Chan C. Adult pancreatoblastoma: A rare malignant tumor of the pancreas. Ann Hepatobiliary Pancreat Surg 2021; 25:436-439. [PMID: 34402449 PMCID: PMC8382863 DOI: 10.14701/ahbps.2021.25.3.436] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/24/2020] [Revised: 02/15/2021] [Accepted: 02/15/2021] [Indexed: 11/21/2022] Open
Abstract
Pancreatoblastoma (PB) is a rare malignant tumor in adults. It has an overall incidence of 0.004 per 100,000 inhabitants. Its diagnosis with fine-needle aspiration (FNA) is difficult due to multiple differentiation lines present on PB that overlap with other tumors. A 76-year-old male patient presented with jaundice, weight loss of 10 kg in 6 months, and appetite loss. Abdominal computed tomography scan showed a tumor in the pancreas head. Transendoscopic ultrasound with FNA biopsy revealed a malignant epithelial neoplasia compatible with PB with immunohistochemistry CK19 (+), P63(+), synaptophysin (–), and Ki67 50%. The patient underwent surgical resection and adjuvant chemotherapy. Currently, the patient is in follow-up with the latest imaging showing no evidence of disease at 32 months after his initial diagnosis. PB is an uncommon malignant neoplasia with an aggressive behavior. Its diagnostic and therapeutic protocols are unclear. Its preoperative diagnosis may be difficult since its imaging features and serological markers are non-specific. However, FNA may be useful in some situations. Surgical resection is currently the recommended treatment that is associated with the best long-term survival.
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Affiliation(s)
| | - Hillary Lizarraga Payan
- Department of General Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | | | - Ismael Domínguez Rosado
- Department of Hepatopancreatobiliary Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Carlos Chan
- Department of Hepatopancreatobiliary Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
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4
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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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5
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Niger M, Prisciandaro M, Antista M, Monica MAT, Cattaneo L, Prinzi N, Manglaviti S, Nichetti F, Brambilla M, Torchio M, Corti F, Pusceddu S, Coppa J, Mazzaferro V, de Braud F, Di Bartolomeo M. One size does not fit all for pancreatic cancers: A review on rare histologies and therapeutic approaches. World J Gastrointest Oncol 2020; 12:833-849. [PMID: 32879662 PMCID: PMC7443847 DOI: 10.4251/wjgo.v12.i8.833] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2020] [Revised: 05/01/2020] [Accepted: 05/20/2020] [Indexed: 02/05/2023] Open
Abstract
Exocrine pancreatic neoplasms represent up to 95% of pancreatic cancers (PCs) and are widely recognized among the most lethal solid cancers, with a very poor 5-year survival rate of 5%-10%. The remaining < 5% of PCs are neuroendocrine tumors that are usually characterized by a better prognosis, with a median overall survival of 3.6 years. The most common type of PC is pancreatic ductal adenocarcinoma (PDAC), which accounts for roughly 85% of all exocrine PCs. However up to 10% of exocrine PCs have rare histotypes, which are still poorly understood. These subtypes can be distinguished from PDAC in terms of pathology, imaging, clinical presentation and prognosis. Additionally, due to their rarity, any knowledge regarding these specific histotypes is mostly based on case reports and a small series of retrospective analyses. Therefore, treatment strategies are generally deduced from those used for PDAC, even if these patients are often excluded or not clearly represented in clinical trials for PDAC. For these reasons, it is essential to collect as much information as possible on the management of PC, as assimilating it with PDAC may lead to the potential mistreatment of these patients. Here, we report the most significant literature regarding the epidemiology, typical presentation, possible treatment strategies, and prognosis of the most relevant histotypes among rare PCs.
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Affiliation(s)
- Monica Niger
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Michele Prisciandaro
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Maria Antista
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Melissa Anna Teresa Monica
- First Pathology Division, Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Laura Cattaneo
- First Pathology Division, Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Natalie Prinzi
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Sara Manglaviti
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Federico Nichetti
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Marta Brambilla
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Martina Torchio
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Francesca Corti
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Sara Pusceddu
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Jorgelina Coppa
- Hepato-biliary-pancreatic Surgery and Liver Transplantation Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Vincenzo Mazzaferro
- Hepato-biliary-pancreatic Surgery and Liver Transplantation Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
- Università degli studi di Milano, Milan 20133, Italy
| | - Filippo de Braud
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
- Università degli studi di Milano, Milan 20133, Italy
| | - Maria Di Bartolomeo
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
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6
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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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7
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Vilaverde F, Reis A, Rodrigues P, Carvalho A, Scigliano H. Adult pancreatoblastoma - Case report and review of literature. J Radiol Case Rep 2016; 10:28-38. [PMID: 27761191 DOI: 10.3941/jrcr.v10i8.2737] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022] Open
Abstract
Most cases of pancreatoblastoma, a rare malignant epithelial tumor of the pancreas, are seen in the pediatric population. The rarity of pancreatoblastoma, the similar radiologic findings to those seen in other pancreatic lesions, and its histopathologic heterogeneity, make its preoperative diagnosis in adults a real challenge. We report ultrasound, computed tomography and magnetic resonance imaging correlative findings of a histologically proven pancreatoblastoma in a 37-year-old woman. Pancreatoblastoma should be considered in the differential diagnosis of a pancreatic mass presenting uncommon imaging features.
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Affiliation(s)
- Filipa Vilaverde
- Department of Radiology, Centro Hospitalar de Entre o Douro e Vouga, Feira, Portugal
| | - Alcinda Reis
- Department of Radiology, Centro Hospitalar de Entre o Douro e Vouga, Feira, Portugal
| | - Pedro Rodrigues
- Department of Surgery, Centro Hospitalar de Entre o Douro e Vouga, Feira, Portugal
| | - Ana Carvalho
- Department of Pathologic Anatomy, Centro Hospitalar de Lisboa Central - Hospital Curry Cabral, Lisboa, Portugal
| | - Horácio Scigliano
- Laboratório de Anatomia Patológica Dr. Albino Oliveira Lda, Centro Hospitalar de Entre o Douro e Vouga, Feira, Portugal
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8
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Omiyale AO. Clinicopathological review of pancreatoblastoma in adults. Gland Surg 2015; 4:322-8. [PMID: 26312218 DOI: 10.3978/j.issn.2227-684x.2015.04.05] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2015] [Accepted: 03/24/2015] [Indexed: 12/23/2022]
Abstract
Pancreatoblastoma (PB) is a rare malignant neoplasm of the pancreas with unknown etiology. It occurs mostly in the pediatric population with very rare documented cases in adults. This is a review of the case reports of the adult pancreatoblastoma in the literature. A total of 35 cases were identified and reviewed with the mean age of 41 years (range, 18-78 years) and the male sex accounted for 51.4% of the cases. Adult Pancreatoblastoma seem to have a predilection for the head of the pancreas which accounted for approximately 49% of the cases reviewed with an average size of 8 cm (range, 1.8-20 cm). The median follow up for patients was 15 months (range, 1-108 months) Metastatic disease and local infiltration of surrounding tissues is common with poor prognosis in adult patients. Preoperative diagnosis is difficult because of the unhelpful tumor markers in adults and the cellular heterogeneity of the tumor which makes fine needle aspiration cytology unreliable. Histopathological review of the tumor is essential for diagnosis. Pancreatoblastomas should be considered a differential diagnosis of solid and cystic pancreatic neoplasms. Surgical resection of the tumor is the treatment of choice with a variable combination with radiotherapy and chemotherapy.
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9
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Abstract
OBJECTIVE The purpose of this article is to describe the sonographic, CT, and MRI features of primary malignant pancreatic tumors of childhood. CONCLUSION Most children with a pancreatic tumor present with a solid pseudopapillary tumor that is usually well marginated and has solid and cystic areas surrounded by a fibrous capsule. Pancreatoblastoma is more aggressive than solid pseudopapillary tumor. The imaging features are those of a large heterogeneous tumor. Ductal adenocarcinoma is rare in children and has a poor prognosis.
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10
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Imaging of pediatric pancreatic neoplasms with radiologic-histopathologic correlation. AJR Am J Roentgenol 2014; 202:1337-48. [PMID: 24848833 DOI: 10.2214/ajr.13.11513] [Citation(s) in RCA: 34] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
OBJECTIVE The purpose of this article is to describe the imaging features of pediatric pancreatic tumors with pathologic correlation. Epithelial and nonepithelial lesions are described. Pancreatic imaging protocols, clinical presentation, and management are also detailed. CONCLUSION Pancreatic neoplasms are rare in children and vary widely between benign and malignant causes and between cystic and solid lesions. Epithelial tumors are most common and include solid pseudopapillary tumor, pancreatoblastoma, islet cell neoplasms, and cystic lesions.
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11
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Hammer STG, Owens SR. Pancreatoblastoma: a rare, adult pancreatic tumor with many faces. Arch Pathol Lab Med 2013; 137:1224-6. [PMID: 23991735 DOI: 10.5858/arpa.2013-0272-cr] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
Abstract
Pancreatoblastomas are malignant epithelial neoplasms of the pancreas that are heterogeneous and have variable cellular differentiation, complicating the diagnosis. We report a case of pancreatoblastoma occurring in an adult patient, presenting as a pancreatic head mass with liver metastasis and jaundice. The initial liver biopsy diagnosis was metastatic neuroendocrine carcinoma based on morphology and synaptophysin positivity. At pancreatic resection, the diagnostic features of pancreatoblastoma were recognized. We review the radiologic and pathologic differential diagnosis, histologic heterogeneity, clinical presentation, and associated genetic syndromes for this unusual tumor that can mimic other types of pancreatic neoplasia.
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12
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Abstract
Pancreatoblastoma is a very rare childhood tumor originating from the epithelial exocrine cells of the pancreas. It is the most common malignant pancreatic tumor in young children and has a mean age of diagnosis of 5 years. It is slow growing and its presentation is varied and often non-specific. Tumors tend to be quite large and appropriate cross sectional imaging is very important to assess for extent, metastatic disease, and resectability. Biopsy for tissue diagnosis is essential. Complete surgical resection is the goal of therapy although many patients are unresectable at initial diagnosis and require neoadjuvant chemotherapy. Adjuvant chemotherapy is also recommended and chemotherapeutic regimens involve cisplatin and doxorubicin. Even with curative resections, these lesions have a high recurrence rate and patients must be followed closely. Knowledge of this rare tumor is important for the clinician confronted with a large retroperitoneal mass in a young child.
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13
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CT Imaging findings of malignant neoplasms arising in the epigastric region in children. Clin Imaging 2011; 35:10-20. [DOI: 10.1016/j.clinimag.2009.11.012] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2009] [Accepted: 11/15/2009] [Indexed: 11/17/2022]
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14
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Pancreatoblastoma In A Six-Month-Old Child. Radiol Case Rep 2009; 4:284. [PMID: 27307808 PMCID: PMC4897978 DOI: 10.2484/rcr.v4i2.284] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
The case of a six-month-old child with pancreatoblastoma is presented. Pancreatoblastoma is a rare primary neoplasm of childhood, usually presenting in children ranging from 2 −8 years old. The radiologic findings vary from solid to complex masses. This case is unusual due to the young age of the patient.
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15
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Abstract
Pancreatoblastoma is an extremely rare pancreatic tumor of childhood. We report our experience in 2 patients with pancreatoblastoma who had long-term survival and review the literature with a focus on chemotherapy in pancreatoblastoma with vascular invasion. The cases were 7-year-old and 4-year-old girls who complained of an abdominal mass without jaundice. Laboratory findings including serum alpha-fetoprotein were within normal limits. Radiologic investigations revealed a mass in the pancreas with vascular invasion. After surgical resection, postoperative chemotherapy included cisplatin, doxorubicin, ifosfamide, and etoposide. They have now been well for 7 years without recurrence.
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16
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Chung EM, Travis MD, Conran RM. Pancreatic tumors in children: radiologic-pathologic correlation. Radiographics 2006; 26:1211-38. [PMID: 16844942 DOI: 10.1148/rg.264065012] [Citation(s) in RCA: 97] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Pancreatic neoplasms are rare in children and have a different histologic spectrum and prognosis than those in adults. In general, these tumors are well demarcated with expansile rather than infiltrating growth patterns. They may be quite large at diagnosis, and central cystic necrosis is common. They infrequently cause biliary duct obstruction. The imaging appearance of each neoplasm reflects its pathologic features. Pancreatoblastoma is the most common pancreatic neoplasm in young children. At imaging, pancreatoblastomas are heterogeneous and often multilocular with hyperechoic and enhancing septa. Solid-pseudopapillary tumor occurs in adolescent girls. It is heterogeneous in internal architecture, with a mixture of solid and cystic hemorrhagic and necrotic elements. This tumor is distinguished by its fibrous capsule and hemorrhagic nature, which are best shown at magnetic resonance imaging as a dark rim on T1- or T2-weighted images and hyper-intense foci on T1-weighted images, respectively. Islet cell tumors in children are insulinomas or gastrinomas. These tumors manifest early due to hormonal syndromes and are distinguished by their small size, homogeneous appearance, and intense enhancement with intravenous contrast material. All pancreatic neoplasms in children are capable of producing metastases, usually to the liver and lymph nodes; however, on the whole, these tumors have a better clinical outcome than most pancreatic tumors in adults. Knowledge of the differential diagnosis of pancreatic masses in children and their relatively good prognosis may promote correct preoperative diagnosis and appropriate treatment.
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Affiliation(s)
- Ellen M Chung
- Department of Radiologic Pathology, Armed Forces Institute of Pathology, Alaska and Fern streets NW, Washington, DC 20306-6000, USA.
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17
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Kuxhaus L, Swayne LC, Chevinsky A, Samli B. Adult metastatic pancreaticoblastoma detected with Tc-99m MDP bone scan. Clin Nucl Med 2005; 30:577-8. [PMID: 16024962 DOI: 10.1097/01.rlu.0000170228.87136.a6] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
Abstract
Osteotropic radiopharmaceutical uptake has been reported in a wide variety of benign and malignant soft tissue tumors. We present an unusual case of pancreaticoblastoma with mesenteric and omental metastases detected by bone scan in a 69-year-old man who presented with fever, weight loss, and renal insufficiency. Pancreaticoblastoma is a rare childhood tumor that may occur in adults, although only two cases of adults with metastatic disease have been described.
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Affiliation(s)
- Lee Kuxhaus
- Department of Diagnostic Radiology, Morristown Memorial Hospital, Morristown, NJ 07960, USA
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18
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Muguerza R, Rodriguez A, Formigo E, Montero M, Vázquez JL, Páramo C, Campos C. Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature. J Pediatr Surg 2005; 40:1341-4. [PMID: 16080945 DOI: 10.1016/j.jpedsurg.2005.05.025] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/01/2022]
Abstract
A case of pancreatoblastoma (PB) in a 2 month-old male infant with incomplete Beckwith-Wiedemann syndrome is presented. Clinical examination disclosed left hemihypertrophy, macroglossia, bilateral exophthalmos, and enlargement of the left testis. Imaging with ultrasound and computed tomography scan showed a well-defined, heterogeneous, and grossly cystic mass arising from the head of the pancreas. Serum alpha-fetoprotein (AFP) level was elevated. The tumor was completely resected, and the histological analysis showed PB. The patient's recovery was uneventful, and AFP returned to normal values after surgery. The child has been disease-free for 5 years, and his serum AFP remained within normal values. Six other examples of this association, PB, and Beckwith-Wiedemann syndrome are recorded in the literature. The risk of developing tumor in this syndrome (complete and incomplete form) increases when hemihypertrophy is present, and the need for routine screening examination is warranted. Beckwith-Wiedemann syndrome was suggested to be a favorable biological marker for survival in children who have intraabdominal tumors.
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Affiliation(s)
- Rosa Muguerza
- Department of Pediatric Surgery, Complejo Hospitalario Xeral-Cíes, Pizarro 22, 36204 Vigo, Spain.
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19
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Rosebrook JL, Glickman JN, Mortele KJ. Pancreatoblastoma in an adult woman: sonography, CT, and dynamic gadolinium-enhanced MRI features. AJR Am J Roentgenol 2005; 184:S78-81. [PMID: 15728031 DOI: 10.2214/ajr.184.3_supplement.01840s78] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
Affiliation(s)
- Joshua L Rosebrook
- Division of Abdominal Imaging and Intervention, Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis St., Boston, MA 02115, USA
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20
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Nijs E, Callahan MJ, Taylor GA. Disorders of the pediatric pancreas: imaging features. Pediatr Radiol 2005; 35:358-73; quiz 457. [PMID: 15536562 DOI: 10.1007/s00247-004-1326-1] [Citation(s) in RCA: 52] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/02/2004] [Revised: 07/23/2004] [Accepted: 08/05/2004] [Indexed: 01/27/2023]
Abstract
The purpose of this manuscript is to provide an overview of the normal development of the pancreas as well as pancreatic pathology in children. Diagnostic imaging plays a major role in the evaluation of the pancreas in infants and children. Familiarity with the range of normal appearance and the diseases that commonly affect this gland is important for the accurate and timely diagnosis of pancreatic disorders in the pediatric population. Normal embryology is discussed, as are the most common congenital anomalies that occur as a result of aberrant development during embryology. These include pancreas divisum, annular pancreas, agenesis of the dorsal pancreatic anlagen and ectopic pancreatic tissue. Syndromes that can manifest pancreatic pathology include: Beckwith Wiedemann syndrome, von Hippel-Lindau disease and autosomal dominant polycystic kidney disease. Children and adults with cystic fibrosis and Shwachman-Diamond syndrome frequently present with pancreatic insufficiency. Trauma is the most common cause of pancreatitis in children. In younger children, unexplained pancreatic injury must always alert the radiologist to potential child abuse. Pancreatic pseudocysts are a complication of trauma, but can also be seen in the setting of acute or chronic pancreatitis from other causes. Primary pancreatic neoplasms are rare in children and are divided into exocrine tumors such as pancreatoblastoma and adenocarcinoma and into endocrine or islet cell tumors. Islet cell tumors are classified as functioning (insulinoma, gastrinoma, VIPoma and glucagonoma) and nonfunctioning tumors. Solid-cystic papillary tumor is probably the most common pancreatic tumor in Asian children. Although quite rare, secondary tumors of the pancreas can be associated with certain primary malignancies.
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Affiliation(s)
- Els Nijs
- Department of Radiology, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium
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Dhebri AR, Connor S, Campbell F, Ghaneh P, Sutton R, Neoptolemos JP. Diagnosis, treatment and outcome of pancreatoblastoma. Pancreatology 2004; 4:441-51; discussion 452-3. [PMID: 15256806 DOI: 10.1159/000079823] [Citation(s) in RCA: 87] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/12/2003] [Accepted: 12/10/2003] [Indexed: 12/11/2022]
Abstract
BACKGROUND Pancreatoblastoma is a rare tumour mainly presenting in childhood but also in adults. OBJECTIVES The aim was to determine the clinical course of pancreatoblastoma by an analysis of reported cases. METHODS Patients with pancreatoblastoma were identified from Medline and combined with patients identified from the Royal Liverpool University Hospital. RESULTS There were 153 patients with a median (range) age at presentation of 5 (0-68) years and a male:female ratio of 1.14:1. The most frequent site was the head of pancreas (48/123, 39%). The median and 5-year (95% CI) survival rates were 48 months and 50% (37-62%) respectively. At presentation there were 17 (17%) out of 101 patients with metastases, the liver being the commonest site (15/17, 88%). On univariate analysis, factors associated with a worse prognosis were synchronous (p = 0.05) or metachronous metastases (p < 0.001), non-resectable disease at presentation (p < 0.001) and age > 16 years at time of presentation (p = 0.02). On multivariate analysis, resection (p = 0.006) and metastases post-resection (p = 0.001) but not local recurrence influenced survival. CONCLUSIONS Pancreatoblastoma is one of the pancreatic tumours with a relatively good prognosis. The treatment of choice is complete resection with long-term follow-up aiming to treat any early local recurrence or metastasis.
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Affiliation(s)
- A R Dhebri
- Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK
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Shen J, Liang BL, Wang HY. Septal enhancement within pancreatoblastoma of the pancreatic head presenting with progressive anaemia. Pediatr Radiol 2004; 34:583. [PMID: 15205844 DOI: 10.1007/s00247-004-1144-5] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/08/2003] [Revised: 12/14/2003] [Accepted: 12/21/2003] [Indexed: 11/30/2022]
Affiliation(s)
- Jun Shen
- Department of Radiology, Second Affiliated Hospital, Sun Yat-Sen University, 107 West Yanjiang Road, Guangzhou 510120, Guangdong, People's Republic of China.
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Benya EC. Pancreas and biliary system: imaging of developmental anomalies and diseases unique to children. Radiol Clin North Am 2002; 40:1355-62. [PMID: 12479715 DOI: 10.1016/s0033-8389(02)00055-6] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Abstract
The focus of this article is on the development of the pancreas and biliary system, with a review of the most common congenital anomalies affecting the pancreas and biliary tree, including pancreas divisum, annular pancreas, congenital shortening of the pancreas, and choledochal cysts. Additionally, biliary atresia and pancreatoblastoma--both diseases of the pancreas and biliary system that almost exclusively affect children--are considered, with a discussion regarding the clinical presentation and imaging appearances of these disorders.
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Affiliation(s)
- Ellen C Benya
- Department of Medical Imaging, Children's Memorial Hospital, 2300 Children's Plaza #9, Chicago, IL, USA.
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Henke AC, Kelley CM, Jensen CS, Timmerman TG. Fine-needle aspiration cytology of pancreatoblastoma. Diagn Cytopathol 2001; 25:118-21. [PMID: 11477717 DOI: 10.1002/dc.2017] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Pancreatoblastoma is a rare pancreatic neoplasm seen most commonly in the pediatric age group. We report on the aspiration cytology and immunohistochemical findings of a pancreatoblastoma in a 16-yr-old male.
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Affiliation(s)
- A C Henke
- Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242-1009, USA
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Ogawa B, Okinaga K, Obana K, Nakamura K, Hattori T, Ito T, Yanagawa Y, Tanaka F, Imamura T. Pancreatoblastoma treated by delayed operation after effective chemotherapy. J Pediatr Surg 2000; 35:1663-5. [PMID: 11083448 DOI: 10.1053/jpsu.2000.18347] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
A case of pancreatoblastoma in a 4-year-old girl is presented. She was admitted with an abdominal mass and vomiting in August 1994. Computed tomography scan showed a 10- x 8- x 7-cm mass occupying both the head and body of the pancreas. Serum alpha-fetoprotein (AFP) level was 9,600 ng/mL (normal, <8.3 ng/mL). Results of open biopsy of the tumor showed pancreatoblastoma. Chemotherapy was administered using the new A-1 regimen consisting of cyclophosphamide, etoposide, pirarubicin, and cisplatin. After 3 cycles of chemotherapy, the size of the tumor was reduced to 5 x 4 x 3 cm, the portal vein became patent, and the AFP value decreased to 98.1 ng/mL. Total removal of the tumor was performed leaving the head and tail of the pancreas. Postoperative chemotherapy continued for 2 years. The patient has been disease free for 5 years, and her serum AFP remained within normal levels.
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Affiliation(s)
- B Ogawa
- Second Department of Surgery, Teikyo University School of Medicine, Tokyo, Japan
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Abstract
This article reviews the CT imaging features of the most frequent mesenchymal tumors of the pancreas and stresses important distinctive patterns that may help distinguish specific entities. Various neoplasms (lymphangioma, lipoma, teratoma, pancreatoblastoma, schwannoma, neurofibroma, lymphoma, and sarcoma) are reviewed, with key differential points (structure, fatty and water densities, calcification, pattern of contrast enhancement, vascularization, and necrotic or regressive changes) emphasized. In addition, epithelial tumors are considered in the differential diagnosis.
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Affiliation(s)
- F Ferrozzi
- Dipartimento di Diagnostica per Immagini, Università di Parma, Italy.
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Montemarano H, Lonergan GJ, Bulas DI, Selby DM. Pancreatoblastoma: imaging findings in 10 patients and review of the literature. Radiology 2000; 214:476-82. [PMID: 10671596 DOI: 10.1148/radiology.214.2.r00fe36476] [Citation(s) in RCA: 65] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
PURPOSE To describe the features of pancreatoblastoma at magnetic resonance (MR) imaging, computed tomography (CT), and ultrasonography (US). MATERIALS AND METHODS Imaging and surgical findings in 10 patients (age range, 2-20 years; mean age, 6.8 years) with pathologically proved pancreatoblastoma were reviewed for tumor size, organ of origin, definition and quality of tumor margins, tumor heterogeneity, calcification, enhancement, ascites, biliary and/or pancreatic ductal dilatation, local invasion, adenopathy, vascular invasion, vascular encasement, metastases, and signal intensity on MR images. Results from 10 CT, seven US, and three MR imaging examinations were reviewed. RESULTS Five of the 10 tumors were pancreatic; four others appeared to be pancreatic or hepatic. Most had well-defined margins (nine of 10), were heterogeneous (nine of 10), and enhanced (10 of 10). Other findings included calcification (two of 10), biliary and pancreatic ductal dilatation (one of 10), and ascites (three of 10). Hepatic (two patients) and pelvic (two patients) metastases were present. Adenopathy (two patients) and vascular invasion (one patient) were not identified radiologically. Tumors had low to intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. CONCLUSION Pancreatoblastoma is typically a heterogeneous tumor with well-defined margins that may appear to arise from the pancreas or liver. It may behave aggressively, with localized vascular or bowel invasion or with widespread metastatic disease. Although it is rare, it should be considered in the differential diagnosis of an upper abdominal mass in a child.
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Affiliation(s)
- H Montemarano
- Department of Diagnostic Imaging and Radiology, Children's National Medical Center, Washington, DC, USA.
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Abstract
BACKGROUND Pancreatoblastoma is a rare pancreatic tumor with distinct acinar and squamoid cell differentiation that generally affects infants and young children. Just over 50 cases have been reported in the literature. METHODS Five cases of pathologically proven pancreatoblastoma treated at Seoul National University Hospital from 1984 to 1994 were reviewed. There were three girls and two boys who were 2 years to 5 years of age. All cases came to medical attention because of an abdominal mass. RESULTS Abdominal pain was observed in one case and anorexia, vomiting, and weight loss in one case. There was marked elevation of serum alpha-fetoprotein (27,000 ng/mL) in one case of liver metastases. Complete excision was performed in two cases in which the tumors were located in the tail of the pancreas. Partial excision was performed in two patients who had unresectable tumors of the head of the pancreas. One patient had an unresectable tumor at diagnosis and needle aspiration biopsy was carried out under ultrasound guidance. Electron microscopy was performed on pathological specimens of three cases and showed zymogen granules but not neuroendocrine granules. Immunocytochemical studies for alpha-fetoprotein, insulin, glucagon and somatostatin were performed in one patient, and results were all negative. Of two patients who underwent complete excision, one patient presented with liver metastases 4 months after operation and received chemotherapy, but died of tumor 6 months after operation. The other patient had local recurrence 1 year after operation. Reoperation and chemotherapy were performed, and the child is now alive without evidence of disease for 32 months. All three patients who had unresectable tumor died of tumor despite adjuvant radiotherapy and chemotherapy. CONCLUSIONS The authors emphasize that the diagnosis of pancreatoblastoma in childhood should be suspected with palpation of an abdominal mass, and the chance for cure may be determined by complete excision of the tumor.
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Affiliation(s)
- Y Chun
- Department of Pediatric Surgery, Seoul National University Children's Hospital, Korea
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