Ampullary Pancreatoblastoma in an Elderly Patient: A Case Report and Review of the Literature

Du, Emma Z.; Katz, Matthew J.; Weidner, Noel; Yoder, Suzanne; Moossa, A. R.; Shabaik, Ahmed

doi:10.5858/2003-127-1501-apiaep

Cited by 25 publications

(10 citation statements)

References 5 publications

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“…The overwhelming majority of pancreatoblastomas occur within the pancreas 2,20 . Rare case reports exist of extrapancreatic pancreatoblastomas, including a single case reported in the ampulla of Vater in a 78-year-old woman with similar clinical presentation and pathological findings to our case 8 . Though easily explained by the ampullary location, obstructive jaundice is otherwise uncommon in pancreatic pancreatoblastomas.…”

Section: Discussionsupporting

confidence: 75%

“…2,20 Rare case reports exist of extrapancreatic pancreatoblastomas, including a single case reported in the ampulla of Vater in a 78-year-old woman with similar clinical presentation and pathological findings to our case. 8 Though easily explained by the ampullary location, obstructive jaundice is otherwise uncommon in pancreatic pancreatoblastomas. The comparatively small tumor size of these 2 ampullary pancreatoblastoma, when compared with an average size of 10.6 cm in pancreatic tumors, 4 suggests that jaundice likely expedites diagnosis, compared with the nonspecific or asymptomatic presentation of pancreatic pancreatoblastoma.…”

Section: Discussionmentioning

confidence: 99%

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A Subset of Pancreatoblastomas May Arise From an Adenomatous Precursor

Slack¹,

Bründler²,

Box³

et al. 2022

Pancreas

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Pancreatoblastomas are rare pediatric tumors. In adults, they are exceedingly rare and seem to have a worse prognosis. Most are sporadic, though rare, cases occur in patients with familial adenomatous polyposis. Unlike pancreatic ductal adenocarcinomas, pancreatoblastomas are not believed to arise from dysplastic precursor lesions. Clinical history, along with endoscopic, pathological, and molecular findings, was reviewed for a 57-year-old male patient with an ampullary mass who presented with obstructive jaundice. Microscopic examination showed a pancreatoblastoma subjacent to an adenomatous polyp with intestinal differentiation and low-grade dysplasia. Both tumors had abnormal p53 (complete loss) and nuclear β-catenin immunostaining. Mutational panel analysis showed an identical CTNNB1 (p.S45P) mutation in both. This case adds to our understanding of the pathogenesis of these rare tumors and suggests that a subset may arise from an adenomatous precursor. In addition, this case is just the second pancreatoblastoma to originate in the duodenal ampulla, and the preceding case suggests that an ampullary location leads to earlier diagnosis. Moreover, this case highlights the difficulty in diagnosing pancreatoblastoma on limited tissue specimens and illustrates the need to include pancreatoblastoma in the differential diagnosis in all tumors in and around the pancreas, including those in adult patients.

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Section: Discussionsupporting

confidence: 75%

Section: Discussionmentioning

confidence: 99%

A Subset of Pancreatoblastomas May Arise From an Adenomatous Precursor

Slack¹,

Bründler²,

Box³

et al. 2022

Pancreas

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“…Serum markers are generally not helpful in the diagnosis of PB. Significantly elevated serum levels of CEA and/or a-fetoprotein level have been reported in 30-50% of paediatric cases, though they are not usually high with adult pancreatoblastoma patients [9,17,22,26]. Elevated serum levels of tumor markers at diagnosis, usually fall down to normal after the tumor resection and serve as potential indicators of subsequent tumor recurrence.…”

Section: Discussionmentioning

confidence: 99%

Pancreatoblastoma in an Adult Patient with Late Recurrence

Sedloev¹

2015

J Cytol Histol

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Pancreatoblastoma (PB) is among the most frequent pancreatic tumor in childhood, but exceedingly rare in adults. The prognosis of PB in adults is very poor with a mean survival time of 10 months.The case describes a 36-year-old woman with a painful palpable tumor in the upper part of the abdomen. The complete resection of the tumor confirmed a 14 cm well-circumscribed tumor with heterogeneous morphological appearance with three main components -neuroendocrine, acinar and squamous. The morphologic and immunohistochemical features proved to be consistent with PB. The patient did not receive any adjuvant chemotherapy. Seventy-two months later a bulky relapsing abdominal tumor was discovered. The histology revealed undifferentiated tumor with solid sheets of medium large atypical cells, areas of tumor necrosis and high mitotic index. Despite the different morphology in the relapse, neuroendocrine differentiation was documented with immunohistochemistry.The case presents a rare malignant tumor of the exocrine pancreas in adult patient. The discussion focuses on the characteristic macroscopic and histological characteristics, immunohistochemical profile and molecular genetics, considering a list of common differential diagnostic entities. This study underlines the importance of recognizing the pancreatoblastoma as a malignant tumor in non-paediatric group in which surgical resection is the best choice of treatment, associated with long-term survival.

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“…1–11 These tumors seem to be more aggressive in adults than in children but less aggressive than classical pancreatic ductal adenocarcinoma. 12–43,56 Fewer than 70 adult pancreatoblastomas have been reported in the English literature. 1…”

Section: Introductionmentioning

confidence: 99%

Adult Pancreatoblastoma: Report of 3 new Cases With Genetic Diversity and Autopsy Findings

et al. 2022

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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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Ampullary Pancreatoblastoma in an Elderly Patient: A Case Report and Review of the Literature

Cited by 25 publications

References 5 publications

A Subset of Pancreatoblastomas May Arise From an Adenomatous Precursor

A Subset of Pancreatoblastomas May Arise From an Adenomatous Precursor

Pancreatoblastoma in an Adult Patient with Late Recurrence

Adult Pancreatoblastoma: Report of 3 new Cases With Genetic Diversity and Autopsy Findings

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