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Pancreatoblastoma. A clinicopathologic study and review of the literature
D S Klimstra1, B M Wenig, C F Adair
1Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
Insights
Pancreatoblastoma, a rare pancreatic tumor, typically affects children but also occurs in adults. Pediatric cases show better outcomes and chemotherapy response compared to adults.
Area of Science:
- Oncology
- Pediatric Oncology
- Gastroenterology
Background:
- Pancreatoblastoma is a rare malignant neoplasm of the pancreas.
- It predominantly affects infants and young children, but adult cases are also documented.
Purpose of the Study:
- To characterize the clinicopathologic features, immunohistochemical profile, and outcomes of pancreatoblastoma.
- To compare the behavior and prognosis of pancreatoblastoma in pediatric versus adult patients.
Main Methods:
- Retrospective analysis of 14 newly diagnosed pancreatoblastoma cases.
- Review of 41 previously reported cases from the literature.
- Immunohistochemical and ultrastructural examination of tumor tissues.
Main Results:
- The study included 14 cases (9 pediatric, 5 adult) and 41 reviewed cases.
- Histologically, tumors showed acinar structures, ductal formations, and squamoid corpuscles.
- Immunohistochemistry revealed acinar, endocrine, and ductal differentiation.
- Metastasis occurred in 36% of patients, predominantly to the liver.
- Adult patients had a poorer prognosis (3/5 died) compared to pediatric patients (5/6 alive).
- Pediatric cases showed good response to chemotherapy.
Conclusions:
- Pancreatoblastoma exhibits diverse differentiation and variable behavior.
- Pediatric pancreatoblastoma generally has a better prognosis and responds well to chemotherapy compared to adult cases.
Abstract:
Pancreatoblastoma is a rare pancreatic tumor with a distinctive histologic appearance that generally affects infants and young children. We have studied 14 cases of pancreatoblastoma and reviewed 41 previously reported examples. Nine of our cases occurred in children (from newborn to 4 years old; mean, 2.4), and five affected adults (from 19 to 56 years old; mean, 40). There were 8 male cases and 6 female cases. Most patients presented with incidental abdominal masses, although pain, weight loss, and obstructive jaundice were present, but rarely. The tumors were very cellular microscopically, with cytologically uniform epithelial cells arranged in sheets and nests. Well-formed acinar structures were a consistent feature, and several cases contained ectatic ductular formations, rarely exhibiting intracellular mucin. Consistently present were squamoid corpuscles: circumscribed, whorled nests of plump spindle cells with a squamous appearance and occasional keratinization. The stroma was moderate to abundant and frequently quite cellular (especially in the pediatric cases). By immunohistochemistry, the tumors exhibited acinar, endocrine, and ductal differentiation, with positivity for pancreatic enzymes (100%), endocrine markers (82%), and carcinoembryonic antigen (85%). Ultrastructural examination most commonly revealed acinar differentiation, although mucigen granules and neurosecretory granules were also occasionally found. The behavior was variable: 36% of patients developed metastases, especially to the liver. The adult patients did poorly: three of five died of tumor (mean survival, 18 months), and two were alive at 5 and 15 months, respectively. In contrast, five of the six evaluable pediatric patients were alive from 22 months to 22 years after diagnosis, and only one died of tumor after 16 months. Good responses to chemotherapy were noted in the pediatric group.