Poorly Differentiated Neuroendocrine Carcinoma, Pancreas
Vikram Deshpande, MD
Key Facts
Clinical Issues
Very aggressive neoplasms
Patients may benefit from platinum-based chemotherapy
Microscopic Pathology
Small cell variant
Resembles small cell carcinomas of lung
Scant cytoplasm, hyperchromatic nuclei, prominent nuclear moulding
At least 10 mitotic figures/HPF are required
Large cell variant
Resembles large cell neuroendocrine carcinoma of lung
Frequent mitoses (> 10/10 HPF), vascular invasion,
necrosis
Ancillary Tests
Positive for neuroendocrine markers, keratin
 The pancreatic parenchyma is replaced by a poorly differentiated neuroendocrine carcinoma, featuring small round blue tumor cells organized in sheets and trabeculae. |
 Most poorly differentiated neuroendocrine carcinomas of the pancreas are virtually identical to small cell carcinoma of the lung. Note the hyperchromatic nuclei with moulding and prominent apoptosis. |
TERMINOLOGY
Synonyms
Small cell carcinoma
High-grade neuroendocrine carcinoma
Poorly differentiated endocrine carcinoma
Definitions
Clinically aggressive carcinoma of pancreas with morphological features suggestive of neuroendocrine differentiation and high proliferation (> 10 mitotic figures/10 HPF)
ETIOLOGY/PATHOGENESIS
Unknown
No unequivocal evidence that well-differentiated endocrine neoplasms progress to poorly differentiated carcinomas
CLINICAL ISSUES
Epidemiology
Incidence
Rare, constituting 2-3% of all pancreatic endocrine neoplasms
Presentation
Jaundice
Back pain
Some patients present with hormonal symptoms including Cushing syndrome and hypercalcemia
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