Solid Pseudopapillary Neoplasm
Gene Landon, MD
Key Facts
Clinical Issues
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Typically in young females, in body/tail of pancreas
Cytopathology
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Pseudopapillae with tumor cell layer(s) arranged around capillaries
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± metachromatic myxoid or hyaline stromal pericapillary layer
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Bland nuclei ± longitudinal grooves
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Long, slender cytoplasmic processes
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Intracytoplasmic hyaline globules
Ancillary Tests
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PAS-D(+) hyaline globules
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Positive for β-catenin (nuclear), β-1-antitrypsin, CD10, vimentin, PR, CD56, CD99 (paranuclear dot-like pattern); synaptophysin and keratin both (+/-)
Top Differential Diagnoses
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Pancreatic endocrine neoplasm
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Acinar cell carcinoma
TERMINOLOGY
Definitions
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Low-grade malignant neoplasm of uncertain cellular derivation
CLINICAL ISSUES
Epidemiology and Presentation
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Uncommon, 1-2% of pancreatic exocrine tumors
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Female predominance, rare in men
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Most patients in 20s and 30s, but wide age range
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Vague abdominal pain, anorexia, weight loss, nausea, vomiting
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May have palpable abdominal mass
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