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Pancreas

Endocrine tumors

Clear cell pancreatic endocrine tumor

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 14 December 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Clear cell change is specific for von Hippel-Lindau disease, due to intracytoplasmic lipid (Am J Surg Pathol 2001;25:602)
● Rarely associated with hypercalcemia (Arch Pathol Lab Med 2003;127:241)
● Lipid rich variant of pancreatic endocrine neoplasm may also have clear cells, but is distinct variant (Am J Surg Pathol 2006;30:194, Acta Cytol 2010;54:829)

Gross description
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● Solid, multinodular, golden-yellow
● May be cystic

Gross images
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Pancreatic Endocrine Tumor from patient with von Hippel–Lindau Disease

Micro description
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● Clear cells in nests, cords and tubules with central hemorrhage and associated thin walled vessels
● Cords and gyriform pattern, when present, are suggestive of endocrine tumors
● Vascular invasion common
● May have adjacent serous cystadenoma-like areas

Micro images
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Clear cell morphology


Pancreatic neuroendocrine tumours associated with von Hippel-Lindau disease are characterized by cytoplasmic vacuolization with multiple aggregates of small lipid droplets, some of which may impinge on and indent the nucleus


Figure 1: 8 cm tumor is firm and sharply demarcated from spleen and rest of pancreas
Figure 2: Clear cells are rounded, with large vacuoles in cytoplasm, displacing the nuclei to the periphery
Figure 3: Other clear cells have smaller vacuoles
Figure 4: EM shows 150-200 nm, membrane bound, dense core neuroendocrine granules

Cytology description
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● Large sheets and rounded clusters of polygonal neoplastic cells with relatively abundant cytoplasm containing numerous, small, sharply-demarcated vacuoles, giving rise to "frothy" appearance (Diagn Cytopathol 2009;37:365)

Positive stains
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● Chromogranin, synaptophysin, pancreatic polypeptide

Differential diagnosis
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● Adrenal tumors
● Clear cell hepatocellular carcinoma
● Metastatic renal cell carcinoma: negative for neuroendocrine markers and neurosecretory granules
● Mucinous adenocarcinoma, low grade
● Perivascular epithelioid cell tumor: HMB45+, negative for endocrine markers
● Serous microcystic adenoma: small cystic spaces lined by small cuboidal cells with clear cytoplasm (glycogen), minimal mucin, myoepithelial layer present, round hyperchromatic central nuclei
● Solid pseudopapillary tumors, clear cell variant: pseudopapillae with hyalinized fibrovascular cores lined by several layers of bland fragile epithelial cells with clear to eosinophilic cytoplasm, variable intracytoplasmic PAS+ hyaline globules
● Steroid secreting tumors of ovary/testis

End of Pancreas > Tumors > Clear cell pancreatic endocrine tumor

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