Neuroendocrine neoplasms
Clear cell pancreatic endocrine tumor

Author: Deepali Jain, M.D. (see Authors page)

Revised: 18 September 2018, last major update August 2012

Copyright: (c) 2002-2018,, Inc.

PubMed Search: Clear cell pancreatic endocrine tumor

Cite this page: Jain, D. Clear cell pancreatic endocrine tumor. website. Accessed November 17th, 2018.
Definition / general
Gross description
  • Solid, multinodular, golden yellow
  • May be cystic
Gross images

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Patient with von Hippel-Lindau disease

Microscopic (histologic) description
  • 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
Microscopic (histologic) images

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Clear cell morphology

Pancreatic neuroendocrine tumors 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
  • 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)
Differential diagnosis