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Case History

30-year-old presenting with bilateral papilledema and vision problems. Neuroimaging reveals a 2.7 x 2.1 cm right cerebellar mass

What is the most likely diagnosis?

A. Metastatic Renal Cell Carcinoma
B. Melanoma
C. Hemangioblastoma
D. Angiosarcoma

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  Williams22CasePic3 Williams22CasePic4Alpha-Inhibin staining showing positivity in neoplastic cells

 Answer: C. Hemangioblastoma

Microscopic Description

  • Microscopic examination demonstrates neoplastic stromal cells having abundant microvesicular cytoplasm that appears consistent with lipidized content. 
  • The nuclei of the neoplastic stromal cells appear moderately enlarged and hyperchromatic.
  • Brisk mitotic activity is lacking within this neoplasm. 
  • A relatively abundant network of branched microvasculature is present throughout this neoplasm
  • IHC
    • Inhibin highlights neoplastic cells
    • CD10 and PAX8 are negative within the neoplastic cells

Educational Description

  • Typically occur in the cerebellum but can occur outside the CNS i.e. including but not limited to bone and soft tissue, liver, lung
  • Most commonly found in sporadic cases but also associated with von Hippel-Lindau syndrome (VHL)
  • Hemangioblastomas produce erythropoietin and may cause secondary polycythemia
  • Imaging characteristics: "cyst-enhancing mural nodule" pattern
  • Histopathology: demonstrates two main components
    • Neoplastic stromal cells that are large with cytoplasmic lipid-containing vacuoles
    • Abundant reactive vascular cells
  • Staining
    • Alpha-Inhibin is frequently expressed in the neoplastic stromal cells; these neoplastic cells are also negative for EMA, CD10 and CAM5.2 which helps differentiate them from metastatic renal cell carcinoma
Case contributed by: Michael Williams, M.D., M.Sc. Neuropathology Fellow, UAB Pathology