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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?

   Alpha-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