A BSTRACT Background: Hemangioblastomas (HBL) are benign neoplasms that account for <2% of all CNS tumors, commonest site being cerebellum. They may occur sporadically or in association with Von Hippel-Lindau (VHL) syndrome. Objective: Clinico-morphological, radiological and syndromic association of CNS hemangioblastoma. Material: This study includes 35 diagnosed cases of CNS hemangioblastoma between January 2013 and June 2023. Results: The cerebellum was the commonest site of occurrence, followed by spine, with a male preponderance and the mean age being 46 years. On imaging, 42% of patients had a cystic lesion with a mural nodule; the radiological differentials were metastasis followed by pilocytic astrocytoma. Immunohistochemistry (IHC) revealed stromal positivity for S-100 and Inhibin. CD10, EMA and GFAP were performed to rule out close differentials, including metastatic renal cell carcinoma, meningioma, glioma and ependymoma, depending on the location of tumor. Germline testing for VHL mutations was performed in 20% of cases, identifying four syndromic patients. Conclusion: HBLs are slow-growing CNS grade 1 tumors with excellent prognosis after complete excision. The combination of the location, imaging and morphologic characteristics are very useful in the diagnosis of these unique and uncommon tumors.
Banerjee et al. (Fri,) studied this question.