Cerebellar hemangioblastoma

Von Hippel-Lindau disease is inherited on an autosomal dominant gene that may show marked expressive variability of cancer phenotype in certain patients and families. A patient with a strongly positive family history of this disease, at age 28, underwent craniotomy with removal of a cystic cerebellar hemangioblastoma. At age 48 he developed syringomyelia of the spinal cord, became quadriplegic, and had a progressive downhill course. At autopsy, hemangioblastomas... 

Because the hemangioblastoma is nonfibrillar, it should not resemble an astrocytoma. However, the resemblance may occur for two reasons sampling and artifact. Cerebellar hemangioblastomas are often cystic, with the actual neoplasm embedded somewhere in the wall of the cyst as a mural nodule. Biopsy specimens of the cyst wall may show conspicuously GFAP-positive gliosis (see the Gliosis versus Glioma section later in this chapter). 

Tachibana O, Yamashima T, Yamashita J. Immunohistochemical study of erythropoietin in cerebellar hemangioblastomas associated with secondary polycythemia. Neurosurgery. 1991 28 24-26. 

Feldenzer JA, McKeever PE. Selective localization of gamma-enolase in stromal cells of cerebellar hemangioblastomas. Acta... 

McComb RD, Eastman PJ, Hahn FJ, et al. Cerebellar hemangioblastoma with prominent stromal astrocytosis Diagnostic and histogenetic considerations. Clin Neuropathol. 1987 6 149-154. 

---