Surgical management of cerebellar hemangioblastomas in patients with von Hippel–Lindau disease

Abstract

Object Despite the frequency of cerebellar hemangioblastomas in von Hippel–Lindau (VHL) disease, their optimum contemporary management has not been defined, and is made complex because of the multiple, progressive, and protean nature of the tumors found in patients with this disorder. To examine modern management and outcomes of cerebellar hemangioblastomas in VHL disease, the authors reviewed findings in patients with this disease who underwent resection of cerebellar hemangioblastomas. Methods Consecutive patients with VHL disease who underwent surgery for cerebellar hemangioblastoma(s) at the National Institutes of Health were included. Eighty consecutive patients (44 female and 36 male patients) underwent 126 operations for removal of 164 cerebellar hemangioblastomas (age at surgery 37.8 ± 10.3 years, follow-up duration 96.0 ± 60.3 months). Serial clinical examinations, imaging studies, and operative records were analyzed. Results Symptoms and signs included headache (94 operations; 75%), ataxia (55%), dysmetria (29%), and hydrocephalus (28%). Although the primary objective of surgery was resection of the hemangioblastoma considered responsible for symptoms (136 of the hemangioblastomas [83%]), 28 additional hemangioblastomas (17%) were removed during the same surgeries. Tumors associated with symptoms were larger (diameter 1.8 ± 1.9 cm; volume 2.8 ± 3.4 cm3; p < 0.05) and more likely to be associated with peritumoral edema or peritumoral cysts (100% associated with edema and/or cyst; p < 0.05) than asymptomatic tumors (diameter 1.1 ± 0.9 cm; volume 0.7 ± 0.4 cm3; 18%). More tumors were located in the posterior (74%) compared with the anterior (26%) half of the cerebellum (p < 0.05). Three months after resection, symptom improvement/stabilization had occurred following 124 of the operations (98%). Preoperative hydrocephalus resolved after tumor removal in 33 cases (94%) and did not require cerebrospinal fluid diversion. Long-term imaging follow-up (61.5 ± 15.0 months) revealed no recurrences. Conclusions Symptoms and signs caused by cerebellar hemangioblastomas in VHL disease are associated with edema and peritumoral cyst formation/propagation and are treated safely and effectively with resection. Cerebrospinal fluid diversion is rarely necessary after complete tumor removal in patients with preoperative hydrocephalus. Cerebellar hemangioblastomas are preferentially distributed in the posterior half of the cerebellum, as they are in the brainstem and spinal cord. Tumor recurrence is avoided by meticulous extracapsular resection.

Genetic analysis of von Hippel-Lindau disease

“…Patients with numerous tumors experienced growth and quiescent phases simultaneously, suggesting that a combination of acquired genetic lesions and hormonal activity influence tumor growth. The study advises increased monitoring of patients during growth phases or if a cyst develops [Jagannathan et al, 2008;Wanebo et al, 2003].…”

Multifocal central nervous system hemangioblastoma: a case report and review of the literature

“…The nodules invaded cyst cavities, penetrated the cyst wall, or remained outside the cyst wall. An uncut nodule may retain the ability to produce cyst fluid and ultimately induce tumor regeneration (Miyagami and Katayama, 2004;Jagannathan et al, 2008). Therefore, we attempted to remove the cyst wall and the nodule.…”

Dr. Arvid Lindau and discovery of von Hippel-Lindau disease

“…He emphasized that meticulous inspection of the cyst wall was critical or mural hemangioblastoma could be missed (a feature described in the microsurgical era). 10 He described their location as frequently near or on the cerebellar surface and characterized by marked hypertrophy of afferent and efferent vessels. Microscopically, he described hemangioblastomas as complex lesions composed of vascular elements containing no nerve or glial cells.…”