Posterior Fossa Hemangioblastoma.

Chun, Tae Ho; Kim, Gook Ki; Lim, Young Jin; Kim, Hyung Doo; Kim, Tae Sung; Rhee, Bong Arm; Leem, Won

Original Article

Journal of Korean Neurosurgical Society 1995;24(12): 1568-1576.

Tae Ho Chun, Gook Ki Kim, Young Jin Lim, Hyung Doo Kim, Tae Sung Kim, Bong Arm Rhee, Won Leem

Department of Neurosurgery, School of Medicine, Kyung Hee University, Seoul, Korea.

ABSTRACT

Hemangioblastomas are histologically benign tumors but their clinical course is often unfavorable because of the high frequency of recurrence and it presents itself in multicentric forms with involvement of other organs. The tumor sometimes presents as a solid lesions, and is often extremely difficult to manage in contrast to the cystic hemangiolastoma. We present a retrospective analysis of 13 cases of posterior fossa hemangioblastoma diagnosed between 986 and 1993 at our institution, and the clinical characteristics, radiological and histological findings, treatment and outcome is discussed. There were nine males and four females with the mean age of 36.7 years(range 14 to 59) at the time of their first neurosurgical presentation Two patients were compatible with von-Hippel-Lindau complex, one of which was associated with retinal angioma and the other with multiple cysts in the pancreas and the kidney. Six patients had elevated hemoglobin levels which normalized postoperatively. In three out of 4 patients with recurrence of the tumor, the hemoglobin level remained elevated above 16gm/dl. The most common type of tumor in descending order was type II(simple cyst) and type IV(microcystic form) in one case each. The cerebellar hemisphere was the most common location of the tumor(76.9%), followed by the vermis and the brainstem. One case had a multiple location of the tumor. Angiographic studies demonstrate that the most common feeding artery of the mural nodule was the PICA(66.7%). In 4 patients who had a subtotal removal of the tumor mass at their initial operation, there was a recurrence between 2 and 9 years postoperatively. It is concluded from these studies that although hemangioblastomas may be histologically benign, its wide spectrum of clinical presentations, occurrence in multiple locations, and its tendency to recur if surgically excised subtotally renders the tumor a clinical challenge to manage surgically.

Key Words: Hemangioblastoma; Von Hippel-Lindau complex; Hemoglobin level; Recurrence; Multicentric from; Type