2 Cases of Multifocal and Recurrent Hemangioblastomas with Von Hippel-Lindau Syndrome: Case Report

Hwang, Sung Kyun; Oh, Chang Wan; Kim, Hyun Jib; Choi, Kil Soo; Han, Dae Hee

Abstract

Hemangioblastomas are benign tumors that most commonly occur in the posterior fossa around the 4th ventricle. Recurrent mutifocal hemangioblastomas are often found as a part of a systemic autosomal dominant disease, the Von Hippel-Lindau syndrome. Surgical removal of recurrent multifocal tumors are technically more difficult and challenging than solitary ones due to their proximity to critical structures, disturbed anatomical landmark and adehesion to the surrounding tissue, all of which may lead to high postoperative morbidity and mortality. Authors have experienced 2 cases of the multiple and recurrent hemangioblastomas with Von Hippel-Lindau syndrome. The patients of each of the cases underwent surgery for tumor removal at least 2 times. In the first patient, a 42-year-old male, a solitary cerebellar hemangioblastoma had been removed 6 years prior to recurrence. On followup MRI, more than three solid, homogenously enhanced nodules were found in the cerebellum. These tumors were operated on through the previous craniectomy site, but only two of them could be removed. This patient was discharged without additional neurologic deficit. The 2nd patient, a 40-year-old-male, had undergone three prior operations for cerebellar hemangioblastomas. On followup examination, he presented with progressive ataxia and confusion. Brain MRI showed multiple recurrent lesions of homogenously strongenhanced masses in the 4th ventricle and cerebellar vermis surrounded by cystic lesions, accompanied by obstructive hydrocephalus. No additional surgery of tumors were attempted, and only ventriculoperitoneal shunt was performed. He was discharged with improved neurological symtomes and sign.