Recurrent Intracranial Hemangiopericytoma with Extracranial and Unusual Multiple Metastases: Case Report and Review of the Literature

Abstract

Hemangiopericytoma is a rare tumor with uncommon location in the central nervous system. It has only recently been included (WHO classification 1993) in a specific group of CNS tumors and subsequently (WHO classification 1997 and 2000) as a group by itself, while before it was confused with meningeal tumors. We report on a case of a 48-year-old woman affected by this tumor. The neoplasm was located in the posterior fossa. The patient underwent primary surgery in 1990, not followed by any adjuvant therapy because of the histopathological diagnosis of meningioma. After being free from disease for eight years she developed a local recurrence in 1998. Subtotal excision of the tumor, which was finally identified as a hemangiopericytoma, was carried out, followed by adjuvant radiotherapy (64 Gy). After six months multiple metastases were found in the liver and right kidney. A radical metastasectomy was performed, followed by systemic chemotherapy. One year later (2001) the tumor recurred again intracranially and a metastases was detected in the right breast, so the patient again underwent cranial irradiation (40 Gy) and second-line chemotherapy. She died in September 2002, 12 years after the diagnosis. We may conclude that, despite the tumor's natural tendency to recur several times and the ability of intracranial hemangiopericytoma to spread outside the CNS, it is possible to ensure a long survival time.