Intracranial hemangiopericytoma

Abstract

INTRODUCTION:Intracranial hemangiopericytoma (HPC) is a malignant meningothelial tumor. Because of its rarity, few guidelines exist for optimal management. METHODS: University of California at San Francisco patients managed for intracranial HPC were compiled into a single database based on a retrospective review of patient records. Univariate and multivariate regression was performed to determine factors that independently predicted treatment outcomes. RESULTS: A total of 40 patients with intracranial HPC were treated from 1989 to 2010. Treatment and follow-up information was available for analysis on 35 patients. The median survival for all patients was 16.2 years after date of diagnosis, with 1-year, 5-year, and 10-year survival rates of 100%, 92%, and 68%, respectively. Nineteen patients (54%) had HPC recurrence. The median time until recurrence was 5 years, with 1-year, 5-year, and 10-year progression-free survival rates of 96%, 49%, and 28%, respectively. Seven patients (20%) developed extracranial metastasis. Tumor characteristics associated with earlier recurrence included size !6 cm (log-rank, P < .05) and nonskull base location (log-rank, P < .05). Strategies combining adjuvant radiation with tumor resection appeared to hinder tumor progression, but had no effect on overall survival or the development of metastasis. Greater extent of resection was associated with increased overall survival (log-rank, P < .05). CONCLUSIONS: Adjuvant radiation may show promise in preventing tumor progression, but recurrence remains a common treatment outcome regardless of initial strategy. When safe and feasible, gross total resection should be pursued as an initial surgical strategy to maximize overall survival. The propensity of these tumors to metastasize makes detailed staging imaging necessary.

Chewing-Gum as a Confounder for Intracranial Haemangiopericytoma Oral Metastasis

“…Upon finding an anomalous shaped hyperdense image on surveillance CT, as in our case, dangerous diagnoses such as a metastasis must be ruled out readily. This was especially important in our patient, due to the presence of early recurrence risk factors, notably a ≥ 6 cm sized primary lesion, non-skull base location, a first STR surgery, local recurrence, and tumour grade (anaplastic) [1].…”

“…Local recurrence rates are around 55%, the majority between 1-5 years after initial treatment. Metastasis rates are about 20%, and mortality at ten years is 35% [1]. The extracranial head and neck location of primary or recurrent disease is also uncommon, at 16% of all HPC.…”

Rare Primary Central Nervous System Tumors

“…It is now commonly classified as WHO grade II, with an anaplastic variant classified as WHO grade III. 35,36 Grossly, hemangiopericytomas are solitary and well circumscribed tumors. 37 They often have grossly visible vascular structures.…”

“…They develop from malignant transformation of pericytes, which are contractile spindle cells surrounding the walls of capillaries and post-capillary venules. 35 Morphologically, hemangiopericytomas are remarkably similar to meningioma. To distinguish the two tumors, a number of immunohistochemical and genetic markers have been developed.…”

“…37 Reported rates of complete resection range from 30-70%. 35,36,40-42 Greater extent of resection has been associated with improved survival. 35 With surgery alone, local recurrence rate has been reported to be as high as 90%, and relapse can occur as early as 7 months after the initial treatment.…”