Intracranial Hemangiopericytoma – A Case Report (original) (raw)

Intracranial Hemangiopericytoma—Our Experience in 30

2013

Meningeal hemangiopericytoma (HPC) is a rare, aggressive central nervous system tumor that tends to invade locally and to metastasize, and has a high rate of recurrence.This study presents a retrospective review of patients managed for intracranial HPC at Rome University Hospital.A total of 43 patients with intracranial HPC were treated from 1980 to 2010. Treatment and follow-up information was available for analysis on 36 patients. The median survival for all patients was 83.5 months after date of diagnosis, with 1-year, 5-year, and 10-year survival rates of 100%, 94.4%, and 72.2%, respectively. Eighteen patients (41.86%) had HPC recurrence. The median time until recurrence was 72.24 months, with 1-year, 5-year, and 10-year progression-free survival rates of 98%, 51%, and 29%, respectively. Five patients (11.62%) developed extracranial metastasis. Patients undergoing any form of adjuvant radiation treatment, including external beam radiotherapy, Gamma Knife radiosurgery, and/or proton beam therapy, had no longer median overall survival (OS) (178 vs. 154 months, respectively; P = .2); but did have a significantly improved recurrence-free interval (108 vs. 64 months; P = .04) compared with patients who did not undergo radiation treatment. Tumor characteristics associated with earlier recurrence included size ≥7 cm (log-rank, P < .05) and sinus invasion (log-rank, P < .05).Strategies combining adjuvant radiation with tumor resection seemed to hinder tumor progression, but had no effect on OS or the development of metastases. Greater extent of resection was associated with increased OS (log-rank, P < .05). Anaplastic HPC was associated with reduced OS and with reduced recurrence interval (log-rank, P < .05).

Intracranial Hemangiopericytoma: Study of 12 Cases

Acta Neurochirurgica, 2001

Most hemangiopericytomas (HPCs) are located in the musculoskeletal system and the skin, while the intracranial location is rare. They represent 2 to 4% in large series of meningeal tumours, thus accounting for less than 1% of all intracranial tumours. Many authors have argued about the true origin of this tumour. The current World Health Organization classification of Central Nervous System tumours distinguishes HPC as an entity of its own, and classified it into the group of “mesenchymal, non-meningothelial tumours”. Radical surgery is the treatment of choice, but must be completed with postoperative radiotherapy, which has proved to be the therapy most strongly related to the final prognosis. HPCs have a relentless tendency for local recurrence and metastases outside the central nervous system which can appear even many years after diagnosis and adequate treatment of the primary tumour. Twelve patients with intracranial HPC were treated at our Unit between 1978 and 1999. There were 10 women and 2 men. Ten tumours were supratentorial and most located at frontoparietal parasagittal level. The most common manner of presentation was a focal motor deficit. All tumours were hyperdense in the basal Computed Tomography scans and most enhanced homogeneously following intravenous contrast injection. In 50% of cases, tumour margins were irregular or lobulated. Seven tumours were studied with Magnetic Resonance Imaging, being six of them iso-intense with the cortical gray matter on T1-weighted and T2-weighted images. Twenty operations were performed in the 12 patients. In 10 cases radical excision could be achieved with no operative mortality. Total recurrence rate was 33.3%. Eight patients were treated with external radiotherapy at some time through the course of their disease. Eight out of the 12 patients in this series are disease-free (Glasgow Outcome Scale categories 1 and 2) after a mean follow up of 52 months.

Intracranial hemangiopericytoma: A case report and review of the literature

2012

Abstract Intracranial hemangiopericytomas (HPCs) are rare central nervous system tumors arising from Zimmermann pericytes or mesenchymal cells. It is often difficult to distinguish these tumors from meningiomas based on clinical features and radiological findings. HPCs have been clinically detected in many of the intracranial compartments; here, we present a case with presumed meningioma that was adjacent to the right lateral ventricle and was confirmed by histopathology analysis.

Recurrent intracranial hemangiopericytoma with extracranial and unusual multiple metastases: case report and review of the literature

Tumori

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 ...

Combined Surgical Approach to Intracranial and Extracranial Hemangiopericytoma: Case Report and Literature Review

Cureus

Hemangiopericytoma (HPC) is a rare vascular tumor that was first described in 1942 and whose classification and treatment continue to develop. The proper classification for HPC is still under discussion, being considered a solitary fibrous tumor (SFT), classified as an aggressive biological form. The World Health Organization (WHO) has considered it to be part of extrapleural solitary fibrous tumors, however, neuropathologists still consider it to be an HPC when it is found in the central nervous system. We present a case of a patient with HPC of complex localization in the infratemporal fossa and middle floor of the skull base, which confirmed the diagnosis of HPC after resection by the craniofacial approach. Hemangiopericytomas are tumors that can present along with distant metastasis in 23% of cases even after resection. Surgery is the therapeutic basis; however, the still-controversial pathological classification of these vascular tumors and their uncertain biological behavior are the main reasons the ideal treatment continues to be investigated.

Management of intracranial meningeal hemangiopericytomas: outcome and experience

Neurosurgical Review, 2006

Hemangiopericytomas represent rare intracranial tumors that have a tendency to recur locally and have the unique characteristic of giving extracranial metastases. Our current communication reviews a series of patients diagnosed with hemangiopericytoma who were treated in our facility. Eleven patients with a mean age of 51.2 years underwent follow-up for a mean time of 7.1 years. Their neuroimaging preoperative evaluation included plain skull X-rays, head CT scans, brain MRI, angiograms, and (1)HMRS. Preoperative embolization of the tumor was employed in 6/11 patients. All patients underwent craniotomy for tumor resection and postoperative radiation treatment was employed on all but one. Grade I resection was accomplished in 6/11 (54.5%), grade III in 4/11 (36.4%), and grade IV in 1/11 (9.1%). Local recurrence was detected in 3/11 (27.3%) at a mean period of 5 (range 2-7.5) years. Extracranial metastatic disease was documented in 4/11 (36.4%) patients at a mean of 4.9 (range 2.5-7) years after the initial diagnosis. The GOS score was: 7/11 (63.6%) scored 5, while 4/11 (36.4%) died at a mean time of 5.5 (range 3-8) years after the initial diagnosis. Intracranial hemangiopericytomas management requires aggressive surgical resection, postoperative radiation treatment, and extensive follow-up to rule out local recurrences and delayed extracranial metastases.

Review Article Hemangiopericytoma: A Case Report and Mini-Review

2015

The term hemangiopericytoma was first used by Stout and Murray in 1942 describing a tumor which is distinguished histologically from other types of vascular neoplasm by its proliferation of pericytes. Hemangiopericytoma is a rare neoplasm that was originally described as a vascular tumor derived from the pericytes. They account for 2-3% of all soft tissue sarcomas in humans and they occur mainly in the musculoskeletal system. 15-30% of all hemangiopericytomas occur in the head and neck region. Only 5% are located in the sinonasal region, where they display a more benign behavior than in other parts of the body. Herein, we are presenting an extremely rare case report of hemangiopericytoma in a 32 year old male patient with a brief overview regarding its epidemiology, macro-and microscopical characteristics, the clinic-pathological findings and the treatment of this extremely rare vascular neoplasm.