Abstract
Objective: Primary intracranial or spinal hemangiopericytoma (HPC) represents a rare tumour that is more difficult to distinguish from other CNS tumours based on clinical symptoms and imaging findings. In this article, here we present 5 cases with HPC (3 intracranial and 2 spinal) with histologically confirmed were treated at our hospital between June 2009 to January 2010, and a review of the literature pertaining to the diagnosis, clinical management, and so on. Methods: Clinical data of the 5 patients with HPCs from cerebra and spinal cord including the manifestations of imaging, pathology, treatment and prognosis factors were investigated, and relevant literatures were reviewed. Of which, 3 males, 2 females, age ranged from 6-35 years old, median age at primary diagnosis was 29 years. Results: 3 patients underwent craniotomy and the other 2 patients underwent spinal surgery. Total mass removal was achieved in 1 case and subtotal removal in 4 cases. All 5 patients received radiotherapy after resection, A total dose of 59.4 Gy/33f was delivered in a fractionation of 5 x1.8 Gy per week for the 3 patients with cerebral lesions using three dimensional conformal radiation therapy (3D-CRT) or intensive modulated radiation therapy (IMRT) technique, and 45Gy in 25 fractions for the 2 patients with spinal lesions. The patients were followed up varied from 6 to 27 months, and none of the patients was found with evidence of radiation complications. Acute toxicity was mild including skin erythema and alopecia. Follow-up of the patients has not yet discovered that a tumour recurrence and extra-cranial metastasis all remain alive up to date. Conclusions: Multidisciplinary care should be highly advocated in the management of intracranial or spinal HPC. Surgical resection, either complete or subtotal excision, followed by postoperative radiotherapy, will provide the patients with higher probability for disease-free survival.
Highlights
Hemangiopericytoma (HPC, or HAP) of the central nervous system (CNS) is a rare vascularized mesenchymal tumour that originate from pericytes, the contractile cells surrounding capillaries, and develops from malignant transformation of pericytes, it can occur anywhere in the human body because of the ubiquity of pericytes in all mesenchymal tissues, especially in bone, kidney, liver, skin, and central nervous system [1]
Intracranial HPC accounts for 2% ~ 4% of meningeal tumour, and it remains a rare entity and has been estimated to represent 0.4% of all primary central nervous system tumours, there is no significant difference of the HPC incidence in gender, and ages of around 40-50 are most commonly affected, the natural course of the disease can be very long
We report our experience from June 2009 to January 2010 in treating five patients with intracranial HPC using radiotherapy after tumour surgical resection, and evaluate the role of three dimensional conformal radiation therapy (3D-CRT) and intensive modulated radiation therapy (IMRT) in the management of the HPC of the CNS, and offer an analysis of treatment outcomes based on our experience
Summary
Hemangiopericytoma (HPC, or HAP) of the central nervous system (CNS) is a rare vascularized mesenchymal tumour that originate from pericytes, the contractile cells surrounding capillaries, and develops from malignant transformation of pericytes, it can occur anywhere in the human body because of the ubiquity of pericytes in all mesenchymal tissues, especially in bone, kidney, liver, skin, and central nervous system [1]. Microscopic examination typically reveals highly cellular tumours, with round or fusiform tumour cells demonstrating ill-defined cytoplasm, oval nuclei, and small prominent nucleoli, and more common nuclear fission [2,3]. The immunohistochemical profile of HPC of the CNS differs from that of meningioma and is imperative in securing the diagnosis. Whereas both meningiomas and HPCs are often vimentin positive, reflecting their mesenchymal origin. When the tumour is small, imaging often misdiagnosed as meningioma. MRI examination of these tumours is typically isointense with cortical gray matter on T1WI and isointense to mildly hyper-intense on T2WI. Contrast enhancement images typically demonstrate intensely enhancing extraaxial lesions, often with a discernable dural attachment.
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