Gengler C, Guillou L. Solitary fibrous tumour and haemangiopericytoma: evolution of a concept

University Institute of Pathology, Rue du Bugnon 25, CH-1011 Lausanne, Switzerland.
Histopathology (Impact Factor: 3.45). 02/2006; 48(1):63-74. DOI: 10.1111/j.1365-2559.2005.02290.x
Source: PubMed


Haemangiopericytoma (HPC) was described in 1942 by Stout and Murray as a distinctive soft tissue neoplasm, presumably of pericytic origin, exhibiting a characteristic well-developed "staghorn" branching vascular pattern. Over the years, it appeared that this growth pattern was a non-specific one, shared by numerous, unrelated benign and malignant lesions, and that HPC was better considered as a diagnosis of exclusion. Three categories of lesion may now be individualized within the heterogeneous group of HPC-like neoplasms. The first category corresponds to those non-HPC neoplasms that occasionally display HPC-like features (e.g. synovial sarcoma). Lesions belonging to the second category show clear evidence of myoid/pericytic differentiation and correspond to true HPCs. They generally show a benign clinical course, and include glomangiopericytoma/myopericytoma, infantile myofibromatosis (previously called infantile HPC), and a subset of sinonasal HPCs. The third category is the solitary fibrous tumour (SFT) lesional group, which includes fibrous-to-cellular SFTs, and related lesions such as giant cell angiofibromas and lipomatous HPCs. In practice, any HPC-like lesion can be allocated to one of these categories, leaving the ill-defined "haemangiopericytoma" category empty.

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    • "Myxoid change is common. Likewise, immunohistochemistry-based studies revealed an expression profile of certain markers (CD34 80%, CD99 70%, and Bcell lymphoma-2 [BCL-2] 30%) in the vast majority of SFTP [9]. "
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    ABSTRACT: Solitary fibrous tumors of the pleura (SFTP) are rare neoplasms originating from submesothelial mesenchymal cells with fibroblastic differentiation. The clinical behavior of SFTPs is mostly benign; however, up to 20% of patients develop local recurrence and/or distant metastasis. Although different risk-stratification models have been described, definitive criteria to predict a malignant clinical course of SFTP are still lacking. In a retrospective analysis at a single-institution, 25 patients with histologically proven SFTP were identified. Clinicopathologic and survival data were collected and pathologic sections reviewed. Different markers and risk-stratification models were correlated with disease- and overall-free survival by Kaplan-Meier analysis. Of 25 SFTP, 8 tumors (32%) were classified as malignant according to the World Health Organization criteria. Three patients (12%) developed recurrence. Cohort median follow-up was 28 mo, and median overall survival was 160 mo. Comparison of proliferation markers showed higher mitosis count per high-power field and MIB-1 labeling index (MIB) in malignant compared with nonmalignant SFTP. MIB was identified as a predictor for disease-free survival. Applying the previously reported classifications to categorize SFTP according to the probability to show malignant behavior, significant differences in disease-free survival were also present in our cohort. In the present analysis of rare SFTP, previously proposed staging systems were applicable for prediction of disease-free survival. Independently of treatment, MIB was the only sole predictive marker. A prospective multi-institutional database could be helpful in establishing detailed predictive criteria in patients diagnosed with SFTP. Copyright © 2015 Elsevier Inc. All rights reserved.
    Journal of Surgical Research 01/2015; 195(2). DOI:10.1016/j.jss.2015.01.049 · 1.94 Impact Factor
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    • "SFT and hemangiopericytoma display immunoreactivity for CD34, Bcl-2 and CD99 antigens. Ultrastructural studies have demonstrated pericytic, fibroblastic, and myofibroblastic differentiation in both tumors [3] [16]. These findings have led pathologists to consider hemangiopericytoma and SFT as a spectrum of the same entity [22]. "
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    ABSTRACT: The solitary fibrous tumor (SFT) is a rare soft tissue tumor with a substantially benign clinical behavior. The SFT of the oral cavity is a very uncommon entity. It is also of complicated diagnosis because of its extensive morphologic diversity and because of its similarity to many mesenchymal tumors. A 44-year-old man was referred for management of an asymptomatic lesion in the left buccal mucosa, which had been identified 10 years earlier. Intra-oral examination revealed a well-demarcated, fibroelastic, rounded exophytic mass located in the left buccal mucosa. The mass was covered with a non-ulcerated mucosa of normal color and measured approximately 4.0cm in diameter. Histopathological examination showed proliferation of spindle-shaped cells arranged in fascicles and in a patternless pattern, highly vascularized, with focal staghorn vessels. Immunohistochemical analysis revealed diffuse positivity for CD34 and focal positivity for Bcl-2. Awareness of the morphological diversity of SFT coupled to a judicious use of appropriate immunohistochemical probes should prove valuable to accurately segregate SFT from other spindle cell neoplasms. Copyright © 2014 Elsevier GmbH. All rights reserved.
    Pathology - Research and Practice 12/2014; 210(12):1064-7. DOI:10.1016/j.prp.2014.09.018 · 1.40 Impact Factor
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    • "Hemangiopericytoma was first described in 1942 by Stout and Murray [1] as a distinct soft tissue neoplasm, presumably of pericyte origin, exhibiting a characteristic well-developed “staghorn” branching vascular pattern. However, hemangiopericytoma has been reclassified as a fibroblastic neoplasm similar to a solitary fibrous tumor (SFT) [2,3]. It typically affects adults aged 20–70 years, with a median age in the 40s [4,5]. "
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    ABSTRACT: Hemangiopericytoma is a rare disease entity of soft-tissue sarcoma (STS) that can be cured with surgical resection. In cases of inoperable recurrence or metastasis, palliative chemotherapy is indicated, though there is currently no approved chemotherapy regimen. Therefore new treatment regimens are needed. We describe three cases of metastatic hemangiopericytoma. In the first case, five lines of chemotherapeutic agents were used unsuccessfully in a patient with a 12-year history of metastatic hemangiopericytoma. After one cycle of pazopanib therapy, however, chest radiography showed a decrease in tumor volume of more than 30%. A marked decrease in FDG uptake on PET CT was also noted, and the patient is now on her 5th month of pazopanib therapy. The second case is a patient with a brain hemangiopericytoma with multiple liver, lung, and bone metastases. Pazopanib induced radiologic stabilization of metastatic disease over the course of 8 months. The third case is a patient with a retroperitoneal hemangiopericytoma with pleural and peri-renal metastases. For more than 8 months, he has exhibited stable disease with pazopanib treatment. Pazopanib may be useful for treatment of metastatic hemangiopericytoma, though further studies are needed to confirm the efficacy of this medication and to investigate its molecular mechanism of action.
    09/2014; 4(1):13. DOI:10.1186/2045-3329-4-13
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