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Neoplastic Stromal Cells of Intracranial Hemangioblastomas Disclose Pericyte-derived Mesenchymal Stromal Cells-like Phenotype

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Background: Stromal cells (SCs) of hemangioblastomas (HBs) have been regarded as true neo-plastic components, but their ontogeny remains unclear. Convincing evidence suggests that em-bryonic mesenchymal cells may be the cells of origin of HBs. The aim of the present study was to investigate the immunophenotypic characteristics of neoplastic SCs using a set of markers against endothelial cells (ECs), vascular smooth muscle cells (vSMCs), mesenchymal stromal cells (MSCs), and pericytes. Methods: Intracranial HBs (n=46), angiolipoma (n=9), and pyogenic granuloma (n=11) were retrieved and the immunophenotypic profile of SCs was determined by immune stainings. Results: The MIB-1 labeling index was significantly higher in SCs compared to that of ECs and vSMCs, regardless of the type of lesion. The neoplastic SCs of HBs consistently ex¬pressed both MSC and pericyte markers, but did not express markers of ECs and vSMCs. Dou¬ble immunofluorescent staining demonstrated that the neoplastic SCs of HBs expressing MSC or pericyte markers directly abutted onto the ECs of capillaries/venules. Conclusions: The results suggest that the neoplastic SCs of HBs share the immunophenotypic profile and distribution with those of pericyte-derived MSCs. Thus, HBs might originate from a distinctive population of peri-cyte-derived MSCs in the central nervous system.

Abstract

Background: Stromal cells (SCs) of hemangioblastomas (HBs) have been regarded as true neo-plastic components, but their ontogeny remains unclear. Convincing evidence suggests that em-bryonic mesenchymal cells may be the cells of origin of HBs. The aim of the present study was to investigate the immunophenotypic characteristics of neoplastic SCs using a set of markers against endothelial cells (ECs), vascular smooth muscle cells (vSMCs), mesenchymal stromal cells (MSCs), and pericytes. Methods: Intracranial HBs (n=46), angiolipoma (n=9), and pyogenic granuloma (n=11) were retrieved and the immunophenotypic profile of SCs was determined by immune stainings. Results: The MIB-1 labeling index was significantly higher in SCs compared to that of ECs and vSMCs, regardless of the type of lesion. The neoplastic SCs of HBs consistently ex¬pressed both MSC and pericyte markers, but did not express markers of ECs and vSMCs. Dou¬ble immunofluorescent staining demonstrated that the neoplastic SCs of HBs expressing MSC or pericyte markers directly abutted onto the ECs of capillaries/venules. Conclusions: The results suggest that the neoplastic SCs of HBs share the immunophenotypic profile and distribution with those of pericyte-derived MSCs. Thus, HBs might originate from a distinctive population of peri-cyte-derived MSCs in the central nervous system.

참고문헌 (30)

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