Trabelsi Saoussen, Mama Nadia, Chourabi Maroua, Mastouri Maroua Haddaji, Ladib Mohamed, Popov Sergey, Burford Anna, Mokni Moncef, Tlili Kalthoum, Krifa Hedi, Jones Chris, Yacoubi Mohamed Tahar, Saad Ali, Brahim Dorra H'mida-Ben
Department of Cytogenetics, Molecular Genetics and Reproductive Biology, Farhat Hached University Hospital, Sousse, Tunisia E-mail :
Asian Pac J Cancer Prev. 2015;16(16):6871-6. doi: 10.7314/apjcp.2015.16.16.6871.
The meningeal hemangiopericytoma (MHPC) is a vascular tumor arising from pericytes. Most intracranial MHPCs resemble meningiomas (MNGs) in their clinical presentation and histological features and may therefore be misdiagnosed, despite important differences in prognosis.
We report 8 cases of MHPC and 5 cases of MNG collected from 2007 to 2011 from the Neuro-Surgery and Histopathology departments. All 13 samples were re reviewed by two independent pathologists and investigated by immunohistochemistry (IHC) using mesenchymal, epithelial and neuro-glial markers. Additionally, we screened all tumors for a large panel of chromosomal alterations using multiplex ligation probe amplification (MLPA). Presence of the NAB2-STAT6 fusion gene was inferred by immunohistochemical staining for STAT6.
Compared with MNG, MHPCs showed strong VIM (100% of cases), CD99 (62%), bcl-2 (87%), and p16 (75%) staining but only focal positivity with EMA (33%) and NSE (37%). The p21 antibody was positive in 62% of MHPC and less than 1% in all MNGs. MLPA data did not distinguish HPC from MNG, with PTEN loss and ERBB2 gain found in both. By contrast, STAT6 nuclear staining was observed in 3 MHPC cases and was absent from MNG.
MNG and MHPC comprise a spectrum of tumors that cannot be easily differentiated based on histopathology. The presence of STAT6 nuclear positivity may however be a useful diagnostic marker.
脑膜血管外皮细胞瘤（MHPC）是一种起源于周细胞的血管性肿瘤。大多数颅内MHPC在临床表现和组织学特征上与脑膜瘤（MNG）相似，因此尽管预后存在重要差异，但仍可能被误诊。
我们报告了2007年至2011年从神经外科和组织病理学部门收集的8例MHPC和5例MNG。所有13个样本由两名独立病理学家重新审查，并使用间充质、上皮和神经胶质标记物通过免疫组织化学（IHC）进行研究。此外，我们使用多重连接探针扩增（MLPA）对所有肿瘤进行了一大组染色体改变的筛查。通过STAT6免疫组织化学染色推断NAB2-STAT6融合基因的存在。
与MNG相比，MHPC显示出强烈的波形蛋白（VIM）染色（100%的病例）、CD99（62%）、bcl-2（87%）和p16（75%）染色，但仅EMA（33%）和NSE（37%）呈局灶性阳性。p21抗体在62%的MHPC中呈阳性，而在所有MNG中阳性率不到1%。MLPA数据无法区分HPC和MNG，两者均发现PTEN缺失和ERBB2增加。相比之下，在3例MHPC病例中观察到STAT6核染色，而MNG中未观察到。
MNG和MHPC包括一系列基于组织病理学不易区分的肿瘤。然而，STAT6核阳性的存在可能是一种有用的诊断标志物。
