Mayo Clinic, Dept of Anatomic Pathology, Hilton 11, 200 First St, SW, Rochester, MN 55905;
Am J Clin Pathol. 2013 Nov;140(5):670-4. doi: 10.1309/AJCPGUNGP52ZSDNS.
Meningeal hemangiopericytoma (HPC) and mesenchymal chondrosarcoma are aggressive neoplasms with a propensity to involve the meninges and dura. In addition to similar clinical presentations, both meningeal HPC and mesenchymal chondrosarcoma share overlapping morphologic features, including ovoid cells, variable collagen deposition, and a branching vascular pattern. Recently, a novel HEY1-NCOA2 fusion was reported as a recurrent event in mesenchymal chondrosarcomas.
Thirteen mesenchymal chondrosarcomas and 18 meningeal HPCs were identified from surgical pathology archives, and the tumors were evaluated for HEY1-NCOA2 fusion with reverse transcriptase-polymerase chain reaction (RT-PCR).
HEY1-NCOA2 fusion transcript was detected in all six cases of mesenchymal chondrosarcoma but in none of the meningeal HPC cases (0/11) that were evaluable with RT-PCR.
These results show that (1) meningeal HPC and mesenchymal chondrosarcoma are distinct at the molecular level, and (2) the identification of HEY1-NCOA2 can be used as an auxiliary diagnostic tool to differentiate these entities.
脑脊膜血管外皮细胞瘤(HPC)和间叶性软骨肉瘤是具有脑膜和硬脑膜侵袭倾向的侵袭性肿瘤。除了相似的临床表现外,脑脊膜 HPC 和间叶性软骨肉瘤还具有重叠的形态学特征,包括卵圆形细胞、可变的胶原沉积和分支状血管模式。最近,一种新型的 HEY1-NCOA2 融合被报道为间叶性软骨肉瘤的复发性事件。
从外科病理档案中确定了 13 例间叶性软骨肉瘤和 18 例脑脊膜 HPC,并通过逆转录聚合酶链反应(RT-PCR)评估这些肿瘤中是否存在 HEY1-NCOA2 融合。
在可通过 RT-PCR 评估的 6 例间叶性软骨肉瘤中均检测到 HEY1-NCOA2 融合转录本,但在 11 例脑脊膜 HPC 中均未检测到(0/11)。
这些结果表明:(1)脑脊膜 HPC 和间叶性软骨肉瘤在分子水平上是不同的;(2)HEY1-NCOA2 的鉴定可作为辅助诊断工具,用于区分这些实体。