Department of Histopathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Department of Radiotherapy, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Neurol India. 2022 Jan-Feb;70(1):285-288. doi: 10.4103/0028-3886.338726.
Extraskeletal mesenchymal chondrosarcoma (MCS) of the central nervous system (CNS) is extremely rare. Herein, we present the clinicopathological features of five CNS extraskeletal MCS.
Over the past 10 years, five cases of CNS MCS have been retrieved from in the archives of histopathology department. All biopsies were stained with vimentin, S-100, CD99, desmin, GFAP, INI1, WT1, STAT6, and EMA.
There were four males and one female patient in the age group of 1.5-35 years. The clinical and radiological impression was meningioma in three cases, glomus jugulare and primitive neuroectodermal tumor in one case each. All showed classic biphasic morphology, areas of undifferentiated small blue round cells sharply demarcated from the island of cartilage. Three patients experienced multiple recurrences and died subsequently.
Extraskeletal MCS of CNS is rare and favors children and young adults. They show aggressive behavior and tend to recur despite surgery and radiotherapy.
中枢神经系统（CNS）的骨外间叶性软骨肉瘤（MCS）极为罕见。本文报告了 5 例 CNS 骨外间叶性软骨肉瘤的临床病理特征。
在过去的 10 年中，我们从组织病理学部门的档案中检索到 5 例 CNS MCS 病例。所有活检均用波形蛋白、S-100、CD99、结蛋白、GFAP、INI1、WT1、STAT6 和 EMA 染色。
患者年龄 1.5-35 岁，男 4 例，女 1 例。3 例临床和影像学表现为脑膜瘤，1 例为颈静脉球瘤，1 例为原始神经外胚层肿瘤。所有病例均表现为经典的双相形态，未分化的小蓝圆形细胞区与软骨岛截然分开。3 例患者经历了多次复发，随后死亡。
中枢神经系统的骨外间叶性软骨肉瘤罕见，好发于儿童和青年。尽管经过手术和放疗，它们仍具有侵袭性，且易于复发。
