Szumera-Ciećkiewicz Anna, Ptaszyński Konrad, Grabowski Piotr, Krajewski Romuald, Tacikowska Małgorzata
Department of Pathology, Maria Sklodowska-Curie Memorial Cancer Center, Warsaw, Poland.
Pol J Pathol. 2012 Mar;63(1):80-4.
Mesenchymal chondrosarcoma (MChS) is a rare, high-grade malignant tumor which occurs both in the bone and soft tissue. The extraskeletal location comprises one third of all MChS and in review of the up-to-date literature, about 30 cases of the orbital involvement were found. The authors present clinical, radiological and pathological findings of two cases of MChS of the orbit occurring in young adult females: primary extraskeletal MChS of the orbit and skeletal MChS of the ethmomaxillary complex with secondary orbit involvement. The histopathological examination revealed a characteristic biphasic pattern composed of small round to spindle-shaped cells, mimicking Ewing sarcoma family of tumors, with areas of a haemangiopericytoma-like pattern and admixed cartilage foci. One of the patients had local recurrence 3 years after initial surgical removal. Subsequently, she underwent enucleation followed by chemotherapy. The other patient had a biopsy and debulking resection of the tumor and started chemotherapy. Ten months follow-up of this patient show no evidence of metastasis.
间叶性软骨肉瘤(MChS)是一种罕见的高级别恶性肿瘤,可发生于骨骼和软组织。骨外部位占所有MChS的三分之一,在查阅最新文献时,发现约有30例眼眶受累病例。作者介绍了两例发生在年轻成年女性的眼眶MChS的临床、放射学和病理学发现:原发性眼眶骨外MChS和筛上颌复合体骨MChS伴继发性眼眶受累。组织病理学检查显示出一种特征性的双相模式,由小圆形至梭形细胞组成,类似于尤因肉瘤家族肿瘤,伴有血管外皮细胞瘤样区域和混合的软骨灶。其中一名患者在初次手术切除3年后出现局部复发。随后,她接受了眼球摘除术并进行了化疗。另一名患者进行了肿瘤活检和减瘤切除术,并开始化疗。对该患者进行10个月的随访未发现转移迹象。