Derenda Marek, Borof Damian, Kowalina Ireneusz, Wesołowski Wojciech, Kloc Wojciech, Iżycka-Świeszewska Ewa
Department of Neurosurgery, Regional Hospital, Elblag, Poland.
Department of Pathology, Regional Hospital, Elblag, Poland.
Surg J (N Y). 2017 Jul 20;3(3):e117-e123. doi: 10.1055/s-0037-1604159. eCollection 2017 Jul.
Mesenchymal chondrosarcomas (MCSs) are rare malignant tumors of the bone and soft tissues. Only a few cases of such tumors originating from the spinal canal meninges have been described in the literature. The authors report on a case of a 22-year-old woman with MCS of the arachnoid at the T12-L1 level with a 14-year-long observation. The tumor was totally resected using osteoplastic laminotomy with reconstruction of laminar roof. This small spindle cell tumor was initially microscopically suspected of synovial sarcoma, but correctly verified with widened immunophenotyping and molecular studies as MCS. At its first recurrence, the neoplasm showed microscopically a typical bimorphic pattern of small round cell component with foci of hyaline cartilage. The patient experienced three local recurrences: 4, 6, and 10 years after the initial resection, respectively. The techniques of laminotomy and relaminotomy were also used during three following operations. The repeated surgical removal, radiotherapy, and chemotherapy were the methods of complex oncological treatment. The patient remains now in complete remission, fully self-dependent with slight motor disturbance, and mild sensory deficits. Current views on the clinicopathological characteristics and treatment modalities of the chondrosarcomas of the spinal canal are discussed.
间叶性软骨肉瘤(MCS)是一种罕见的骨与软组织恶性肿瘤。文献中仅描述了少数几例起源于椎管脑膜的此类肿瘤。作者报告了一例22岁女性患者,其T12 - L1水平的蛛网膜发生间叶性软骨肉瘤,并进行了长达14年的观察。采用骨成形性椎板切开术并重建椎板顶对肿瘤进行了全切。这种小梭形细胞瘤最初在显微镜下怀疑为滑膜肉瘤,但通过扩大免疫表型分析和分子研究正确地确认为间叶性软骨肉瘤。首次复发时,肿瘤在显微镜下呈现出典型的双相模式,即小圆形细胞成分伴有透明软骨灶。患者经历了三次局部复发,分别在初次切除后的4年、6年和10年。在随后的三次手术中也采用了椎板切开术和再次椎板切开术。反复手术切除、放疗和化疗是综合肿瘤治疗的方法。患者目前处于完全缓解状态,完全自理,但有轻微运动障碍和轻度感觉障碍。本文讨论了目前关于椎管软骨肉瘤临床病理特征和治疗方式的观点。