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脊柱间叶性软骨肉瘤:一例罕见恶性肿瘤的病例报告

Spinal mesenchymal chondrosarcoma: A case report of a rare malignant tumor.

作者信息

Dehneh Younes, Aldabbas Mohannad, Elfarissi Mohammed Alamine, Khoulali Mohamed, Oulali Noureddine, Moufid Faycel

机构信息

Department of Neurosurgery, University Hospital Center Mohammed VI Oujda, Morocco.

Faculty of Medicine and Pharmacy, Mohammed Premier Oujda, Morocco.

出版信息

Surg Neurol Int. 2023 Apr 7;14:128. doi: 10.25259/SNI_206_2023. eCollection 2023.

Abstract

BACKGROUND

Mesenchymal chondrosarcoma is an uncommon malignant variant of chondrosarcoma that mainly affects the bones and cartilaginous tissues, but may rarely involve the spine. Careful preoperative planning for surgical tumor removal and spine reconstruction is mandatory and must be based on oncologic and surgical staging.

CASE DESCRIPTION

Over 1 month, a 16-year-old female became paraplegic with a T9 sensory level and urinary dysfunction. The magnetic resonance imaging revealed an intraspinal extradural T7-T9 mass that was isointense in T1W1 and markedly enhanced with gadolinium. The patient underwent gross-total tumor resection followed by an osteoplastic laminectomy with fusion. The histological examination was consistent with a mesenchymal chondrosarcoma. She had received radiation and chemotherapy. One year later, she was readmitted for tumor recurrence with multiple metastases involving L1, the lung, and peritoneum. Despite full course of radiotherapy and chemotherapy, she died after 6 months of the second surgery.

CONCLUSION

Total resection of mesenchymal chondrosarcomas is the gold standard for treatment and is typically followed by radiation and/or chemotherapy. However, the status of residual tumor, local extension, and or metastases best determine the overall survival which may prove extremely limited.

摘要

背景

间叶性软骨肉瘤是软骨肉瘤的一种罕见恶性变异类型,主要累及骨骼和软骨组织,但很少累及脊柱。术前仔细规划手术切除肿瘤及脊柱重建是必要的,且必须基于肿瘤学和手术分期。

病例描述

一名16岁女性在1个多月的时间里出现截瘫,感觉平面在T9,伴有排尿功能障碍。磁共振成像显示T7 - T9椎管内硬膜外肿块,在T1加权像上呈等信号,钆增强后明显强化。患者接受了肿瘤全切术,随后进行了椎板成形术并融合。组织学检查结果符合间叶性软骨肉瘤。她接受了放疗和化疗。1年后,她因肿瘤复发再次入院,出现多处转移,累及L1、肺和腹膜。尽管接受了全程放疗和化疗,但在第二次手术后6个月死亡。

结论

间叶性软骨肉瘤的全切术是治疗的金标准,通常随后进行放疗和/或化疗。然而,残余肿瘤的状态、局部扩展和/或转移情况最能决定总体生存期,总体生存期可能非常有限。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3381/10159291/bee2e2c21f73/SNI-14-128-g001.jpg

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