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曲贝替定单药治疗复发性转移性间叶性软骨肉瘤的长期缓解。

Long lasting response with trabectedin monotherapy in relapsed metastatic mesenchymal chondrosarcoma.

作者信息

Tansir Ghazal, Rastogi Sameer, Barwad Adarsh, Dhamija Ekta

机构信息

Sarcoma Medical Oncology Clinic, All India Institute of Medical Sciences (AIIMS), New Delhi, India.

Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Clin Sarcoma Res. 2020 Aug 27;10:16. doi: 10.1186/s13569-020-00138-4. eCollection 2020.

Abstract

BACKGROUND

Mesenchymal chondrosarcoma is an exceedingly rare malignancy, accounting for around 5% of all patients with chondrosarcoma. It is a translocation-related sarcoma that tends to have both local and distant recurrences. Surgery is the mainstay of treatment in localised cases however treatment of advanced cases remains a challenge. The rarity of the disease precludes dedicated clinical trials and hence guidelines for its management are not well defined. The dearth in literature makes it pertinent that the cases treated with newer therapies must be reported to contribute to existing knowledge.

CASE PRESENTATION

We hereby report a case of a 39-year old male without any comorbidity presenting with pelvic pain and was diagnosed as mesenchymal chondrosarcoma of the pelvis. He underwent an initial curative resection followed by a disease-free interval of 7 months. Subsequently, he was treated with pulmonary metastatectomy and local debulking surgery at time of initial relapse. He was then exposed to multiple lines of palliative chemotherapy, which limited our treatment options upon subsequent disease progression. Based on recent data, the patient was given trabectedin monotherapy as fourth line chemotherapy. He tolerated the therapy well and attained a progression-free survival of 12 months, which is an impactful figure in relapsed setting in this patient population.

CONCLUSION

This report aims to present a comprehensive review into available and newer treatment choices for mesenchymal chondrosarcoma, and to highlight trabectedin monotherapy as a possible therapeutic option for mesenchymal chondrosarcoma in the relapsed setting.

摘要

背景

间叶性软骨肉瘤是一种极为罕见的恶性肿瘤,约占所有软骨肉瘤患者的5%。它是一种与易位相关的肉瘤,往往会出现局部和远处复发。手术是局限性病例的主要治疗方法,然而晚期病例的治疗仍然是一项挑战。该疾病的罕见性使得专门的临床试验无法开展,因此其管理指南尚不明确。文献资料的匮乏使得有必要报告采用新疗法治疗的病例,以丰富现有知识。

病例介绍

我们在此报告一例39岁男性,无任何合并症,因骨盆疼痛就诊,被诊断为骨盆间叶性软骨肉瘤。他最初接受了根治性切除,随后有7个月的无病间期。随后,在初次复发时,他接受了肺转移瘤切除术和局部减瘤手术。然后,他接受了多线姑息化疗,这限制了我们在后续疾病进展时的治疗选择。根据最近的数据,该患者接受了曲贝替定单药治疗作为四线化疗。他对该治疗耐受性良好,无进展生存期达到12个月,这在该患者群体的复发情况下是一个有意义的数字。

结论

本报告旨在全面综述间叶性软骨肉瘤现有的和新的治疗选择,并强调曲贝替定单药治疗作为间叶性软骨肉瘤复发情况下一种可能的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f9a1/7453529/1353dbe7c2e5/13569_2020_138_Fig1_HTML.jpg

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