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一名年轻亚洲女性因内生软骨瘤引发胸壁继发性软骨肉瘤。

Chest wall secondary chondrosarcoma arising from enchondroma in a young Asian female.

作者信息

Nieh Chih Chiang, Chua Yang Chong, Thirugnanam Agasthian, Hlwan Moe Htet

机构信息

Department of Cardiac, Thoracic and Vascular Surgery, National University Hospital, Singapore.

Department of Cardiac, Thoracic and Vascular Surgery, National University Hospital, Singapore.

出版信息

Int J Surg Case Rep. 2014;5(12):968-71. doi: 10.1016/j.ijscr.2014.10.008. Epub 2014 Oct 18.

Abstract

INTRODUCTION

Enchondromas are benign tumours derived from cartilaginous tissue and they rarely undergo malignant transformation unless they occur in multiple sites such as in the case of Ollier disease.

PRESENTATION OF CASE

We report a rare case of a 36-year-old Chinese female, without any past medical history, who had a right chest wall tumour which was initially diagnosed to be benign, for which she was loss to follow up. The mass had been stable in size for the past 7 years until recently, when it gradually grew bigger. computed tomography (CT) scan of the thorax revealed that the mass had invaded the right chest wall suggestive of malignant change. She subsequently underwent an elective open right chest wall resection of the mass and chest wall reconstruction with mesh. A 3cm by 3cm tumour arising from the anterior aspect of the 4th rib was found intra-operatively. Post-operative course was uneventful. Histologically, the mass was confirmed to be chrondrosarcoma and resection margins were negative. The patient was discharged on 4th post-operative day and her latest CT thorax during follow up 2 years after operation did not show any tumour recurrence.

DISCUSSION

The aim of chest wall reconstruction is to avoid instability and prevent thoracic organs from being exposed if the defect size is ≥10cm or at least three ribs are resected. Polypropylene mesh may be relatively weak in terms of mechanical support for the chest wall, however, in this case, the defect is not too large and the laterally reflected pectoral muscle can provide enough support together with the mesh.

CONCLUSION

Secondary chondrosarcoma in a young Asian female arising from a benign solitary enchondroma in the absence of multiple enchondromatosis, such as Ollier disease or Maffucci syndrome, is extremely rare. Regular follow up including physical assessment and radiological imaging can result in earlier detection of malignant transformation obviating the need for aggressive surgical treatment involving multiple rib resections.

摘要

引言

内生软骨瘤是源自软骨组织的良性肿瘤,除非发生在多个部位,如 Ollier 病,否则很少发生恶性转化。

病例介绍

我们报告一例罕见病例,一名 36 岁的中国女性,无既往病史,患有右胸壁肿瘤,最初被诊断为良性,随后失访。在过去 7 年中,肿块大小一直稳定,直到最近逐渐增大。胸部计算机断层扫描(CT)显示肿块侵犯右胸壁,提示发生恶变。她随后接受了择期开放性右胸壁肿块切除术及胸壁网状重建术。术中发现一个 3cm×3cm 的肿瘤,起源于第 4 肋的前部。术后过程顺利。组织学检查证实肿块为软骨肉瘤,切缘阴性。患者术后第 4 天出院,术后 2 年随访时最新的胸部 CT 未显示肿瘤复发。

讨论

胸壁重建的目的是避免不稳定,如果缺损尺寸≥10cm 或至少切除三根肋骨,则防止胸腔器官暴露。聚丙烯网在对胸壁的机械支撑方面可能相对较弱,然而,在这种情况下,缺损不大,外侧翻转的胸大肌可与网一起提供足够的支撑。

结论

在没有 Ollier 病或 Maffucci 综合征等多发内生软骨瘤病的情况下,年轻亚洲女性由良性孤立性内生软骨瘤继发的软骨肉瘤极为罕见。包括体格检查和影像学检查在内的定期随访可早期发现恶变,从而避免需要进行涉及多根肋骨切除的积极手术治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a1d/4276080/68cd91525e13/gr1.jpg

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