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胸壁韧带样型纤维瘤病:一例报告

Desmoid-type fibromatosis of the chest wall: a case report.

作者信息

Xiao Yi, He Jinyuan, Gong Chulian, Liu Libao, Huang Shaohong

机构信息

The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.

出版信息

Ann Transl Med. 2020 Oct;8(20):1322. doi: 10.21037/atm-20-5237.

Abstract

Desmoid-type fibromatosis (DF), also known as deep fibromatosis or desmoid tumor, is an extremely rare neoplasm that develops from fascia and musculoaponeurotic tissue. These tumors are characterized by slow progressive growth, local invasion, and local recurrence after surgical excision, but they lack metastatic potential. DF accounts for 3.5% of all fibrous tumors, with an annual incidence of approximately 2-4/million. Until now, only a small number of cases have been found in the chest wall. Herein, we present a rare case of chest wall DF in a 43-year-old female, which was discovered accidentally due to a thoracic wall mass that extended outward from the sternum. Computed tomography scans revealed a subcutaneous soft tissue mass anterior to the sternum, which was considered to be a mesenchymal tumor or an inflammatory lesion. The patient underwent surgical excision of the mass. The mass was completely removed and all margins were negative. According to the pathological results, the patient was finally diagnosed as DF. Postoperative radiotherapy was suggested subsequently, especially considering the locally aggressive and infiltrative nature of the tumor. However, this was rejected by the patient, and biannual re-examination was recommended instead. Despite the absence of postoperative radiotherapy, there was no evidence of local recurrence 2 years later. We consider regular postoperative follow-up may be able to replace postoperative radiotherapy, and if there exist an opportunity to completely resect the mass, surgical is a worthwhile choice.

摘要

韧带样型纤维瘤病(DF),也称为深部纤维瘤病或韧带样瘤,是一种极其罕见的肿瘤,起源于筋膜和肌-腱膜组织。这些肿瘤的特点是生长缓慢、局部侵袭性强,手术切除后易局部复发,但无转移潜能。DF占所有纤维性肿瘤的3.5%,年发病率约为2-4/百万。迄今为止,仅在胸壁发现少数病例。在此,我们报告一例43岁女性胸壁DF罕见病例,该病例因胸壁肿物意外发现,肿物从胸骨向外延伸。计算机断层扫描显示胸骨前方皮下软组织肿物,考虑为间叶性肿瘤或炎性病变。患者接受了肿物手术切除。肿物被完全切除,所有切缘阴性。根据病理结果,患者最终被诊断为DF。随后建议术后放疗,尤其考虑到肿瘤局部侵袭性和浸润性。然而,患者拒绝了放疗,建议改为每半年复查一次。尽管未进行术后放疗,但2年后无局部复发迹象。我们认为术后定期随访可能能够替代术后放疗,并且如果有机会完全切除肿物,手术是一个值得的选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f81f/7661858/636ceb763611/atm-08-20-1322-f1.jpg

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