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乳腺隆突性皮肤纤维肉瘤:病例报告

Dermatofibrosarcoma protuberans in the breast: Case report.

作者信息

Kim Hwansoo

机构信息

Department of Surgery, Kangwon National University Hospital, Republic of Korea.

出版信息

Int J Surg Case Rep. 2021 Apr;81:105773. doi: 10.1016/j.ijscr.2021.105773. Epub 2021 Mar 13.

Abstract

INTRODUCTION AND IMPORTANCE

Dermatofibrosarcoma protuberans (DFSP) represents about 1% of soft tissue sarcomas with an estimated incidence of 0.8-5.0 cases per million per year. The involvement of DFSP in breast is very rare and very few cases have been reported in the literature. DFSP was recurred in situ, not spread to distant site. The complete surgical excision with wide, pathologically negative margins of 3 cms is the optimal treatment for primary or recurrent tumor.

PRESENTATION OF CASE

A 46-year-old woman presented with palpable lump in the in the right breast. On ultrasonography, a lesion appeared as hypoechoic, circumscribed mass of approximately 37 mm × 30 mm in diameter in the upper central part of the right breast. The mass of right breast was demonstrated DFPS by pathologic examination. Chest computerized tomography (CT) scan and 2-[18F]-fluoro-2-deoxy-d-glucose (18F-FDG) positron-emission tomography (PET) showed only primary lesion in subcutaneous layer and no enlarged lymph node. The patient underwent excision of the tumor widely. There was no evidence of DFSP local recurrence after five years of follow-up of the patient.

DISCUSSION

DFSP is a rare tumor arising from dermis and subcutaneous mesenchymal tissue. Whereas, characteristic imaging feature of DFSP in the breast are not well-defined. The primary treatment for DFSP is considered to be surgical excision.

CONCLUSION

DFSP in breast is extremely uncommon and can mimic a primary breast tumor. Surgical excision with adequate resection margins is recommended to ensure local control of the disease.

摘要

引言与重要性

隆突性皮肤纤维肉瘤(DFSP)约占软组织肉瘤的1%,估计发病率为每年每百万人口0.8 - 5.0例。DFSP累及乳腺非常罕见,文献报道的病例极少。DFSP原位复发,未发生远处转移。对原发性或复发性肿瘤,最佳治疗方法是进行手术完整切除,切缘宽达3厘米且病理检查阴性。

病例介绍

一名46岁女性,右乳可触及肿块。超声检查显示,右乳上中部有一低回声、边界清晰的肿块,直径约37毫米×30毫米。经病理检查,右乳肿块确诊为DFPS。胸部计算机断层扫描(CT)及2 - [18F] - 氟 - 2 - 脱氧 - D - 葡萄糖(18F - FDG)正电子发射断层扫描(PET)显示仅皮下层有原发性病变,无肿大淋巴结。患者接受了肿瘤广泛切除。对该患者进行五年随访后,无DFSP局部复发迹象。

讨论

DFSP是一种起源于真皮和皮下间充质组织的罕见肿瘤。然而,乳腺DFSP的特征性影像学表现尚不明确。DFSP的主要治疗方法被认为是手术切除。

结论

乳腺DFSP极为罕见,可类似原发性乳腺肿瘤。建议手术切除时保证足够的切缘,以确保对疾病的局部控制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/658d/8024653/5f8fa0ae2dbf/gr1.jpg

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