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隆突性皮肤纤维肉瘤的乳腺影像学表现:病例报告及文献复习

Breast imaging findings in dermatofibrosarcoma protuberans: Case report and literature review.

作者信息

He Jie, Tao Zhizhi, Liu Wangwang, Lou Feifei, Hu Hongjie

机构信息

Department of Radiology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, China.

Department of Pathology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, China.

出版信息

Medicine (Baltimore). 2025 Apr 25;104(17):e42247. doi: 10.1097/MD.0000000000042247.

DOI:10.1097/MD.0000000000042247
PMID:40295258
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12040056/
Abstract

RATIONALE

Dermatofibrosarcoma protuberans (DFSP) of the breast is a rare condition and is often misdiagnosed as benign breast lesions, leading to delays patient treatment. Complete surgical resection with negative margins is the primary treatment option. In this study, we report a case of a patient with DFSP, with clinical and imaging presentations and treatment modalities.

PATIENT CONCERNS

A 15-year-old girl was admitted with a gradually enlarging right breast mass over 3 years. Physical examination revealed symmetrical breasts with no deviation or depression of the nipples, discharge, or orange peel texture or dimpling. A palpable mass of approximately 30 × 25 mm was located in the 2 o'clock position of the right breast.

DIAGNOSES

The imaging characteristics of the breast mass were similar to those of breast fibroadenoma. A preoperative radiological diagnosis of breast fibroadenoma was made; however, postoperative pathology confirmed DFSP.

INTERVENTIONS

The patient underwent local resection of the right breast mass under general anesthesia. The diagnosis of a malignant tumor with capsular invasion of the right breast prompted a second surgery 16 days later that involved radical extended resection.

OUTCOME

The patient has been followed up regularly for approximately 2 years with no signs of recurrence.

LESSONS

Breast DFSP is frequently misdiagnosed as a benign lesion owing to its imaging characteristics and symptom presentation, risking treatment delays. Surgical resection is the standard treatment; however, nonsurgical treatment is also effective. This study highlights the importance the diagnostic accuracy and treatment of this condition.

摘要

理论依据

乳腺隆突性皮肤纤维肉瘤(DFSP)是一种罕见疾病,常被误诊为良性乳腺病变,导致患者治疗延误。手术切缘阴性的完整手术切除是主要治疗选择。在本研究中,我们报告了1例DFSP患者的临床、影像表现及治疗方式。

患者情况

一名15岁女孩因右侧乳腺肿块3年逐渐增大入院。体格检查显示双侧乳房对称,乳头无偏移、凹陷、溢液,亦无橘皮样改变或酒窝征。右侧乳房2点钟位置可触及一约30×25mm的肿块。

诊断

乳腺肿块的影像特征与乳腺纤维腺瘤相似。术前影像学诊断为乳腺纤维腺瘤;然而,术后病理证实为DFSP。

干预措施

患者在全身麻醉下行右侧乳腺肿块局部切除术。右侧乳腺恶性肿瘤伴包膜侵犯的诊断促使在16天后进行了第二次手术,即根治性扩大切除术。

结果

患者已定期随访约2年,无复发迹象。

经验教训

乳腺DFSP因其影像特征和症状表现常被误诊为良性病变,有治疗延误的风险。手术切除是标准治疗方法;然而,非手术治疗也有效。本研究强调了该病诊断准确性及治疗的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5141/12040056/db594135f88d/medi-104-e42247-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5141/12040056/97aeafbc878d/medi-104-e42247-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5141/12040056/039f085b3e4b/medi-104-e42247-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5141/12040056/edf0ccf525e7/medi-104-e42247-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5141/12040056/db594135f88d/medi-104-e42247-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5141/12040056/97aeafbc878d/medi-104-e42247-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5141/12040056/039f085b3e4b/medi-104-e42247-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5141/12040056/edf0ccf525e7/medi-104-e42247-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5141/12040056/db594135f88d/medi-104-e42247-g004.jpg

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本文引用的文献

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Congenital Dermatofibrosarcoma Protuberans-An Update on the Ongoing Diagnostic Challenges.先天性隆突性皮肤纤维肉瘤——当前诊断挑战的最新进展
Cancers (Basel). 2025 Jan 6;17(1):158. doi: 10.3390/cancers17010158.
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Dermatofibrosarcoma Protuberans: An Updated Review of the Literature.隆突性皮肤纤维肉瘤:文献综述更新
Cancers (Basel). 2024 Sep 11;16(18):3124. doi: 10.3390/cancers16183124.
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Dermatofibrosarcoma protuberans of the breast: A rare breast tumor mimicking a cutaneous lesion.乳腺隆突性皮肤纤维肉瘤:一种罕见的模仿皮肤病变的乳腺肿瘤。
Int J Surg Case Rep. 2024 Oct;123:110211. doi: 10.1016/j.ijscr.2024.110211. Epub 2024 Aug 25.
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The epidemiology of dermatofibrosarcoma protuberans incidence, metastasis, and death among various population groups: A Surveillance, Epidemiology, and End Results database analysis.隆突性皮肤纤维肉瘤的流行病学:在不同人群中发病率、转移和死亡的分析:一项监测、流行病学和最终结果数据库分析。
J Am Acad Dermatol. 2024 Nov;91(5):826-833. doi: 10.1016/j.jaad.2024.05.088. Epub 2024 Jun 21.
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Dermatofibrosarcoma protuberans of the breast.乳房隆突性皮肤纤维肉瘤。
BMJ Case Rep. 2024 Apr 11;17(4):e258846. doi: 10.1136/bcr-2023-258846.
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The Role of Postoperative Radiotherapy in the Management of Dermatofibrosarcoma Protuberans: A Multidisciplinary Systematic Review.术后放疗在隆突性皮肤纤维肉瘤治疗中的作用:一项多学科系统评价
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Dermatofibrosarcoma Protuberans: A Study of 148 Patients.隆突性皮肤纤维肉瘤:148 例患者研究。
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