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隐匿性隆突性皮肤纤维肉瘤:一例非典型表现并术后切除并发症的病例报告

Unmasking dermatofibrosarcoma protuberans: Case report of an atypical presentation complicated by post-surgical excision.

作者信息

Zhou Arlene YaQun, Chin Justin, Strutin Millard D, Lomiguen Christine M

机构信息

Department of Primary Care, Touro College of Osteopathic Medicine, New York, NY, United States; Department of Surgery, Saint Clare's Denville Hospital, Denville, NJ, United States.

Department of Primary Care, Touro College of Osteopathic Medicine, New York, NY, United States; Department of Family Medicine, Lifelong Medical Care, Richmond, CA, United States.

出版信息

Int J Surg Case Rep. 2020;69:101-104. doi: 10.1016/j.ijscr.2020.03.020. Epub 2020 Apr 3.

DOI:10.1016/j.ijscr.2020.03.020
PMID:32305027
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7163292/
Abstract

INTRODUCTION

Dermatofibrosarcoma Protuberans (DFSP) is a rare type of malignant tumor of the cutaneous soft tissue that typically involves the dermis and surrounding structures. The typical clinical presentation involves an asymptomatic, slowly enlarging, indurated plaque, usually found on the trunk.

PRESENTATION OF CASE

Here we present an atypical presentation of DFSP in a patient with a recurrent mass that was initially diagnosed as a lipoma, with supporting literature and clinical considerations for post-surgical management DISCUSSION: In its earlier stages, DFSP can mimic other benign or malignant dermatological pathologies, which can complicate diagnosis purely via history and physical exam. Atypical features such as prior surgical management, morphology, and location can complicate the clinical diagnosis of DFSP. Immunohistochemistry is often required for definitive diagnosis as DFSP is CD34+ and is histologically described as spindle-cells in whorled or storiform pattern, with uniform, small elongated cells.

CONCLUSION

DFSP is a rare tumor of the skin and subcutaneous tissue. Though it commonly presents with induration, skin discoloration, and telangiectasias, it is important to keep DFSP as a differential in new masses found on previous surgical sites, especially if the previous mass had a possibility of recurrence.

摘要

引言

隆突性皮肤纤维肉瘤(DFSP)是一种罕见的皮肤软组织恶性肿瘤,通常累及真皮及周围结构。典型的临床表现为无症状、缓慢增大的硬结性斑块,常见于躯干。

病例介绍

在此,我们展示了一例DFSP的非典型表现,患者有复发性肿块,最初被诊断为脂肪瘤,并提供了支持性文献及术后管理的临床考量。

讨论

在早期阶段,DFSP可模仿其他良性或恶性皮肤病变,这使得仅通过病史和体格检查进行诊断变得复杂。诸如既往手术史、形态和位置等非典型特征会使DFSP的临床诊断复杂化。由于DFSP为CD34阳性,组织学上表现为呈漩涡状或席纹状排列的梭形细胞,细胞均匀、细长,因此明确诊断通常需要免疫组化。

结论

DFSP是一种罕见的皮肤及皮下组织肿瘤。尽管它通常表现为硬结、皮肤变色和毛细血管扩张,但对于在既往手术部位发现的新肿块,尤其是如果既往肿块有复发可能时,将DFSP作为鉴别诊断很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0a4/7163292/4c4753605605/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0a4/7163292/97dce6599f53/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0a4/7163292/f5674d252f0f/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0a4/7163292/d9b14d35f194/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0a4/7163292/4c4753605605/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0a4/7163292/97dce6599f53/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0a4/7163292/f5674d252f0f/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0a4/7163292/d9b14d35f194/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0a4/7163292/4c4753605605/gr4.jpg

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