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前额隆突性皮肤纤维肉瘤:1例罕见病例报告及文献复习

Dermatofibrosarcoma Protuberans of the Forehead: Case Report of a Rare Entity and Review of Literature.

作者信息

Kengne Ulrich Igor Mbessoh, Thiam Jaafar Ibn Abou Talib, Fall Amacoumba, Balde Salif, Ndiaye Mamadou, Mekontso Joel Gabin Konlack, Sarr Gorgui, Zoure Etienne Tossou, Sow Mamadou, Ka Sidy

机构信息

Department of Surgical Oncology, Dalal Jamm National Hospital Center, Dakar, Senegal.

Department of Surgery and Specialties, Faculty of Medicine, Pharmacy and Odontostomatology, Cheikh Anta Diop University, Dakar, Senegal.

出版信息

Case Rep Oncol Med. 2025 May 8;2025:9991548. doi: 10.1155/crom/9991548. eCollection 2025.

DOI:10.1155/crom/9991548
PMID:40375939
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12081156/
Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma originating from fibroblasts in the dermal connective tissue, comprising approximately 1% of all soft tissue sarcomas. While most cases involve the trunk and extremities, only 10%-15% occur in the cephalic region, representing less than 1% of all head and neck neoplasms. DFSP is notable for its high propensity for local recurrence following surgical excision and its low metastatic potential. We report a case of recurrent DFSP of the forehead extending to the anterior wall of the left frontal sinus, without brain involvement, in a 33-year-old male with a history of three prior wide local excisions. A multidisciplinary cancer team recommended systemic imatinib therapy. This case highlights the challenges of managing DFSP in an uncommon location, underscoring the importance of a multidisciplinary approach in addressing recurrent and complex presentations.

摘要

隆突性皮肤纤维肉瘤(DFSP)是一种罕见的软组织肉瘤,起源于真皮结缔组织中的成纤维细胞,约占所有软组织肉瘤的1%。虽然大多数病例发生在躯干和四肢,但只有10%-15%发生在头部区域,占所有头颈部肿瘤的比例不到1%。DFSP的显著特点是手术切除后局部复发倾向高,转移潜能低。我们报告一例33岁男性复发性前额DFSP,病变延伸至左额窦前壁,未累及脑部,该患者既往有三次广泛局部切除病史。一个多学科癌症治疗团队建议采用伊马替尼全身治疗。该病例凸显了在不常见部位管理DFSP的挑战,强调了多学科方法在处理复发性和复杂病例中的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/c3afd3072d2b/CRIONM2025-9991548.005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/88261e514dba/CRIONM2025-9991548.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/7eea2aafeaba/CRIONM2025-9991548.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/e9851ab59d04/CRIONM2025-9991548.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/9d920946637a/CRIONM2025-9991548.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/c3afd3072d2b/CRIONM2025-9991548.005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/88261e514dba/CRIONM2025-9991548.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/7eea2aafeaba/CRIONM2025-9991548.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/e9851ab59d04/CRIONM2025-9991548.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/9d920946637a/CRIONM2025-9991548.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e8d/12081156/c3afd3072d2b/CRIONM2025-9991548.005.jpg

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本文引用的文献

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Novel role of fluorescent in situ hybridization technique (FISH) in recommended surgical margins of dermatofibrosarcoma protuberans: A preliminary study.荧光原位杂交技术(FISH)在隆突性皮肤纤维肉瘤推荐手术切缘中的新作用:一项初步研究。
Ann Chir Plast Esthet. 2024 Mar;69(2):124-130. doi: 10.1016/j.anplas.2023.08.006. Epub 2023 Aug 29.
2
Dermatofibrosarcoma protuberans: A rare presentation in the scalp. Case report.隆突性皮肤纤维肉瘤:头皮罕见表现。病例报告。
Stomatologija. 2023;25(1):26-28.
3
Giant dermatofibrosarcoma protuberans head and neck: a diagnostic dilemma.
头颈部巨大隆突性皮肤纤维肉瘤:诊断难题
Indian J Otolaryngol Head Neck Surg. 2023 Jun;75(2):991-995. doi: 10.1007/s12070-022-03274-z. Epub 2022 Nov 15.
4
Recurrent Dermatofibrosarcoma Protuberans Infiltrating the Skull.复发性隆突性皮肤纤维肉瘤侵犯颅骨
Dermatol Surg. 2023 Apr 1;49(4):418-419. doi: 10.1097/DSS.0000000000003715.
5
Dermatofibrosarcoma Protuberans of Face: A Rare Entity and Review of Literature.面部隆突性皮肤纤维肉瘤:一种罕见病例及文献综述
Indian J Otolaryngol Head Neck Surg. 2022 Dec;74(Suppl 3):5469-5472. doi: 10.1007/s12070-021-02790-8. Epub 2021 Aug 3.
6
Epidemiological trends and survival outcomes for dermatofibrosarcoma protuberans of the head and neck region.头颈部隆突性皮肤纤维肉瘤的流行病学趋势和生存结果。
Int J Dermatol. 2023 May;62(5):664-671. doi: 10.1111/ijd.16459. Epub 2022 Nov 1.
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Recurrent Dermatofibrosarcoma Protuberans of the Head and Neck: a Case Series.头颈部复发性隆突性皮肤纤维肉瘤:病例系列
Indian J Surg Oncol. 2023 Mar;14(1):128-136. doi: 10.1007/s13193-022-01636-1. Epub 2022 Sep 1.
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Case report: Identification of a novel heterozygous germline mutation in a patient with dermatofibrosarcoma protuberans.病例报告:隆突性皮肤纤维肉瘤患者中一种新型杂合性种系突变的鉴定。
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