Congenital dermatofibrosarcoma protuberans in a seven-month-old infant initially misdiagnosed as infantile myofibroma: A case report

doi:10.5306/wjco.124132

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World Journal of Clinical Oncology

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2218-4333

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Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA

Case Report

Copyright: ©Author(s) 2026. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution-NonCommercial (CC BY-NC 4.0) license. No commercial re-use. See permissions. Published by Baishideng Publishing Group Inc.

World J Clin Oncol. Aug 24, 2026; 17(8): 124132
Published online Aug 24, 2026. doi: 10.5306/wjco.124132

Congenital dermatofibrosarcoma protuberans in a seven-month-old infant initially misdiagnosed as infantile myofibroma: A case report

Chen-Jun Zheng, Chen-Liang Wan, Chuan-Bo Zhang, Bo Hai, Chun-Feng Huang

Chen-Jun Zheng, Chen-Liang Wan, Chuan-Bo Zhang, Bo Hai, Chun-Feng Huang, Department of General Surgery, Kunming Children’s Hospital, Kunming 650000, Yunnan Province, China

Author contributions: Zheng CJ conceived and designed the study, performed the data analysis, and drafted the manuscript; Wan CL supervised the research project and provided critical intellectual input throughout the study; Zhang CB, Hai B, and Huang CF assisted with the data collection and analysis, and critically revised the manuscript; and all authors reviewed the final manuscript and approved its submission.

AI contribution statement: AI tools, specifically ChatGPT (version 4.0, OpenAI), were used solely for translation assistance to help translate the manuscript from Chinese to English. No AI tool was used to generate research data, interpret results, or formulate conclusions. All AI assisted content was critically reviewed and revised by the authors, who take full responsibility for the accuracy, originality, and integrity of the manuscript.

Supported by Scientific Research Foundation of the Yunnan Provincial Department of Education, No. 2025J0286; Kunming Municipal Health Commission Health Research Project, No. 2025-06-02-011; and Feng Jiexiong Expert Workstation, No. 202605AF350095.

Informed consent statement: Informed written consent was obtained from the patient’s parents for publication of this report and any accompanying images.

Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.

CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).

Corresponding author: Chen-Liang Wan, Associate Chief Physician, Department of General Surgery, Kunming Children’s Hospital, No. 288 Qianxing Rd, Xishan District, Kunming 650000, Yunnan Province, China. wanchengliang@kmmu.edu.cn

Received: June 9, 2026
Revised: July 14, 2026
Accepted: August 18, 2026
Published online: August 24, 2026
Processing time: 78 Days and 18.3 Hours

Abstract

BACKGROUND

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous sarcoma predominantly observed in adults 30 years to 50 years of age. It is exceedingly rare in infants and is often misdiagnosed owing to its indolent growth and resemblance to benign lesions such as hemangiomas or fibromas.

CASE SUMMARY

A 7-month-old male infant presented with a congenital dorsal mass that had gradually enlarged since birth. Initial biopsy of the lesion resulted in a misdiagnosis of infantile myofibroma. Given the tumor’s progressive growth, wide local excision was performed with a 2-cm peripheral margin extending to the muscular fascia. Subsequent histopathological examination and immunohistochemical analysis (CD34+, SMA-, Desmin-, S-100-, HMB45-, and Ki-67 approximately 5%) confirmed the diagnosis of classic DFSP. Owing to resource constraints, testing for the COL1A1-PDGFB fusion gene was not performed. The patient experienced an uneventful recovery and underwent close follow-up. At the 2-year postoperative follow-up, clinical examination and ultrasonography revealed no evidence of local recurrence or metastasis.

CONCLUSION

At the 2-year follow-up, the patient exhibited no signs of disease recurrence. However, congenital DFSP requires prolonged surveillance beyond this period because of its potential for late local recurrence.

Keywords: Dermatofibrosarcoma protuberans; Congenital; Infant; Surgical excision; Case report

Core Tip: Dermatofibrosarcoma protuberans (DFSP) is exceptionally rare in infants and is often misdiagnosed as a benign lesion. We report the case of a 7-month-old male infant with congenital DFSP on the back that was initially misdiagnosed as infantile myofibroma. Wide local excision with clear margins resulted in complete remission, with no recurrence at the 2-year follow-up. This case highlights the importance of maintaining a high index of suspicion, establishing an accurate immunohistochemical diagnosis, and achieving complete surgical resection in the management of congenital DFSP.