Collision of actinic keratosis and dermatofibrosarcoma protuberans within a single lesion as a diagnostic pitfall: a case report

Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive cutaneous sarcoma, whereas actinic keratosis (AK) is a common premalignant epidermal lesion. The coexistence of these two entities within a single facial lesion is extremely rare and may complicate diagnosis, particularly when an initial biopsy captures only the epidermal component. We present the case of an 80-year-old man with a persistent facial lesion initially diagnosed as AK on punch biopsy. Owing to the atypical clinical features, a repeat deep biopsy revealed the coexistence of DFSP and bowenoid AK. Immunohistochemistry confirmed the presence of DFSP with strong CD34 expression. The lesion was excised using Mohs micrographic surgery to achieve clear margins. This case highlights the importance of clinicopathological correlation and repeat biopsy for the accurate diagnosis of complex skin lesions. Surgical excision with sufficient margins is the key for optimal management.

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Publication Details

Journal
Archives of Craniofacial Surgery
Published
2025-12-20
DOI
https://doi.org/10.7181/acfs.2025.0068
Primary Topic
Sarcoma Diagnosis and Treatment
Type
article
Field-Weighted Citation Impact
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article

Collision of actinic keratosis and dermatofibrosarcoma protuberans within a single lesion as a diagnostic pitfall: a case report

Joon Ho Lee, Yujin Ahn
Archives of Craniofacial Surgery
Sarcoma Diagnosis and Treatment
article

Collision of actinic keratosis and dermatofibrosarcoma protuberans within a single lesion as a diagnostic pitfall: a case report

Joon Ho Lee, Yujin Ahn
article en

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive cutaneous sarcoma, whereas actinic keratosis (AK) is a common premalignant epidermal lesion. The coexistence of these two entities within a single facial lesion is extremely rare and may complicate diagnosis, particularly when an initial biopsy captures only the epidermal component. We present the case of an 80-year-old man with a persistent facial lesion initially diagnosed as AK on punch biopsy. Owing to the atypical clinical features, a repeat deep biopsy revealed the coexistence of DFSP and bowenoid AK. Immunohistochemistry confirmed the presence of DFSP with strong CD34 expression. The lesion was excised using Mohs micrographic surgery to achieve clear margins. This case highlights the importance of clinicopathological correlation and repeat biopsy for the accurate diagnosis of complex skin lesions. Surgical excision with sufficient margins is the key for optimal management.

Archives of Craniofacial SurgeryVol. 26(6)
Dongguk University (KR)
Openalex Percentile: Top 27%
Sarcoma Diagnosis and Treatment
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Collision of actinic keratosis and dermatofibrosarcoma protuberans within a single lesion as a diagnostic pitfall: a case report — Joon Ho Lee, Yujin Ahn · Archives of Craniofacial Surgery (2025) | TGRS Research Map | TGRS