Case report: Congenital Bednar tumor with pregnancy-associated growth and pain: a diagnostic pitfall with blue nevus-like features

Bednar tumors are rare pigmented variants of dermatofibrosarcoma protuberans (DFSP), accounting for less than 5% of DFSP cases. We report a rare case of a congenitally pigmented lesion that progressively enlarged during pregnancy and developed pain. A 32-year-old woman presented with a blue-black nodule on her right shoulder, which had been present since birth and was initially misdiagnosed as a blue nevus. Histopathological analysis revealed spindle cell proliferation in a storiform pattern with focal melanin pigment. Immunohistochemistry showed strong CD34 positivity and negative S100 staining, consistent with Bednar tumors. This case highlights the importance of considering Bednar tumors in the differential diagnosis of congenital pigmented lesions, particularly those showing interval growth or symptoms.

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Publication Details

Journal
Frontiers in Oncology
Published
2026-03-12
DOI
https://doi.org/10.3389/fonc.2026.1792949
Primary Topic
Sarcoma Diagnosis and Treatment
Type
article
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article

Case report: Congenital Bednar tumor with pregnancy-associated growth and pain: a diagnostic pitfall with blue nevus-like features

Ying Shi, Ying Chen
Frontiers in Oncology
Sarcoma Diagnosis and Treatment
article

Case report: Congenital Bednar tumor with pregnancy-associated growth and pain: a diagnostic pitfall with blue nevus-like features

Ying Shi, Ying Chen
article en

Abstract

Bednar tumors are rare pigmented variants of dermatofibrosarcoma protuberans (DFSP), accounting for less than 5% of DFSP cases. We report a rare case of a congenitally pigmented lesion that progressively enlarged during pregnancy and developed pain. A 32-year-old woman presented with a blue-black nodule on her right shoulder, which had been present since birth and was initially misdiagnosed as a blue nevus. Histopathological analysis revealed spindle cell proliferation in a storiform pattern with focal melanin pigment. Immunohistochemistry showed strong CD34 positivity and negative S100 staining, consistent with Bednar tumors. This case highlights the importance of considering Bednar tumors in the differential diagnosis of congenital pigmented lesions, particularly those showing interval growth or symptoms.

Frontiers in OncologyVol. 16
Wuhan University (CN), Renmin Hospital of Wuhan University (CN)
Good health and well-being
Openalex Percentile: Top 25%
Sarcoma Diagnosis and Treatment
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