Dermatofibrosarcoma Protuberans Arising in a Digit: A Case Report

Dermatofibrosarcoma protuberans (DFSP) is a rare slow-growing soft tissue sarcoma that is usually found on the trunk or extremities. This report highlights an exceedingly rare case in which a DFSP arose on the digit of a 43-year-old man. Clinical and histologic analysis revealed an atypical CD34-positive tumor with differentials including DFSP and multiple similar entities. However, fluorescence in situ hybridization studies revealed a COL1A1 translocation to the platelet-derived growth factor B (22q13.1) locus, t(17;22)(q22;q13), confirming the diagnosis of DFSP. Although rare, this case highlights the importance of including DFSP on the differential in CD34-positive acral tumors, with cytogenetic analysis crucial for accurate diagnosis and treatment.

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Publication Details

Journal
American Journal of Dermatopathology
Published
2025-02-19
DOI
https://doi.org/10.1097/dad.0000000000002948
Primary Topic
Sarcoma Diagnosis and Treatment
Type
article
Field-Weighted Citation Impact
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article

Dermatofibrosarcoma Protuberans Arising in a Digit: A Case Report

N Landero De Ruiz, Patricia Moody, Samantha Sun, John A. Odom
American Journal of Dermatopathology
Sarcoma Diagnosis and Treatment
article

Dermatofibrosarcoma Protuberans Arising in a Digit: A Case Report

N Landero De Ruiz, Patricia Moody, Samantha Sun, John A. Odom
article en

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare slow-growing soft tissue sarcoma that is usually found on the trunk or extremities. This report highlights an exceedingly rare case in which a DFSP arose on the digit of a 43-year-old man. Clinical and histologic analysis revealed an atypical CD34-positive tumor with differentials including DFSP and multiple similar entities. However, fluorescence in situ hybridization studies revealed a COL1A1 translocation to the platelet-derived growth factor B (22q13.1) locus, t(17;22)(q22;q13), confirming the diagnosis of DFSP. Although rare, this case highlights the importance of including DFSP on the differential in CD34-positive acral tumors, with cytogenetic analysis crucial for accurate diagnosis and treatment.

American Journal of DermatopathologyVol. 47(5)
University of Tampa (US)
Good health and well-being
Openalex Percentile: Top 95%
Sarcoma Diagnosis and Treatment
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