PMID- 34628665 OWN - NLM STAT- MEDLINE DCOM- 20220328 LR - 20220328 IS - 1600-0560 (Electronic) IS - 0303-6987 (Linking) VI - 49 IP - 3 DP - 2022 Mar TI - Dermatofibrosarcoma protuberans with platelet-derived growth factor-D rearrangement; two cases with morphologically distinct presentations. PG - 274-277 LID - 10.1111/cup.14148 [doi] AB - Dermatofibrosarcoma protuberans (DFSP) is a mesenchymal neoplasm that is usually located in the dermis or subcutis and is locally aggressive. Rarely, these lesions may undergo fibrosarcomatous transformation, which is thought to increase their metastatic potential. DFSP is classically associated with a 17;22 translocation (or ring chromosome thereof) resulting in fusion of the COL1A1 and PDGFB genes. However, variant fusions involving PDGFD have been recently reported. Herein, we present two morphologically diverse cases of DFSP with PDGFD rearrangement. Case 1 is a 68-year-old female with a left dorsal foot lesion. Morphologically, the lesion is unusual as it is a well-circumscribed, hypercellular, subcutaneous nodule with uniform CD34-positive spindle cells arranged in a herringbone pattern without storiform arrangement or "honeycombing" fat entrapment. It was diagnosed as pure fibrosarcomatous DFSP. Case 2 is a 37-year-old male with a right supra-auricular lesion. Morphologically, the lesion displays classic DFSP features including bland CD34-positive spindle cells with storiform growth, fat entrapment, and infiltrative borders. Both lesions were negative for COL1A1-PDGFB fusion but positive for PDGFD rearrangement by fluorescence in situ hybridization (FISH) analysis. FISH testing for PDGFD rearrangement should be performed in cases where there is a high suspicion for DFSP but initial studies for COL1A1-PDGFB are negative. CI - (c) 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. FAU - Campbell, Katelynn AU - Campbell K AUID- ORCID: 0000-0002-7855-9424 AD - Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA. FAU - Bridge, Julia A AU - Bridge JA AD - Department of Cytogenetics and Fluorescence In-Situ Hybridization, ProPath, Dallas, Texas, USA. FAU - DiMaio, Dominick AU - DiMaio D AD - Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, Nebraska, USA. FAU - Wilson, Janice AU - Wilson J AD - Department of Dermatology, University of Texas Medical Branch, Galveston, Texas, USA. FAU - Shalin, Sara C AU - Shalin SC AD - Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA. FAU - Gardner, Jerad M AU - Gardner JM AUID- ORCID: 0000-0001-7397-2159 AD - Department of Anatomic and Clinical Pathology, Geisinger Medical Laboratories, Danville, Pennsylvania, USA. LA - eng PT - Case Reports DEP - 20211019 PL - United States TA - J Cutan Pathol JT - Journal of cutaneous pathology JID - 0425124 RN - 0 (Lymphokines) RN - 0 (PDGFD protein, human) RN - 0 (Platelet-Derived Growth Factor) SB - IM MH - Adult MH - Aged MH - Dermatofibrosarcoma/*genetics/pathology MH - Female MH - *Gene Rearrangement MH - Humans MH - In Situ Hybridization, Fluorescence MH - Lymphokines/*genetics MH - Male MH - Platelet-Derived Growth Factor/*genetics MH - Skin Neoplasms/*genetics/pathology OTO - NOTNLM OT - PDGFD OT - dermatofibrosarcoma protuberans OT - fibrosarcomatous transformation EDAT- 2021/10/11 06:00 MHDA- 2022/03/29 06:00 CRDT- 2021/10/10 21:21 PHST- 2021/09/09 00:00 [revised] PHST- 2021/05/24 00:00 [received] PHST- 2021/10/05 00:00 [accepted] PHST- 2021/10/11 06:00 [pubmed] PHST- 2022/03/29 06:00 [medline] PHST- 2021/10/10 21:21 [entrez] AID - 10.1111/cup.14148 [doi] PST - ppublish SO - J Cutan Pathol. 2022 Mar;49(3):274-277. doi: 10.1111/cup.14148. Epub 2021 Oct 19.