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Dermatofibrosarcoma protuberans: a study of clinical, pathologic, genetic, and therapeutic features in Korean patients

 Zhenlong Zheng  ;  Junjei Piao  ;  Ji-Hye Lee  ;  Song-Ee Kim  ;  Soo-Chan Kim  ;  Kee Yang Chung  ;  Mi Ryung Roh 
 YONSEI MEDICAL JOURNAL, Vol.56(2) : 440-446, 2015 
Journal Title
Issue Date
Adolescent ; Adult ; Asian Continental Ancestry Group/genetics* ; Collagen Type I/genetics* ; DNA Primers ; Dermatofibrosarcoma/ethnology ; Dermatofibrosarcoma/genetics* ; Dermatofibrosarcoma/pathology* ; Dermatofibrosarcoma/surgery ; Female ; Humans ; Male ; Middle Aged ; Mohs Surgery ; Multiplex Polymerase Chain Reaction ; Neoplasm Recurrence, Local ; Oncogene Proteins, Fusion/genetics* ; Proto-Oncogene Proteins c-sis/genetics* ; Republic of Korea ; Reverse Transcriptase Polymerase Chain Reaction ; Skin Neoplasms/ethnology ; Skin Neoplasms/genetics* ; Skin Neoplasms/pathology* ; Skin Neoplasms/surgery ; Treatment Outcome
Dermatofibrosarcoma protuberans ; Mohs micrographic surgery ; collagen type I alpha 1-platelet-derived growth factor beta ; histological subtype
PURPOSE: Dermatofibrosarcoma protuberans (DFSP) carries a translocation resulting in the collagen type I alpha 1 (COL1A1)-platelet-derived growth factor beta (PDGFB) fusion gene, which is responsible for PDGFB activation. The purpose of this study is to evaluate the clinicopathological, genetic, and therapeutic features of DFSP in Korean patients. MATERIALS AND METHODS: Clinicopathological features of 37 patients with DFSP were reviewed. Multiplex reverse transcriptase-polymerase chain reaction (PCR) was carried out in 16 patients using formalin-fixed, paraffin-embedded tissues and specific primers for COL1A1 and PDGFB. RESULTS: The mean age of 37 patients was 37.4 years old. The most common tumor location was the trunk. All patients were treated primarily with surgery: 34 (91.7%) cases with Mohs micrographic surgery (MMS) and 3 (8.3%) cases with wide local excision. The median follow-up time was 33.7 months. Two patients, one in each treatment group, demonstrated local recurrence during the follow-up period. The COL1A1-PDGFB fusion gene was expressed in 14 (87.5%) cases, demonstrated by reverse transcriptase PCR analysis. No association was found among the different COL1A1-PDGFB fusion transcripts, the various histological subtypes and clinical features. CONCLUSION: Our results support the effectiveness of MMS in treating DFSP. The COL1A1-PDGFB fusion transcript was observed in 87.5% of patients. Therefore, COL1A1-PDGFB is a useful and accurate tool in diagnosing DFSP in Koreans.
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1. College of Medicine (의과대학) > Dept. of Dermatology (피부과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Soo Chan(김수찬) ORCID logo https://orcid.org/0000-0002-2327-4755
Roh, Mi Ryung(노미령) ORCID logo https://orcid.org/0000-0002-6285-2490
Lee, Ji Hye(이지혜)
Chung, Kee Yang(정기양) ORCID logo https://orcid.org/0000-0003-3257-0297
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