Management of incidental dermatofibrosarcoma protuberans: A single center 5-year experience

Öz

Aim: Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor. The diagnosis is challenging because it is usually hard to differ DFSP from other skin lesions. Distant metastasis is rare, but local recurrence is common.The aim of this study is to review the treatment algorithms in DFSPs that are detected incidentally and to increase awareness about this rare tumor. Material and Methods: 17 patients who underwent excision of epidermal lesions which were considered to be benign and were diagnosed with DFSP, at departmant of general surgery between 2012 and 2017, were analyzed. Result: A total of 17 patients were analyzed retrospectively. No recurrence was detected in any of ourpatients. Conclusion: Awareness of this rare entity is important for diagnosis and management of the disease.

Anahtar Kelimeler

• Dermatofibrosarcoma protuberans
• Mohs micrographic surgery (MMS)
• Mesenchymal tumor

Insidental olarak saptanan dermatofibrosarkom protuberans tedavisi: Tek merkez 5 yıllık deneyimlerimiz

Öz

Aim: Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor. The diagnosis is challenging because it is usually hard to differ DFSP from other skin lesions. Distant metastasis is rare, but local recurrence is common.The aim of this study is to review the treatment algorithms in DFSPs that are detected incidentally and to increase awareness about this rare tumor. Material and Methods: 17 patients who underwent excision of epidermal lesions which were considered to be benign and were diagnosed with DFSP, at departmant of general surgery between 2012 and 2017, were analyzed. Result: A total of 17 patients were analyzed retrospectively. No recurrence was detected in any of ourpatients. Conclusion: Awareness of this rare entity is important for diagnosis and management of the disease.

Anahtar Kelimeler

• Dermatofibrosarcoma protuberans
• mezenkimal tümör
• Mohs micrographic surgery (MMS)

Kaynakça

1. Referans 1 Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data--a study from the French Federation of Cancer Centers Sarcoma Group Marie-Josée Terrier-Lacombe 1 , Louis Guillou, Georges Maire, Philippe Terrier, Dominique Ranchère Vince, Nicolas de Saint Aubain Somerhausen, Françoise Collin, Florence Pedeutour, Jean-Michel Coindre
2. Referans 2 Genomic and transcriptomic features of dermatofibrosarcoma protuberans: Unusual chromosomal origin of the COL1A1-PDGFB fusion gene and synergistic effects of amplified regions in tumor development a,b,∗ a c c a Jan Köster , Elsa Arbajian , Björn Viklund , Anders Isaksson , Jakob Hofvander , d e a a a,b Felix Haglund , Henrik Bauer , Linda Magnusson , Nils Mandahl , Fredrik Mertens