S1 guidelines for dermatofibrosarcoma protuberans (DFSP) – update 2018

Abstract

Summary While dermatofibrosarcoma protuberans (DFSP) is a rare cancer entity overall, it is nevertheless the most common type of cutaneous sarcoma. The tumor is of fibroblastic origin and characterized by slow, undermining and locally destructive growth. Metastatic spread is very rare. Given its nonspecific clinical appearance, diagnosis is frequently delayed. Biopsy and subsequent histopathology are key diagnostic tools. Standard treatment for primary tumors consists of complete excision with surgical margins of 1 to 2 cm. Smaller margins are associated with high local recurrence rates. Inoperable and metastatic DFSP may be treated with radiation therapy. Approximately 80–90 % of DFSP lesions harbor a fusion gene that results in continuous activation of the PDGF‐β signaling pathway. Consequently, molecular targeted therapy inhibiting PDGF‐β is an effective option for advanced (inoperable) and metastatic DFSP. The first agent to be approved for systemic treatment of DFSP is the multikinase inhibitor imatinib, showing objective response rates of about 50 % in clinical trials.

A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

“…It is important to define the optimal surgical margin width around the primary tumor. To achieve histological margin control, both the National Comprehensive Cancer Network guidelines and the S1 guidelines call for at least 2 cm surgical margins investing the fascia of the muscle or pericranium [ 32 , 44 ]. Most cases of breast DFSP followed guidelines.…”

Dermatofibrosarcoma Protuberans Mimicking Primary Breast Neoplasm: A case report and literature review

“…Multikinase inhibitor imatinib is the first agent to be approved for systemic treatment of DFSP and has shown objective response rates of approximately 50% in clinical trials. 16 Imatinib treatment has been also used for metastatic DFSP. 17 Long-term follow-up requires strict monitoring every 6–12 months with ultrasound and tissue biopsy in cases of suspected recurrence.…”

Dermatofibrosarcoma protuberans pigmentado: descripción de un caso pediátrico

“…miento multidisciplinario por los servicios de oncohematología infantil, cirugía plástica infantil y dermatología pediátrica de nuestro centro. Se han realizado controles clínicos y radiológicos periódicos, basados en las recomendaciones de las guías clínicas internacionales 11,12 : durante el primer año, trimestral; durante el segundo y tercer año, cada 4 meses; semestral hasta el quinto año y se recomienda hasta diez años después del diagnóstico, seguimiento anual. En nuestro caso, se ha completado seguimiento durante 5 años, sin recurrencia ni aparición de enfermedad a distancia tras la extirpación completa de la lesión.…”