S1 guidelines for dermatofibrosarcoma protuberans (DFSP) - update 2018.
J Dtsch Dermatol Ges
; 17(6): 663-668, 2019 Jun.
Article
em En
| MEDLINE
| ID: mdl-31115967
ABSTRACT
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-ß signaling pathway. Consequently, molecular targeted therapy inhibiting PDGF-ß 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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Cutâneas
/
Dermatofibrossarcoma
Tipo de estudo:
Guideline
Limite:
Humans
Idioma:
En
Revista:
J Dtsch Dermatol Ges
Assunto da revista:
DERMATOLOGIA
Ano de publicação:
2019
Tipo de documento:
Article
País de afiliação:
Alemanha