Recurrent dermatofibrosarcoma protuberans treated with neoadjuvant imatinib mesylate followed by Mohs micrographic surgery
نویسندگان
چکیده
DFSP: Dermatofibrosarcoma protuberans INTRODUCTION Dermatofibrosarcoma protuberans (DFSP) is an uncommon, soft tissue sarcoma of mesenchymal origin characterized by its slow, infiltrative growth pattern and high risk of local recurrence after standard surgical excision. We describe a young man with a recurrent dermatofibrosarcoma protuberans after wide local excision treated with neoadjuvant imatinib mesylate and Mohs micrographic surgery, which revealed transformed tumor histology.
منابع مشابه
Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans
Dermatofibrosarcoma protuberans (DESP) is a relatively rare and low-grade tumor of the skin and soft tissue. We review the clinical features, pathological findings and management of recurrent DESP with the aim to improve our understanding of this rare tumor. Fifty-seven patients were diagnosed with recurrent DFSP. The mean age at the presentation of DFSP was 30.9 years. The mean age at diagnosi...
متن کاملDiagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline.
Dermatofibrosarcoma protuberans (DFSP) is a skin fibroblastic tumour that is locally aggressive, with a tendency for local recurrence, but rarely metastasizes. A unique collaboration of multi-disciplinary experts from the European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization of Research and Treatment of Cancer (EORTC) was formed to m...
متن کاملDermatofibrosarcoma protuberans: clinicopathological aspects of an unusual cutaneous tumor.
Dermatofibrosarcoma protuberans is a rare cutaneous tumor with particular characteristics and a high frequency of recurrence after inadequate primary treatment. Its histopathological diagnosis might be difficult. Dermatofibrosarcoma protuberans can be safely distinguished from other similar neoplasms of mesenchymal origin based on the immunohistochemical expression of CD34 antigen and the genet...
متن کاملDermatofibrosarcoma protuberans: from translocation to targeted therapy
Dermatofibrosarcoma protuberans (DFSP), the most common dermal sarcoma, is a low-grade, slow growing fibroblastic malignant neoplasm that most frequently affects middle aged adults and is characterized by a high local recurrence rate and a low propensity for metastasis. Wide surgical resection or Mohs micrographic surgery (MMS) are the preferred approaches for localized disease, while radiation...
متن کاملImatinib mesylate as a preoperative therapy in dermatofibrosarcoma: results of a multicenter phase II study on 25 patients.
AIMS The treatment of dermatofibrosarcoma protuberans (DFSP) involves wide local excision with frequent need for reconstructive surgery. A t(17;22) translocation resulting in COL1A1-PDGFB fusion is present in >95% of cases. Certain patient observations and a report on nine patients suggest that imatinib mesylate, targeting platelet-derived growth factor receptor beta, has clinical potential in ...
متن کامل