Dermatofibrosarcoma protuberans

There is evidence that dermatofibrosarcoma protuberans (DFSP) is composed of some type of spindle cell of the peripheral nerve unit rather than of ordinary fibroblasts. They can grow into surrounding tissue but do not spread to other parts of the body. These tumors are related to giant cell fibroblastoma.

Causes of dermatofibrosarcoma protuberans

The cause of DFSP is unknown. Recent studies show abnormalities of chromosomes within the tumour cells, which may account for their unrestrained growth compared to the surrounding normal cells.

Treatment of dermatofibrosarcoma protuberans

Treatment consists of wide excision of the lesion with several millimetres of normal skin around the margins. Mohs micrographic surgery, which is a special surgical technique to control tumour margins, is sometimes used to check that all the abnormal cells have been excised.

Radiotherapy is also used in addition to surgery. Chemotherapy is ineffective.

Amyloidosis
Connective tissue naevi
Dermatofibroma
Dermatofibrosarcoma protuberans
Ehler's Danlos syndrome
Elastosis perforans serpiginosa
Keloids & hypertrophic scars
Lipodystrophy
Lipoma
Mastocytoma
Neurofibroma
Pseudoxanthoma elasticum
Reactive perforating collagenosis
Striae (stretch marks)
Eosinophilic fasciitis
Erythema multiforme
Histiocytoses
Jessner's lymphocytic infiltrate
Langerhans cell histiocytosis
Lymphocytoma cutis
Lymphomatoid papulosis
Mastocytosis
Morphoea
Non-Langerhans cell histiocytosis
Panniculitis
Reticulohistiocytosis
Sweet's disease
Systemic sclerosis
Atypical mycobacterial infection
Granuloma faciale
Granuloma annulare
Cutaneous tuberculosis
Leprosy
Pyogenic granuloma
Sarcoidosis