Dermatofibrosarcoma protuberans

Beskrivning

Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumour in the deep layers of skin. This condition is a type of soft tissue sarcoma, which are cancers that affect skin, fat, muscle, and similar tissues.

In dermatofibrosarcoma protuberans, the tumour most often starts as a small, firm patch of skin, usually 1 to 5 centimeters in diameter, that is usually purplish, reddish, or flesh-colored. The tumour typically grows slowly and can become a raised nodule. Occasionally, the tumour begins as a flat or depressed patch of skin (plaque). Tumours are most commonly found on the torso and can also be found on the arms, legs, head, or neck. Affected individuals usually first show signs of this condition in their thirties, but the age at which a tumour appears varies widely.

In dermatofibrosarcoma protuberans, the tumour has a tendency to return after being removed. However, it does not often spread to other parts of the body (metastasize).

There are several variants of dermatofibrosarcoma protuberans in which different cell types are involved in the tumour. Bednar tumours, often called pigmented dermatofibrosarcoma protuberans, contain dark-colored (pigmented) cells called melanin-containing dendritic cells. Myxoid dermatofibrosarcoma protuberans tumours contain an abnormal type of connective tissue known as myxoid stroma. Giant cell fibroblastoma, which is sometimes referred to as juvenile dermatofibrosarcoma protuberans because it typically affects children and adolescents, is characterised by giant cells in the tumour.

Rarely, the tumours involved in the different types of dermatofibrosarcoma protuberans can have regions that look similar to fibrosarcoma, a more aggressive type of soft tissue sarcoma. In these cases, the condition is called fibrosarcomatous dermatofibrosarcoma protuberans or FS-DFSP. FS-DFSP tumours are more likely to metastasize than tumours in the other types of dermatofibrosarcoma protuberans.

Inheritance patterns

The source lists the following possible inheritance patterns: Not inherited. More than one pattern can apply in different cases.

Related genes

• COL1A1

• PDGFB

Other names

Darier-Ferrand tumour

Darier-Hoffmann tumour

Dermatofibrosarcoma

DFSP