Dermatofibrosarcoma protuberans

The dermatofibrosarcoma protuberans is a rare tumor of the skin, locally aggressive, infiltrative growth, but rarely metastasizes ( specify 5 % to less than 0.5 % of cases). After surgical treatment of the tumor is prone to local recurrence.

Occurrence

The tumor is rare with a new onset of less than 1 per 100,000 population per year. It usually affects patients at the age of 40, with men and women are equally affected. In childhood, the tumor is extremely rare.

Appearance

The tumor usually occurs on the trunk and the proximal portions of the extremities. It spreads irregularly in the skin and in the subcutaneous adipose tissue and has a firm consistency. He usually appears skin color. Yellow, brown or reddish discoloration are possible.

Diagnostics

A definitive diagnosis is usually possible only by histological examination. The tumor has more skin tumors as well as various forms of malignant melanoma be differentiated from various forms of benign Dermatofibroms and dermatomyofibroma one hand, and against the prognosis much worse pleomorphic sarcoma ( malignant fibrous histiocytoma ) as well.

Histologically there is a diffuse spreading along the septa of subcutaneous fat with partially several centimeters long processes. The cells are stored dense, relatively uniform spindle-shaped and often CD34 -positive.

Higher magnification.

Higher magnification.

Immunohistochemistry: CD34

The preoperative examination of the tumor in sonography, computed tomography or magnetic resonance imaging can be helpful, especially at lower infiltration, but the safe representation of the outer tumor strands is not guaranteed.

Therapy

Treatment consists of surgical removal. Because of the possibility of only microscopically detectable tumor foothills of the topographic marker and the complete histological examination of Exzisats is recommended. If then transcend even tumor strands over the cut edge can be resected at the corresponding topographic point again. In this method, a safety distance of 1 cm is considered sufficient. Without this as micrographic surgery procedure called recurrences are common even at much larger distances ( 5cm or more).

If, for example, due to working or infiltration of the tumor can not be removed surgically or with multiple recurrences, a radiation therapy may be indicated.

A recent promising treatment provides the active ingredient imatinib, which is taken as a tablet. Imatinib for the treatment of non- operable primary, locally recurrent, or metastatic Dermatofibrosarcomata protuberans admitted. If possible, the treatment should take place with imatinib in clinical trials.

Swell

• S1 guideline dermatofibrosarcoma protuberans of the German Dermatological Society ( DDG) and the German Cancer Society. In: AWMF online ( 2007)
• Http://www.krebsgesellschaft.de/download/ll_g_05.pdf