While dermatofibrosarcoma protuberans most commonly involves the trunk, extremities, scalp, and neck, occasional cases located in the face.
Dermatofibrosarcoma protuberans (DFSP) is a rare type of malignant tumor that occurs in the dermis, the second layer of the skin. Although metastasis rarely occurs, DFSP is a locally aggressive tumor with a high recurrence rate. It usually presents in early or middle adult life between 20 and 50 years of age, but all ages can be affected. The tumour is rare in children. Dermatofibrosarcoma protuberans usually presents as a large indurated plaque several centimeters in diameter. It is composed of firm, irregular nodules varying in color from flesh to reddish brown. Males are affected slightly more frequently than females. There does not appear to be any racial predilection. It is locally aggressive and highly recurrent malignant neoplasm.
Dermatofibrosarcoma protuberans usually does not spread to other parts of the body (metastasize). Dermatofibrosarcoma protuberans is a cutaneous malignancy that arises from the dermis and invades deeper subcutaneous tissue (eg, fat, fascia, muscle, bone). A small subset of DFSP patients presents with fibrosarcomatous progression. This fibrosarcomatous progression dermatofibrosarcoma protuberans variant is more aggressive in nature and the clinical outcome usually is poor. It usually grows very slowly over months to years. It rarely presents in childhood and is even more rarely present at birth. The clinical diagnosis of DFSP in infancy or childhood may be difficult because, in its early stages, the tumor often resembles a vascular birthmark.
Protuberans of Dermatofibrosarcoma is characterized by its aggressive local invasion. The tumour invades local fabric below by prolonging projections of tentaclelike healthy skin making the displacement complete of the tumour very difficult. Incomplete removal of these neoplastic cells results in a high local recurrence rate. The tumor begins as a very small bump on the surface of the skin, sometimes having a reddish-brown or reddish-blue color. The tumor then undergoes a period of very rapid growth where the many small growths in the dermis form a larger, more noticeable mass. Dermatofibrosarcoma protuberans most commonly occurs on the trunk, followed by the proximal extremities. It rarely occurs above the neck. Sometimes, it may present as a morphealike, atrophic, sclerotic, violaceous plaque without nodularity that may ulcerate as it slowly increases in size.
Because initial the increase phase is this way slow, it can be being caused a couple year before the tumour noticeable and enough inconvenience for the patient to seek a diagnosis. Currently, chemotherapy is rarely used in the treatment of dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans has commonly been treated with wide excision, which means the tumor and a large amount of the tissue surrounding it are removed. Radiation therapy may be recommended for patients if the margins of resection are positive or for situations where adequate wide excision alone may result in major cosmetic or functional deficits. Close follow-up care after radiation therapy is warranted because some of the tumors may become more aggressive. Mohs’ microscopically controlled surgery is another option that has been very effective. This procedure involves removing the tumor, then continuing to remove the edges of tissue around the tumor site.
Dermatofibrosarcoma protuberans usually does not spread to other parts of the body (metastasize). Dermatofibrosarcoma protuberans is a cutaneous malignancy that arises from the dermis and invades deeper subcutaneous tissue (eg, fat, fascia, muscle, bone). A small subset of DFSP patients presents with fibrosarcomatous progression. This fibrosarcomatous progression dermatofibrosarcoma protuberans variant is more aggressive in nature and the clinical outcome usually is poor. It usually grows very slowly over months to years. It rarely presents in childhood and is even more rarely present at birth. The clinical diagnosis of DFSP in infancy or childhood may be difficult because, in its early stages, the tumor often resembles a vascular birthmark.
Protuberans of Dermatofibrosarcoma is characterized by its aggressive local invasion. The tumour invades local fabric below by prolonging projections of tentaclelike healthy skin making the displacement complete of the tumour very difficult. Incomplete removal of these neoplastic cells results in a high local recurrence rate. The tumor begins as a very small bump on the surface of the skin, sometimes having a reddish-brown or reddish-blue color. The tumor then undergoes a period of very rapid growth where the many small growths in the dermis form a larger, more noticeable mass. Dermatofibrosarcoma protuberans most commonly occurs on the trunk, followed by the proximal extremities. It rarely occurs above the neck. Sometimes, it may present as a morphealike, atrophic, sclerotic, violaceous plaque without nodularity that may ulcerate as it slowly increases in size.
Because initial the increase phase is this way slow, it can be being caused a couple year before the tumour noticeable and enough inconvenience for the patient to seek a diagnosis. Currently, chemotherapy is rarely used in the treatment of dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans has commonly been treated with wide excision, which means the tumor and a large amount of the tissue surrounding it are removed. Radiation therapy may be recommended for patients if the margins of resection are positive or for situations where adequate wide excision alone may result in major cosmetic or functional deficits. Close follow-up care after radiation therapy is warranted because some of the tumors may become more aggressive. Mohs’ microscopically controlled surgery is another option that has been very effective. This procedure involves removing the tumor, then continuing to remove the edges of tissue around the tumor site.
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