Dermatofibrosarcoma Protuberans of the Head and Neck

Abstract

\s=b\To elucidate the natural history of dermatofibrosarcoma protuberans of the head and neck, 17 cases were reviewed and compared with 92 others contained within the English literature. The prototypic case is seen as a firm, solitary, slowly enlarging, asymptomatic, cutaneous nodule between 2 and 5 cm in size and of two years' duration. It is more common in men and in persons aged between 30 and 40 years. The scalp and neck-supraclavicular fossa are the most common sites, accounting for half of the cases. Approximately 50% to 75% of the patients experience local recurrence after initial surgery and, of these, half to three fourths manifest within three years of treatment.Metastases, seen in no more than 6% of all cases, are almost invariably preceded by two or more local recurrences and occur primarily through the bloodstream to the lungs and bones and less often to regional lymph nodes. Prognostic factors, differential diagnosis, and histogenesis are included. (Arch Otolaryngol 1984;110:398-404) Dermatofi brosarcoma protuberans (DFSP) is a slowly growing, locally aggressive, rarely metastasiz¬ ing, malignant mesenchymal neo¬ plasm of the skin, which frequently recurs following attempts at exci¬ sion.

Dermatofibrosarcoma protuberans treated by micrographic surgery

“…Similar recurrence rates were found by McPeak in 82 patients, and a series of 119 patients treated by wide excision (McPeak et al, 1967;Hajdu, 1979). The head and neck is a more common site for recurrence, and it is unclear whether this reflects the difficulties in excising DFSP over this area (Barnes and Coleman, 1984;Mark et al, 1993). A small number of patients, however, present with a very delayed recurrence, up to 20 years after excision (McPeak et al, 1967;Mikhail and Lynn, 1978;Roses et al, 1986).…”

“…The risk of metastasis appears to increase with repeated incomplete excision and de-differentiation towards a higher-grade fibrosarcoma (Taylor and Helwig, 1962). Fatalities from direct intracerebral invasion have also been reported (McPeak et al, 1967;Barnes and Coleman, 1984).…”

Sarcomas cutâneos primários

“…27 Most local recurrences (between 50 and 75%) are noted three years after excision. Late recurrences, more than 10 years post-excision, although rare, have been described.…”

“…27 Acral and genital dermatofibrosarcomas are infrequent. 27,28 Initially, it presents as a hardened plaque, asymptomatic, violaceous, reddish-brown, or slightly hyperchromic, similar to a cheloid. The indolent behavior of DFSP and its imprecise characteristics frequently lead to a delay in its being perceived by patients, and hence, a late diagnosis.…”

Distant Metastasis of Dermatofibrosarcoma Protuberans of the Scalp-Case Report-

“…10) DFSP has since been considered as a fibrohistiocytic tumor of intermediate malignancy characterized by local recurrence. 2,7) DFSP has an incidence of only 0.8 cases per million persons per year, 13) and typically presents during early-or mid-adult life and predominantly in males. The location is most frequently on the trunk and proximal extremities, but may occur at almost any site.…”