Dermatofibrosarcoma protuberans of the head and neck

Background: Dermatofibrosarcoma protuberans (DFSP) of the head and neck is a rare, locally infiltrative, low-grade sarcoma. This study defines the cli nical behavior of DFSP, evaluates the role of frozen section analysis, and identifies factors that predict local control. Methods: Hospital records and pathological slides were reviewed for 33 pati ents with pathologically confirmed head and neck DFSP treated at Memorial S loan-Kettering Cancer Center between 1964 and 1999. Factors were analyzed b y using Fisher's exact or chi(2) tests. Results: For 21 primary and 12 recurrent patients, median age and tumor siz e at presentation was 39 years and 2.0 cm, respectively. Thirty-two (97%) p atients were alive at a median follow-up of 82 months, Three patients recur red locally, all with smaller than 2-cm resection margins. Deep rumors were more likely to have a margin-positive resection than superficial lesions ( P = .03). Gross margin 2 cm or more was a significant predictor of a negati ve histological margin (P < .001). There was a trend toward improved recurr ence-free survival for tumors treated with wide (greater than or equal to 2 cm) margin resection (P = .059). Accuracy, sensitivity, specificity, and f alse negative rates of frozen section were 80%, 43%, 100%, and 57%, respect ively. Conclusions: Wide margin resection of head and neck DFSP predicts negative histological margins and impacts favorably on local recurrence-free surviva l. Frozen section analysis does not assess resection margins accurately.