Dermatofibrosarcoma Protuberans of the Head and Neck

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Abstract
Background: Dermatofibrosarcoma protuberans (DFSP) of the head and neck is a rare, locally infiltrative, low-grade sarcoma. This study defines the clinical 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 patients with pathologically confirmed head and neck DFSP treated at Memorial Sloan-Kettering Cancer Center between 1964 and 1999. Factors were analyzed by using Fisher’s exact or (2 tests. Results: For 21 primary and 12 recurrent patients, median age and tumor size at presentation was 39 years and 2.0cm, respectively. Thirty-two (97%) patients were alive at a median follow-up of 82 months. Three patients recurred locally, all with smaller than 2-cm resection margins. Deep tumors were more likely to have a margin-positive resection than superficial lesions (P = .03). Gross margin 2cm or more was a significant predictor of a negative histological margin (P < .001). There was a trend toward improved recurrence-free survival for tumors treated with wide (≥2 cm) margin resection (P = .059). Accuracy, sensitivity, specificity, and false negative rates of frozen section were 80%, 43%, 100%, and 57%, respectively. Conclusions: Wide margin resection of head and neck DFSP predicts negative histological margins and impacts favorably on local recurrence-free survival. Frozen section analysis does not assess resection margins accurately.

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