We report on two young adults with KID syndrome and follicular hyperkeratosis, hidradenitis suppurativa of the groin, progressive development of proliferative pilar cysts and dissecting cellulitis of the scalp, who developed metastatic malignant pilar tumors. Based on our findings, we believe that cancer surveillance in patients with KID syndrome should include screening for pilar tumors and their early removal to avoid development of malignant proliferating pilar tumors with poor prognosis.
Dermatofibrosarcoma protuberans of the head and neck is a tumor that shows extensive infiltration beyond gross margins. Wide local excision with 2- to 3-cm margins results in an unacceptably high recurrence rate; larger excisional margins are necessary to remove all disease. Accurate margin identification achieved by using Mohs surgery with rush paraffin section analysis results in complete tumor removal and excellent control rates. This method should be preferred for treatment of dermatofibrosarcoma protuberans of the head and neck.
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