Unusual cutaneous malignancies of the head and neck region

Abstract

Objectives: Cutaneous malignancies are the most common cancer in the world. Although the more common basal cell, squamous cell, and melanoma are well known, a number of other rare types exist. We review the epidemiology, presentation, diagnosis, treatment, and prognosis of 6 types of unusual cutaneous malignancies presenting in the head and neck region. Methods: We conducted an institutional review of all cases of angiosarcoma, merkel cell carcinoma, microcystic adnexal carcinoma, atypical fibroxanthoma, dermatofibrosarcoma protuberans and sebaceous gland carcinoma presenting in the head and neck region in the last 10 years. Age at diagnosis, gender, site of lesion, predisposing factors (history of radiation, smoking, alcohol, previous malignancy), primary treatment and treatment of recurrence were noted. Results: A total of 49 patients were identified, results are presented per type of lesion. Average age at diagnosis for all lesions was 65. The youngest patient in this series was 21 years old with a diagnosis of dermatofibrosarcoma protuberans. All primary lesions were resected. Five patients with Merkel cell, 1 with microcystic adnexal and 2 of 3 patients with angiosarcoma presented with metastatic disease. The third patient with angiosarcoma developed metastatic disease 8 years after the initial diagnosis. Conclusion: These lesions are relatively rare, but some have a predilection for the head and neck region and can easily be misidentified as more common lesions. Careful pathological examination with clinical correlation is essential in the diagnosis, treatment and prognosis of these unusual cutaneous malignancies.