Periorbital microcystic adnexal carcinoma with distant metastases

Purpose: Microcystic adnexal carcinoma (MAC) is a locally aggressive adnexal carcinoma of the head and neck, which has a high tendency for recurrence. MAC rarely has distant metastasis, with only one previously reported case originating from the periorbital region. The authors present a patient with periorbital MAC with distant metastasis and a review of all reported patients with metastatic disease. Method: The authors present a patient with the youngest reported periorbital MAC with metastases along with a major review of metastatic MAC using PubMed/MEDLINE databases. Case reports or case series that described a case of MAC with metastases were included. Results: A 37-year-old man with left periorbital MAC had local resection and subsequent exenteration for orbital recurrence. Distal metastases were noted between 8 and 13 years postexenteration in the left parotid gland, left sartorius muscle, and right brachialis and trapezius muscles. There are 7 patients, including the authors' patient, with distant metastasis in MAC, of which 2 were of periorbital origin. The median age was 62 with a male predominance (n = 5/7). The authors' patient is the youngest reported patient with metastatic MAC. The median time to metastasis was 8 years. Strategies to control disease included radiotherapy (n = 4/7) and chemotherapy (n = 4/7). Six of 7 cases passed away with disease or have ongoing disease. Conclusion: Periorbital MAC is a locally aggressive disease with potential for metastatic spread and all patients need long-term surveillance for local recurrence and distant metastases.