Merkel cell carcinoma: a report of 34 cases and literature review

Summary Background Merkel cell carcinoma (MCC) is a rare and aggressive skin cancer, with unclear histogenesis. To date there is no consensus on the optimal treatment of this neoplasm, with controversy surrounding the use of radiotherapy and chemotherapy. There are also limited data on biological behaviour and prognosis, with reported survival ranging from 31% at three years to 74% at five years. Method The medical records of 34 patients with a diagnosis of primary MCC, treated at two NHS trusts in Birmingham and Coventry, were reviewed. An extensive review of the English literature was also performed. Results MCC occurred predominantly in Caucasians (97%) with a mean age of 75 years. Identified risk factors were a previous history of SCC (37%), BCC (18%) and AK (20%). Ten percent of patients showed evidence of immunocompromise. Most tumours were located on the extremity, where they reached a mean size of 2.1cm. Fifty percent had regional metastasis during the course of their disease. A sub group analysis of the excision margins showed that a 2-cm excision margin, extending to the deep fascia, resulted in a 50% incomplete excision rate and a 33% local recurrence rate. In contrast a 3-cm margin including deep fascia resulted in no incomplete excisions and a 10.5% local recurrence rate Prognosis was poor with a 40% 3-year survival. Combining the data from two trusts has produced a relatively large series and highlighted differences in patient characteristics and management between the units. We advocate a 3-cm excision margin, including fascia wherever possible, combined with post-operative radiotherapy to offer the best chance of local control. Survival is fairly dismal and in keeping with the aggressive nature of this tumour. The respective roles of radiotherapy and chemotherapy remain controversial.