Management of Merkel cell carcinoma of unknown primary origin: the Vienna Medical School experience

Merkel cell carcinoma is a rare, but highly aggressive skin tumor. We describe our single-institution experience with the diagnosis and treatment of Merkel cell carcinoma of unknown primary (MCCUP). We conducted a retrospective medical chart review of patients treated with MCCUP at the Vienna General Hospital between 2002 and 2011. Clinicopathologic variables and outcomes were analyzed. Of the entire cohort of 57 patients, 8 patients (14 %) were diagnosed with MCCUP. Three patients presented with parotid gland involvement, four patients with positive inguinal lymph nodes and one with axillar lymph nodes. CK20 staining was positive in all tumor specimens, whereas MCPyV protein was positive in four out of six patients. The primary surgical modality in five cases was wide local excision. In one patient excisional biopsy was followed by re-resection. In one case only excisional biopsy was performed due to metastatic disease at first diagnosis. Two patients underwent concomitant parotidectomy and neck dissection, and four patients received adjuvant radiation therapy. Median recurrence-free survival was 20 months. Four patients died, three of disease and one of other cause. Recurrent disease was observed in two patients and treated with radiotherapy and chemotherapy. The 1- and 3-year overall survival rates were 87.5 and 37.5 %, respectively. The 1- and 3-year disease-specific survival rates were 87.5 and 62.5 %, respectively. Our study shows a poor outcome in patients with MCCUP, particularly in patients with node involvement of the trunk. We therefore suggest an aggressive and multimodal treatment approach for patients with MCCUP.