Merkel cell carcinoma (MCC) is a rare cutaneous malignancy occurring mostly in older immunocompromized Caucasian males. A growing incidence of MCC has been reported in epidemiological studies. Treatment of MCC usually consists of surgical excision, pathological lymph node evaluation, and adjuvant radiotherapy. This paper reports the experience of a single tertiary center institution with 17 head and neck Merkel cell carcinoma patients. Median followup for the cohort was 37.5 months. After five years, recurrence-free survival, disease specific survival, and overall survival were 85%, 90%, and 83%, respectively. Our limited data support the use of adjuvant radiotherapy. We also report two cases of MCC located at the vestibule of the nose and two cases of spontaneous regression after diagnostic biopsy. About 40% of our patients were referred to our center for surgical revision and pathological lymph node evaluation. Increased awareness of MCC and an interdisciplinary approach are essential in the management of MCC. 1. Introduction In 1875, Merkel, professor of anatomy at the University of Rostock, Germany, for the first time described “Tastzellen” (touch cells)—later known as Merkel cells—in the epidermis of domestic animals and humans [1]. In 1972, Toker first reported a case series of five patients with “trabecular carcinoma” and recognized a “distinct pathological entity,” with a “capricious clinical behaviour” [2]. It took Tang and Toker another six years to determine that trabecular carcinoma “most probably” derives from Merkel cells [3]. More recently, in 2008, a previously unknown polyomavirus was found to be integrated in Merkel cell carcinoma (MCC) [4]. Now known as Merkel Cell polyomavirus (MCV), this virus is indeed thought to be a causative agent in MCC [5, 6] and has been associated with about 80% of MCC cases [4, 7–10]. A previous study from our institution reported similar prevalence of MCV in MCC [11]. Conflicting evidence exists about the prognostic value of MCV status [7, 8, 12–14]. Important differential diagnoses of MCC are basal cell carcinoma, small cell melanoma, lymphoma, small blue round cell tumours, and especially metastatic small cell lung carcinoma [15]. A thorough histo- or cytopathological workup including immunohistochemistry (e.g., CK20, TTF-1, and neuroendocrine markers) [16] combined with entire examination and clinical history usually allows to differentiate the above mentioned entities. A recent study by Smith et al. [17] drawn from an US-population based database (SEER) with over 4,300 MCC patients showed that 48% of
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