Merkel cell carcinoma: a systematic review of ENT presentations
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REVIEW ARTICLE
Merkel cell carcinoma: a systematic review of ENT presentations Federico Maria Gioacchini • Valentina Postacchini Oriana Simonetti • Annamaria Offidani • Giuseppe Magliulo • Massimo Re
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Received: 26 April 2012 / Accepted: 8 November 2012 / Published online: 29 November 2012 Springer-Verlag Berlin Heidelberg 2012
Abstract The aim of this study was to perform a systematic literature review of Merkel cell carcinomas (MCCs) originating exclusively in the ear, nose and throat (ENT) district. An appropriate string was run on PubMed to retrieve articles dealing with ENT presentations of MCC. A double cross-check was performed on citations and full-text articles found using the selected inclusion and exclusion criteria. In total, 43 articles were finally included in the study, describing 51 cases of MCC involving the ENT region: 22/51 (43 %) cases involving the ear; 20/51 (39 %) cases involving the mucosal sites; 9/51 (18 %) cases involving other ENT regions. Patients with mucosal site involvement showed a higher mortality rate from the disease (45 %) in comparison with the other two groups, especially when compared with those patients having primary involvement of the ear (22 %). The ENT specialist should suspect and consider MCC, especially in elderly patients presenting with a suspicious lesion of the auricular pavilion, so as to avoid misdiagnosis and delayed treatments.
F. M. Gioacchini (&) Otolaryngology Department, University Hospital of Modena, Via del Pozzo 71, 41100 Modena, Italy e-mail: [email protected] V. Postacchini O. Simonetti A. Offidani Dermatology Department, Marche Polytechnic University, Ancona, Italy G. Magliulo Department of Otorhinolaryngology ‘‘G. Ferreri’’, ‘‘La Sapienza’’ University, Rome, Italy M. Re Otorhinolaryngology Department, Marche Polytechnic University, Ancona, Italy
Keywords Merkel cell carcinoma Pinna Nose ENT mucosal sites Parotid gland
Introduction Merkel cell carcinoma (MCC) is an extremely rare primary neuroendocrine neoplasm of the skin that shows aggressive behavior and a poor prognosis [1]. This tumor, also known as trabecular carcinoma of the skin and primary neuroendocrine carcinoma of the skin, among other designations, has been the subject of numerous articles and case reports [2]. The estimated incidence is 0.23 per 1,00,000 Caucasians and only 0.01 per 1,00,000 in black populations [3]. It occurs, especially in elderly and immunosuppressed patients, particularly in organ transplant recipients with a slight male predominance [4]. MCC manifests typically as a slowly enlarging, pink or bluish-red plaque or nodular subcutaneous lesion, which is rarely ulcerated [5]. Its anatomical–pathological diagnosis is complicated, as it can easily be confused with cutaneous metastases of other tumors [6]. The survival rate is largely dependent on tumor size at presentation [4], with a recently reported 3-year rate of 31–68 % and a local recurrence rate of 55–79 % [7]. MCC typically invades both lymphatic channels and blood vessels, which often leads to
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