Evidence and Considerations on Treatment of Small Size Merkel Cell Head and Neck Carcinoma

THIEME Original Research Evidence and Considerations on Treatment of Small Size Merkel Cell Head and Neck Carcinoma Elena Festa Kotelnikova1 Melissa Laus2 Adelchi Croce1 1 Department of ENT, University “G. d’Annunzio” of Chieti-Pescara, Hospital “SS Annunziata,” Chieti, Italy 2 Department of Otolaryngology, General Hospital “S. Giovanni Calibita - Fatebenefratelli”, the Tiber Island, Rome, Italy Address for correspondence Laus Melissa, MD, Department of Otolaryngology, General Hospital “S. Giovanni Calibita Fatebenefratelli,” the Tiber Island, Rome, Italy (e-mail: ). Int Arch Otorhinolaryngol 2020;24(4):e487–e491. Abstract Keywords ► merkel cell carcinoma ► merkel cell carcinoma treatment ► neuroendocrine tumor ► rare tumor ► head and neck cancer Introduction Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine malignant cancer. It is an epidermal cancer common in the head and neck. Objectives Though there is limited number of cases described in the literature for the treatment difficult to obtain. Our purpose was to present the clinical course and treatment of four patients with MCC. Methods We conducted a retrospective analysis and obtained detailed clinical information for all 4 patients treated for MCC at the ENT Department of the SS Annunziata Hospital in Chieti, Italy, from 2013 through 2015. Results In our study, two patients presented with the tumor in a rare site (lower eyelid). All of the patients underwent surgical treatment: three patients had free excision margins and negative sentinel lymph nodes (SLNs) while 1 patient had free excision margins and positive SLNs. The latter patient underwent ipsilateral neck dissection. In another patient, the fluorodeoxyglucose positron emission topography (FDG PET)/computed tomography (CT) performed 6 months after the surgery has shown high metabolic activity in the left parotid gland, and the patient underwent total parotidectomy and a neck dissection. Conclusion Sentinel lymph node biopsy is a useful technique in small size MCCs of the head and neck. However, the parotid gland should be strictly controlled in patients with lower eyelid tumors. Introduction Merkel cell carcinoma (MCC) is a rare and highly aggressive cutaneous-neuroendocrine tumor that presents as a rapidly growing, solitary, cutaneous or subcutaneous, pink-to-violet nodule located mostly on sun-exposed areas. The MCC prognosis is considered as bad as that of melanoma. The incidence rate ranges from 0.13 per 100,000 people in Europe (between 1995 and 2002) to 1.6 per 100,000 people in Australia (between 1993 and 2010).1–4 The average age at the time of diagnosis is 76 years old. At the primary diagnosis, loco-regional metastases are received December 16, 2018 accepted January 26, 2020 DOI https://doi.org/ 10.1055/s-0040-1709114. ISSN 1809-9777. already present in
30%5 of the patients, and the rate of local recurrence after treatment is of between 40 and 77%. Metastases are localized in the skin (28%), lymph nodes (27%), the liver (13%), the lungs (10%), bones (10%) and the brain (6%), and the estimated mortality rate is between 33 and 46%.1,3,6,7 Merkel cell carcinoma takes its name from the small grains of the tumor cells similar to the grains of the Merkel cells. The markers are neuron specific enolase, chromogranin, synaptophysin and CD56, the same of pulmonary microcytoma, and cytokeratin 20. Copyright © 2020 by Thieme Revinter Publicações Ltda, Rio de Janeiro, Brazil 487 International Archives of Otorhinolaryngology Vol. 24 No. 4/2020 Abbreviations: AJCC/TNM, American Joint Committee on Cancer/Tumor lymph nodes and metastasis Staging Classification; LN, lymph node; MCC, Merkel cell carcinoma; LN, lymph node; MGUS, monoclonal gammopathy of undetermined significance; NED, No evidence of disease; PET/CT, positron emission topography/computed tomography; RA, rheumatoid arthritis; SLN, sentinel lymph node; SLNB, sentinel lymph node biopsy; UV, ultraviolet. NED at 7 months LOCAL RECURRENCE after 19 months F 4 88 05/29/2015 Left eyebrow pT1cN0M0 RA, MGUS 08/21/2015 Wide local retroauricular excision þ SLNB FREE MARGINS SLN(1 LN in the very superficial part of the parotid gland anterior to the tragus) – WIDE RESECTION AND RECONSTRUCTION NED at 28 months – NED NECK DISSECTION (N-) FREE MARGINS SLNþ (MCC in 2 LNs of the parotid gland) 07/13/2015 M 3 66 05/01/2015 Left lower eyelid pT1cN0 (pN1)M0 – Wide local excision þ SLNB (superficial parotidectomy) NED at 34 months – NED – 01/07/2015 M 2 81 11/21/2014 Right auricle pT1cN0M0 UV rays exposure Wide local excision þ SLNB FREE MARGINS SLN(1 LN in the neck) LEFT PAROTIDECTOMY þ NECK DISSECTION þ PORT PET/CT POSITIVE after 6 months – FREE MARGINS SLN(2 LNs in the lower part of the parotid gland) 09/11/2013 RA pT1cN0M0 Left lower eyelid 07/31/2013 M 68 TYPE OF TREATMENT DATE OF TREATMENT RISK FACTORS STAGING AJCC/TNM (8th ed., 2016) PRIMARY TUMOUR SITE FIRST DIAGNOSIS (excisional biopsy) AGE 1 All of the patients signed an informed consent form for the processing of personal data. GENDER Ethical Considerations PATIENT We examined 4 patients who arrived at the ENT Department of our Hospital with a diagnosis of cutaneous MCC between August 2013 and June 2015. The patients age ranged from 68 to 88 years old (mean age of 75.75 years old). The MCCs were localized in the upper part of the face (lower eyelid in 2 patients, eyebrow in 1 patient, auricle in 1 patient). The diagnosis was made via an excisional biopsy with close margins performed in other hospitals a few weeks before. The MCCs were initially staged according to the American Joint Committee on Cancer (AJCC) Tumor, lymph nodes and metastasis (TNM) Staging Classification for Merkel Cell Carcinoma (7th ed., 2010). For the present study we retrospectively applied the most recently published AJCC TNM Staging Classification for Merkel Cell Carcinoma (8th ed., 2016) staging criteria.10 All of the patients were pT1cN0M0 at presentation. In agreement with the National Comprehensive Cancer Network (NCCN) guidelines and with the clinical practice guidelines of our institute, we performed a wide local excision (WLE) of the lesion and a sentinel lymph node biopsy (SLNB). All of the patients had follow-up visits (with clinical examination, neck ultrasound and whole body fluorodeoxyglucose positron emission topography (FDG PET)/computed tomography (CT) at least once a year, and magnetic resonance imaging (MRI) with contrast or CT with contrast in some clinical circumstances) scheduled every 3 to 6 months. All of the patients were free of disease at the latest follow-up visit. Because of the small number of patients, statistical analysis was not performed for prognostic indications. ►Table 1 lists the patients (numbered 1–4) and the details of the treatments performed. HISTOLOGY Method Wide local excision þ SLNB FOLLOW-UP SECOND TREATMENT FOLLOW-UP Risk factors for MCC are ultraviolet (UV) radiation ex (...truncated)