Primary Merkel Cell Carcinoma of the Submandibular Gland: When CK20 Status Complicates the Diagnosis

Merkel cell carcinoma is a neuroendocrine tumor that occurs predominantly on the sun-exposed skin, with rare cases in the extracutaneous sites. It represents one of the extremely rare malignant neuroendocrine tumors of the salivary glands. We report a case of primary Merkel cell carcinoma of the rig...

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Veröffentlicht in:Head & neck pathology (Totowa, N.J.) N.J.), 2015-06, Vol.9 (2), p.309-314
Hauptverfasser: Lombardi, Davide, Accorona, Remo, Ungari, Marco, Melocchi, Laura, Bell, Diana, Nicolai, Piero
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container_issue 2
container_start_page 309
container_title Head & neck pathology (Totowa, N.J.)
container_volume 9
creator Lombardi, Davide
Accorona, Remo
Ungari, Marco
Melocchi, Laura
Bell, Diana
Nicolai, Piero
description Merkel cell carcinoma is a neuroendocrine tumor that occurs predominantly on the sun-exposed skin, with rare cases in the extracutaneous sites. It represents one of the extremely rare malignant neuroendocrine tumors of the salivary glands. We report a case of primary Merkel cell carcinoma of the right submandibular gland. The preoperative diagnosis was doubtful and the definitive histological diagnosis proved to be very difficult considering the extreme rarity of this tumor. The intraoperative evaluation of the macroscopic characteristics of the lesion led to an elective lymph node dissection. The extreme aggressiveness of the disease has resulted in the necessity of a new post-operative staging and in a multimodal treatment. This is the first primary submandibular gland Merkel cell carcinoma described in the literature. Differential diagnosis may be challenging and proper hematoxylin-eosin staining and immunohistochemical studies are mandatory.
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subjects Aged
Biomarkers, Tumor - metabolism
Carcinoma, Merkel Cell - diagnosis
Carcinoma, Merkel Cell - metabolism
Carcinoma, Merkel Cell - pathology
Case Report
Combined Modality Therapy
Dentistry
Diagnosis, Differential
Female
Humans
Immunohistochemistry
Keratin-20 - metabolism
Magnetic Resonance Imaging
Medicine
Medicine & Public Health
Neuroendocrine Tumors - diagnosis
Neuroendocrine Tumors - metabolism
Neuroendocrine Tumors - pathology
Oral and Maxillofacial Surgery
Otorhinolaryngology
Pathology
Submandibular Gland Neoplasms - diagnosis
Submandibular Gland Neoplasms - metabolism
Submandibular Gland Neoplasms - pathology
Treatment Outcome
title Primary Merkel Cell Carcinoma of the Submandibular Gland: When CK20 Status Complicates the Diagnosis
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