Lombardi Davide, Accorona Remo, Ungari Marco, Melocchi Laura, Bell Diana, Nicolai Piero
Department of Otorhinolaryngology, University of Brescia, Piazza Spedali Civili 1, 25100, Brescia, Italy.
Head Neck Pathol. 2015 Jun;9(2):309-14. doi: 10.1007/s12105-014-0573-1. Epub 2014 Oct 15.
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.
默克尔细胞癌是一种神经内分泌肿瘤，主要发生在暴露于阳光的皮肤上，皮肤外部位罕见。它是唾液腺极其罕见的恶性神经内分泌肿瘤之一。我们报告一例右下颌下腺原发性默克尔细胞癌。术前诊断存疑，鉴于该肿瘤极其罕见，明确的组织学诊断非常困难。术中对病变宏观特征的评估导致了选择性淋巴结清扫。该疾病的极度侵袭性导致了新的术后分期的必要性以及多模式治疗。这是文献中描述的首例原发性下颌下腺默克尔细胞癌。鉴别诊断可能具有挑战性，苏木精-伊红染色和免疫组化研究是必不可少的。
