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口腔底部的大细胞神经内分泌癌:罕见部位的罕见实体。

Large cell neuroendocrine carcinoma on the floor of the mouth: a rare entity in an unusual location.

机构信息

Oral and Maxillofacial Surgery Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain; XXX.

Oral and Maxillofacial Surgery Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain.

出版信息

Int J Oral Maxillofac Surg. 2022 Jun;51(6):742-745. doi: 10.1016/j.ijom.2021.08.021. Epub 2021 Sep 2.

Abstract

Neuroendocrine carcinomas (NEC) are a group of malignant neoplasms usually located in the lungs or gastrointestinal tract. Fewer cases are located in the head and neck, and in these rare presentations, the lingual tonsil, larynx, and major salivary glands are the most frequently affected sites. NECs exhibit similar characteristics regardless of where they arise. However, because these neoplasms are rare, a clear understanding of their aetiopathogenesis has yet to be described, and options for treatment have varied and are not unified. A rare NEC of the floor of the mouth is reported here; it appears that this location has not been reported previously. The diagnosis was established through histopathological analysis, and the patient underwent systemic treatment. He had a partial response to treatment in the first 3 months, but died 6 months after the initial diagnosis. This highly uncommon tumour can pose a significant diagnostic challenge for clinicians and pathologists alike and can result in diagnostic delay.

摘要

神经内分泌癌(NEC)是一组恶性肿瘤,通常位于肺部或胃肠道。较少的病例位于头颈部,在这些罕见的表现中,舌扁桃体、喉和大涎腺是最常受影响的部位。无论起源于何处,NEC 都具有相似的特征。然而,由于这些肿瘤很少见,其病因发病机制尚未明确描述,治疗选择也各不相同,尚未统一。本文报告一例罕见的口底 NEC,据报道,该部位以前尚未报道过。通过组织病理学分析确定诊断,患者接受了全身治疗。他在最初 3 个月的治疗中出现部分缓解,但在初始诊断后 6 个月死亡。这种极罕见的肿瘤可能对临床医生和病理学家都构成重大诊断挑战,并可能导致诊断延迟。

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