Unit of Otorhinolaryngology, Department of Neuroscience, Reproductive Sciences and Dentistry, Federico II University of Naples, Naples, Italy.
Unit of Otorhinolaryngology, Hospital Cardarelli, Naples, Italy.
J Int Med Res. 2021 May;49(5):3000605211014784. doi: 10.1177/03000605211014784.
This current report describes a rare clinical case of neuroendocrine carcinoma (NEC) of the larynx with associated Lambert-Eaton myasthenic paraneoplastic syndrome (LEMS). A 68-year-old male patient presented with severe dysphonia and dysphagia. He underwent a total laryngectomy and the excised lesion was extremely large. A pathological examination demonstrated 'morphological findings of a poorly differentiated carcinoma (G3) with aspects of neuroendocrine differentiation' (i.e. a poorly differentiated neuroendocrine carcinomas [PD-NEC]). Based on the patient's medical history and the immunohistochemical findings, he was treated with three cycles of neoadjuvant chemotherapy (cisplatin-etoposide) and then radiotherapy with a total dose of 70 Gy. Of the 10 cases of paraneoplastic syndrome (PNS) related to laryngeal NEC reported in the literature, nine of these syndromes were of an endocrine type. Only one case of PNS associated with laryngeal cancer had a neurological manifestation, which was LEMS. To the best of our knowledge, this current case has only one similar precedent in the literature and it is the second report of an association between a PD-NEC and LEMS. Laryngeal NECs are rare lesions with different prognostic characteristics. The diagnosis should be made using an endocrinological, neurological, radiological and histological multidisciplinary approach. A radical surgical approach is recommended.
本现报告描述了一例罕见的喉神经内分泌癌(NEC)合并 Lambert-Eaton 肌无力综合征(LEMS)的临床病例。一名 68 岁男性患者因严重声音嘶哑和吞咽困难就诊。他接受了全喉切除术,切除的病变组织非常大。病理检查显示“形态学表现为低分化癌(G3),具有神经内分泌分化特征”(即低分化神经内分泌癌[PD-NEC])。根据患者的病史和免疫组织化学检查结果,他接受了三个周期的新辅助化疗(顺铂-依托泊苷),然后接受了 70Gy 的放疗。在文献报道的 10 例与喉 NEC 相关的副肿瘤综合征(PNS)中,有 9 例为内分泌类型。仅有一例 PNS 与喉癌有关,表现为 LEMS。据我们所知,目前该病例在文献中仅有一个类似的先例,这是 PD-NEC 与 LEMS 之间关联的第二例报告。喉 NEC 是具有不同预后特征的罕见病变。诊断应采用内分泌学、神经病学、影像学和组织学的多学科方法。建议采用根治性手术方法。