Ferlito A, Barnes L, Rinaldo A, Gnepp D R, Milroy C M
Department of Otolaryngology-Head and Neck Surgery, University of Udine, Italy.
J Laryngol Otol. 1998 Sep;112(9):827-34. doi: 10.1017/s0022215100141830.
Neuroendocrine neoplasms of the larynx have been divided into those of epithelial or neural origin. The latter consist of paragangliomas while the epithelial origin group can be divided into the typical and atypical carcinoids and small cell neuroendocrine carcinomata, the latter consisting of the oat cell type, the intermediate cell type and the combined cell type. There are now over 500 cases of neuroendocrine neoplasms of the larynx in the literature. The diagnosis is primarily based on light microscopy, and, in some instances, it may be supported by special histochemical studies. It should be confirmed by immunocytochemical and/or ultrastructural investigation. The different biological behaviour of neuroendocrine neoplasms of the larynx makes a specific diagnosis of paramount importance, since treatment depends on diagnostic accuracy. Typical carcinoid is an extremely rare lesion. It is treated preferably by conservative surgery; elective neck dissection is not necessary because of the lack of lymph node metastases at diagnosis. Chemotherapy and/or radiotherapy have not been effective in the limited number of patients treated thus far. Prognosis is excellent with cure following surgery. Atypical carcinoid is the most frequent non-squamous carcinoma of the larynx. The mainstay of treatment is surgery. Elective neck dissection should be performed because of the high likelihood of cervical lymph node metastases. Primary radiation therapy with adjuvant chemotherapy is not indicated. The survival rate is 48 per cent at five years and 30 per cent at 10 years. Although the larynx is one of its most common extrapulmonary sites, small cell neuroendocrine carcinoma is still a rare tumour. Surgical results for this tumour have been disappointing and is reserved for cases of local relapse with no evidence of metastasis. Chemotherapy and radiotherapy currently appear to offer the least disabling and most effective forms of therapy. The two- and five-year survival rates are 16 per cent and five per cent, respectively. Paraneoplastic syndromes have occasionally been reported in association with carcinoid tumours (typical and atypical) and small cell neuroendocrine carcinoma. There have been also rare reports of an elevated neuropeptide serum level. Paraganglioma is the only laryngeal neuroendocrine neoplasm with a female preponderance (3:1). Confusion with atypical carcinoid has led to incorrect diagnosis and inappropriate classification schemes, erroneously suggesting that laryngeal paraganglioma has the potential for aggressive behaviour. Conservative surgery represents the treatment of choice; elective neck dissection is not necessary, and the prognosis is excellent.
喉神经内分泌肿瘤可分为上皮源性或神经源性。后者包括副神经节瘤，而上皮源性肿瘤可分为典型类癌、非典型类癌和小细胞神经内分泌癌，小细胞神经内分泌癌又包括燕麦细胞型、中间细胞型和混合型。目前文献中报道的喉神经内分泌肿瘤病例超过500例。诊断主要基于光镜检查，在某些情况下，特殊组织化学研究可能会提供支持。应通过免疫细胞化学和/或超微结构检查来确诊。喉神经内分泌肿瘤不同的生物学行为使得准确诊断至关重要，因为治疗取决于诊断的准确性。典型类癌是一种极其罕见的病变。首选保守手术治疗；由于诊断时无淋巴结转移，无需进行选择性颈清扫术。化疗和/或放疗对目前有限数量的患者治疗效果不佳。手术治愈后预后极佳。非典型类癌是喉最常见的非鳞状癌。治疗的主要方法是手术。由于颈部淋巴结转移的可能性高，应进行选择性颈清扫术。不建议采用原发性放射治疗加辅助化疗。五年生存率为48%，十年生存率为30%。尽管喉是小细胞神经内分泌癌最常见的肺外部位之一，但它仍然是一种罕见的肿瘤。该肿瘤的手术效果令人失望，仅适用于无转移证据的局部复发病例。目前化疗和放疗似乎是致残性最小且最有效的治疗方式。两年和五年生存率分别为16%和5%。类癌肿瘤（典型和非典型）及小细胞神经内分泌癌偶尔会伴有副肿瘤综合征的报道。也有罕见报道称神经肽血清水平升高。副神经节瘤是唯一一种女性发病率较高（3:1）的喉神经内分泌肿瘤。与非典型类癌的混淆导致了错误的诊断和不恰当的分类方案，错误地认为喉副神经节瘤具有侵袭性行为的可能性。保守手术是首选的治疗方法；无需进行选择性颈清扫术，预后良好。
