喉部小细胞神经内分泌癌患者长期无病生存1例报告及文献复习

目的：分析长期无病生存的喉部小细胞神经内分泌癌（LSCNC）患者的临床特点，为LSCNC的诊治提供依据。方法：收集1例原发性LSCNC患者的临床资料，结合相关文献，分析LSCNC和肺外小细胞癌（EPSCC）的临床特点、诊断及治疗方法。结果：患者为55岁男性，既往有长期大量吸烟及饮酒史，以颈部包块起病，行喉镜检查见会厌喉面左侧不平肿物；颈部肿物穿刺活检提示淋巴结转移癌。患者随后行喉切除术和双侧颈部淋巴结清扫术。术后病理诊断为LSCNC；免疫组织化学法检查，CD56（NK-1）（+），CK-pan （+），CgA （-），Syn （-），Ki-67（+90%）。患者术后行瘤床区及淋巴结引流区放疗加6个疗程依托泊苷和顺铂（EP）方案化疗，未行脑预防照射（PCI）。规律复查喉镜、头颅核磁等检查，未见复发和转移，目前仍存活，无病生存期已达38个月。结论：LSCNC恶性程度高，确诊主要依靠病理组织形态学和免疫组织化学检查。目前LSCNC多采用手术、化疗和放疗联合的综合治疗，不推荐应用PCI，综合治疗可以改善预后。

Long-term disease-free survival in patient with laryngeal small cell neuroendocrine carcinoma:A case report and literature review

Objective: To analyze the clinical characteristics of patient with laryngeal small cell neuroendocrine carcinoma(LSCNC) with long-term disease-free survival,and to provide the basis for the diagnosis and treatment of LSCNC.Methods: The clinical materials of a patient with primary LSCNC were collected,and the clinical features,diagnosis and treatment of LSCNC and extrapulmonary small cell cancer (EPSCC) combined with the relative literatures were analyzed.Results: The patient was a 55-year-old man who had a long history of heavy smoking and drinking, and presented with neck mass.The laryngoscope revealed the uneven mass on the left side of epiglottic laryngeal surface.The subsequent biopsy of the neck mass indicated lymph node metastasis.The patient underwent laryngectomy and bilateral cervical lymph node dissection.The postoperative pathology indicated LSCNC; the immunohistochemistry results showed CD56(NK-1)(+), CK-pan(+), CgA(-), Syn(-)and Ki-67(+90%).The patient underwent radiotherapy to the tumor bed and lymph node drainage area and 6 courses of chemotherapy with EP regimen.No prophylactic cranial irradiation (PCI) was conducted.Regular follow-up with laryngoscope, crainial MRI and other examinations showed no recurrence and metastasis.The patient still alive with disease-free survival of 38 months now.Conclusion: LSCNC is highly malignant.The diagnosis mainly depends on the pathohistomorphology and immunohistochemistry examination.The combination of surgery, chemotherapy and radiotherapy is commonly used for LSCNC at present.PCI is not recommended routinely.Multimodality treatment can improve the prognosis.