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食管原发性神经内分泌肿瘤——单机构14例报告及文献复习

Primary neuroendocrine neoplasm of the esophagus - Report of 14 cases from a single institute and review of the literature.

作者信息

Tustumi Francisco, Takeda Flavio Roberto, Uema Rodrigo Hideki, Pereira Guilherme Luiz Stelko, Sallum Rubens Antonio Aissar, Cecconello Ivan

机构信息

Cirurgia do Aparelho Digestivo, Faculdade de Medicina, Hospital das Clínicas, USP, SP, Brasil.

Instituto do Câncer, Faculdade de Medicina, Hospital das Clínicas, USP, SP, Brasil.

出版信息

Arq Gastroenterol. 2017 Jan-Mar;54(1):4-10. doi: 10.1590/S0004-2803.2017v54n1-01.

DOI:10.1590/S0004-2803.2017v54n1-01
PMID:28079231
Abstract

BACKGROUND

: Most prevalent esophageal neoplasm is squamous cell carcinoma and adenocarcinoma. Other tumors are uncommon and poorly studied. Primary neuroendocrine esophageal neoplasm is a rare carcinoma and most of its therapy management is based on lung neuroendocrine studies. Neuroendocrine tumors can be clustered in the following subtypes: high grade (small cell carcinoma or large cell carcinoma) and low grade (carcinoids).

OBJECTIVE

: The present study aims to assess clinical and pathological neuroendocrine esophageal tumors in a single oncologic center.

METHODS

: A retrospective analysis of patients and review of the literatures was performed.

RESULTS

: Fourteen patients were identified as neuroendocrine tumors, 11 male and 3 female patients. Mean age was 67.3 years old. Ten patients were classified as small cell, 3 as large cell and 1 as carcinoid. Four patients presented squamous cell carcinoma simultaneously and 1 also presented adenocarcinoma. Main sites of metastasis were liver, peritoneum, lung and bones. Most patients died before 2 years of follow-up. Patient with longer survival died at 35 months after diagnosis.

CONCLUSION

: Neuroendocrine esophageal tumors are rare; affect mainly men in their sixties or seventies. High grade tumors can be mixed to other subtypes neoplasms, such as adenocarcinoma and squamous cell carcinoma. Most of these patients have poor overall survival rates.

摘要

背景

最常见的食管肿瘤是鳞状细胞癌和腺癌。其他肿瘤并不常见且研究较少。原发性食管神经内分泌肿瘤是一种罕见的癌症,其大多数治疗管理基于肺部神经内分泌研究。神经内分泌肿瘤可分为以下亚型:高级别(小细胞癌或大细胞癌)和低级别(类癌)。

目的

本研究旨在评估单一肿瘤中心的临床和病理神经内分泌食管肿瘤。

方法

对患者进行回顾性分析并查阅文献。

结果

14例患者被确诊为神经内分泌肿瘤,其中男性11例,女性3例。平均年龄为67.3岁。10例患者被分类为小细胞癌,3例为大细胞癌,1例为类癌。4例患者同时患有鳞状细胞癌,1例还患有腺癌。主要转移部位为肝脏、腹膜、肺和骨骼。大多数患者在随访2年之前死亡。存活时间较长的患者在诊断后35个月死亡。

结论

食管神经内分泌肿瘤罕见;主要影响六七十岁的男性。高级别肿瘤可与其他亚型肿瘤混合,如腺癌和鳞状细胞癌。这些患者大多数总体生存率较差。

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