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一例罕见的原发性肺癌病例报告,该肺癌包含腺癌和非典型类癌肿瘤,其中类癌成分存在重排。

A rare case report of a primary lung cancer comprising adenocarcinoma and atypical carcinoid tumor, with the carcinoid component harboring rearrangement.

作者信息

Hu Wenbin, Zhao Jiaming, Wang Guoxia, Wang Qihao, Deng Mingming, Shen Jie, Hofman Paul, Urbanska Edyta Maria, Santoni-Rugiu Eric, Christopoulos Petros, Ramirez Robert A, Hida Toyoaki, Lu Xiaoqing, He Binjun

机构信息

Department of Thoracic Surgery, Affiliated Hospital of Shaoxing University (The Shaoxing Municipal Hospital), Shaoxing, China.

Department of Anesthesia Operation, Affiliated Hospital of Shaoxing University (The Shaoxing Municipal Hospital), Shaoxing, China.

出版信息

Transl Lung Cancer Res. 2024 May 31;13(5):1150-1162. doi: 10.21037/tlcr-24-352. Epub 2024 May 24.

Abstract

BACKGROUND

The occurrence of pulmonary adenocarcinoma coexisting with atypical carcinoid tumors is a rare phenomenon. The presence of fusion in an atypical carcinoid component of a histologically mixed tumor is even more uncommon. Due to their infrequency, the origin and pathogenesis of these mixed tumors remain largely unknown. The advances of therapy development in such patients are still limited and there is no standard treatment. We present a case of collision tumor in the lung consisting of atypical carcinoid and adenocarcinoma to better understand the clinical characteristics of this disease.

CASE DESCRIPTION

We report an extremely rare case of rearrangement in a pulmonary atypical carcinoid tumor that coexisting with adenocarcinoma. A 58-year-old woman, who was asymptomatic, underwent pulmonary lobectomy due to the detection of a gradually enlarging solitary pulmonary nodule in the right upper lung. Histological examination of the resected tumor revealed the presence of both atypical carcinoid (approximately 80%) and adenocarcinoma (approximately 20%) components. Metastases by the carcinoid component were observed in mediastinal lymph nodes (station 2R and 4R) and in the primary tumor. Anaplastic lymphoma kinase () rearrangement was detected in both the primary and metastatic lesions of the carcinoid tumor. Four cycles of chemotherapy with etoposide and carboplatin were dispensed after surgery.

CONCLUSIONS

This is the first reported case of coexisting pulmonary adenocarcinoma and atypical carcinoid tumor with an fusion only detected in the carcinoid component. The presence of rearrangement in pulmonary carcinoid tumor is very uncommon, and there is currently no standard treatment for advanced stages. Therefore, comprehensive molecular testing, including rearrangement analysis, should be recommended for mixed tumors exhibiting features of atypical carcinoid. inhibitors could represent a potential treatment strategy for selected patients.

摘要

背景

肺腺癌与非典型类癌肿瘤共存是一种罕见现象。在组织学上为混合性肿瘤的非典型类癌成分中出现融合更为罕见。由于其罕见性,这些混合性肿瘤的起源和发病机制在很大程度上仍不清楚。此类患者治疗进展仍然有限,且尚无标准治疗方法。我们报告一例肺部碰撞瘤,由非典型类癌和腺癌组成,以更好地了解该疾病的临床特征。

病例描述

我们报告一例极其罕见的肺非典型类癌肿瘤中存在重排且与腺癌共存的病例。一名58岁无症状女性因右上肺发现逐渐增大的孤立性肺结节而接受肺叶切除术。切除肿瘤的组织学检查显示存在非典型类癌(约80%)和腺癌(约20%)成分。在纵隔淋巴结(2R区和4R区)和原发肿瘤中观察到类癌成分发生转移。在类癌肿瘤的原发灶和转移灶中均检测到间变性淋巴瘤激酶()重排。术后给予依托泊苷和卡铂进行四个周期的化疗。

结论

这是首例报道的肺腺癌与非典型类癌肿瘤共存且仅在类癌成分中检测到融合的病例。肺类癌肿瘤中存在重排非常罕见,目前晚期尚无标准治疗方法。因此,对于表现出非典型类癌特征的混合性肿瘤,应推荐进行包括重排分析在内的全面分子检测。抑制剂可能是部分患者的潜在治疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b70/11157374/68374f81ca82/tlcr-13-05-1150-f1.jpg

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