Department of Pathology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Department of Respiratory Medicine, King George's Medical University, Lucknow Uttar Pradesh, India.
Gulf J Oncolog. 2021 May;1(36):76-78.
Primary enteric adenocarcinoma (PEAC) of the lung is a rare variant characterized by the presence of colorectal adenocarcinoma like components. A rare case of PEAC of lung with over-expression of mesenchymal epithelial transition factor (MET) receptor tyrosine kinase protein is being presented.
A 75-year old female presented with chest pain, cough, hemoptysis and fever for 2 months duration. Computerized tomography (CT) scan revealed a spiculated mass in left upper lobe of lung. A needle core biopsy was performed. The histopathological examination revealed a tumor composed of tall columnar cells with vesicular nuclei arranged in an acinar and glandular formation with luminal necrosis. On immunostaining, cytokeratin 7, thyroid transcription factor 1 and CDX-2 were positive. Based on the morphological and immunostaining profile a diagnosis of PEAC of the lung was rendered. Ancillary testing for driver mutations revealed over expression of c-MET while epidermal growth factor receptor (EGFR) mutations, anaplastic lymphoma kinase (ALK) rearrangement and C-ros oncogene 1, receptor tyrosine kinase (ROS 1) were negative.
PEAC of the lung which was first described by Tsao and Fraser, is an uncommon variant and rarely harbors any driver mutations. MET over expression is present in 3-7% cases of lung adenocarcinoma and is indicative of aggressive tumor behavior. Colo-rectal adenocarcinomas with MET amplification have poor overall survival. The histogenesis of PEAC may be implicated to the dysregulation of MET pathway. Targeted therapy using MET inhibitors in cases that have MET amplification has predictive implications.
This is a rare case of PEAC with c-MET over expression. The MET axis that is implicated in pathogenesis of colonic adenocarcinomas might also be the molecular pathway for the development of PEAC.
原发性肺肠型腺癌(PEAC)是一种罕见的变体,其特征是存在结直肠腺癌样成分。本文呈现了一例肺 PEAC 伴间质上皮转化因子(MET)受体酪氨酸激酶蛋白过表达的罕见病例。
一名 75 岁女性因胸痛、咳嗽、咯血和发热 2 个月就诊。计算机断层扫描(CT)显示左肺上叶有一个锯齿状肿块。进行了针芯活检。组织病理学检查显示肿瘤由高柱状细胞组成,核呈泡状,排列呈腺泡和腺体形成,伴有管腔坏死。免疫组化染色显示细胞角蛋白 7、甲状腺转录因子 1 和 CDX-2 阳性。根据形态学和免疫组化特征,诊断为肺 PEAC。辅助检测驱动基因突变显示 c-MET 过表达,而表皮生长因子受体(EGFR)突变、间变性淋巴瘤激酶(ALK)重排和 C-ros 原癌基因 1,受体酪氨酸激酶(ROS 1)均为阴性。
由 Tsao 和 Fraser 首次描述的肺 PEAC 是一种罕见的变体,很少携带任何驱动基因突变。MET 过表达见于 3-7%的肺腺癌病例,提示肿瘤具有侵袭性行为。MET 扩增的结直肠腺癌总体生存率较差。PEAC 的组织发生可能与 MET 通路的失调有关。在 MET 扩增的情况下,使用 MET 抑制剂进行靶向治疗具有预测意义。
这是一例罕见的 c-MET 过表达肺 PEAC 病例。参与结直肠腺癌发病机制的 MET 轴也可能是 PEAC 发展的分子途径。