Roxas Richard Benedict Supan, Bernardo Marie Christine Fajatin, Jacoba Araceli Pacis, Lim-Dy Janet, Alvarado Anarose Cariaga, Metovic Jasna, Annaratone Laura, Papotti Mauro
1 Quirino Memorial Medical Center, Manila, Philippines.
2 University of Turin, Torino, Italy.
Int J Surg Pathol. 2019 May;27(3):315-321. doi: 10.1177/1066896918803673. Epub 2018 Sep 27.
We describe the first case of primary thymic adenocarcinoma with signet ring cell features. The patient was a 39-year-old Filipino male who presented with a huge anterior mediastinal mass extending to the left supraclavicular fossa, which underwent an incisional biopsy. Extensive clinicoradiological work-up showed no evidence of any primary tumor in other organs, and radiological imaging confirmed a primary tumor location in the thymic area. He later developed bilateral pleural and pericardial effusions and eventually died of his tumor. The biopsy contained a neoplastic growth of solid nests made of cells with prominent signet ring features in sclerotic stroma. Immunohistochemically, the tumor cells were reactive for cytokeratin 7, carcinoembryonic antigen, and CD5 and negative for cytokeratin 20, TTF1, napsin A, α-fetoprotein, PAX-8, CD-117, CA19-9, CA-125, CDX2, p63, and CD99. No genetic alterations of ALK, RET, and ROS1 were found, nor was any ALK or ROS1 immunostaining detected, as known to occur in a fraction of primary pulmonary adenocarcinomas. Morphologically, this thymic tumor resembled signet ring cell adenocarcinomas of other locations, including, stomach, pancreas, and lung, but CD5 immunoreactivity strongly supported the clinical and radiological evidence of a primary thymic origin. In the English literature, only 58 cases of primary thymic adenocarcinoma are on record and this is the first report of a signet ring cell variant, which further broadens the morphological spectrum of thymic adenocarcinoma subtypes.
我们描述了首例具有印戒细胞特征的原发性胸腺腺癌病例。患者为一名39岁的菲律宾男性，表现为巨大的前纵隔肿块，延伸至左锁骨上窝，接受了切开活检。广泛的临床放射学检查未发现其他器官有任何原发性肿瘤的证据，放射影像学证实原发性肿瘤位于胸腺区域。他后来出现双侧胸腔和心包积液，最终死于肿瘤。活检显示肿瘤呈实性巢状生长，由硬化基质中具有明显印戒特征的细胞组成。免疫组织化学检查显示，肿瘤细胞对细胞角蛋白7、癌胚抗原和CD5呈阳性反应，对细胞角蛋白20、TTF1、 napsin A、甲胎蛋白、PAX-8、CD-117、CA19-9、CA-125、CDX2、p63和CD99呈阴性反应。未发现ALK、RET和ROS1的基因改变，也未检测到任何ALK或ROS1免疫染色，而在一部分原发性肺腺癌中已知会出现这种情况。形态学上，这种胸腺肿瘤类似于其他部位（包括胃、胰腺和肺）的印戒细胞腺癌，但CD5免疫反应性有力地支持了原发性胸腺起源的临床和放射学证据。在英文文献中，仅记录了58例原发性胸腺腺癌病例，这是印戒细胞变异型的首例报告，进一步拓宽了胸腺腺癌亚型的形态学谱。
