Kubouchi Yasuaki, Matsuoka Yuki, Araki Kunio, Kidokoro Yoshiteru, Haruki Tomohiro, Nakamura Hiroshige, Umekita Yoshihisa
Division of Organ Pathology, Department of Pathology, Faculty of Medicine, Tottori University, Tottori, Japan.
Division of General Thoracic Surgery, Department of Surgery, Tottori University, 86, Nishicho, Yonago, Tottori, 683-8503, Japan.
Surg Case Rep. 2018 Jul 4;4(1):70. doi: 10.1186/s40792-018-0478-4.
Pure high-grade fetal adenocarcinoma of the lung (H-FLAC) is a very rare tumor.
An annual check-up revealed an abnormal shadow in the left middle lung field of a 63-year-old Japanese man. Chest computed tomography (CT) showed a 3.6 × 2.8 cm pulmonary lesion with clear boundary in the left upper lobe. A transbronchial lung biopsy revealed non-small cell carcinoma. A left upper lobectomy and mediastinal lymph node dissection were performed. Histologically, the tumor consisted of solid proliferation of atypical cell with clear cytoplasm. Another histological component and morulae were not contained. Immunohistochemically, the tumor was focally positive for alpha-fetoprotein (AFP) and beta-catenin in the cell membrane. Accordingly, we made the diagnosis of pure H-FLAC, pT2aN2M0, stage IIIA. Two courses of adjuvant chemotherapy (cisplatin and vinorelbine) were administered but then the treatment was discontinued due to the patient's adverse reaction. At 25 months after the surgery, the patient had relapsed.
We report a very rare case of pure H-FLAC. This histology has been considered to predict an extremely poor prognosis; therefore, the elucidation of genetic abnormalities and effective treatment is awaited.
纯高分化胎儿型肺腺癌(H-FLAC)是一种非常罕见的肿瘤。
一名63岁日本男性的年度体检显示左肺中叶有异常阴影。胸部计算机断层扫描(CT)显示左上叶有一个3.6×2.8厘米的肺部病变,边界清晰。经支气管肺活检显示为非小细胞癌。进行了左上叶切除术和纵隔淋巴结清扫术。组织学上,肿瘤由具有透明细胞质的非典型细胞实性增生组成。未发现其他组织学成分和桑椹体。免疫组织化学检查显示,肿瘤在细胞膜上α-甲胎蛋白(AFP)和β-连环蛋白呈局灶性阳性。因此,我们诊断为纯H-FLAC,pT2aN2M0,ⅢA期。给予了两个疗程的辅助化疗(顺铂和长春瑞滨),但由于患者的不良反应,治疗中断。术后25个月,患者复发。
我们报告了一例非常罕见的纯H-FLAC病例。这种组织学类型被认为预后极差;因此,期待对基因异常和有效治疗方法的阐明。