Huang Qiujuan, Liu Changxu, Tang Ruijun, Li Jie, Li Wei, Zhu Lei, Guo Yuhong, Zhang Lin, Qu Tongyuan, Yang Lingyi, Li Lingmei, Wang Yalei, Sun Baocun, Meng Bin, Yan Jie, Xu Wengui, Zhang Huilai, Cao Dengfeng, Cao Wenfeng
Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin; Tianjin's Clinical Research Center for Cancer, Tianjin Medical University, Ministry of Education, Tianjin 300060, China.
Department of Pathology, Guilin TCM Hospital of China, Guilin 541002, China.
Cancer Biol Med. 2018 Nov;15(4):461-467. doi: 10.20892/j.issn.2095-3941.2018.0054.
Extragonadal primary yolk sac tumor of the intestinal tract origin is exceedingly rare. Through a multiple disciplinary team, the diagnosis and treatment of primary intestinal yolk sac tumor were further defined. We report 2 such cases with detailed histologic and immunohistochemical analysis. The two patients were a 7-year-old girl and a 29-year-old woman. Both of them preoperatively had an elevated serum alpha fetoprotein (AFP) level (≥ 1,210 ng/mL). The tumors are located in the intestine and imaging examination indicated the rectum as the primary site. Grossly the mass was grey-white and crisp texture. Microscopic examination featured reticular, microcystic, macrocystic, papillary, solid, and some glandular patterns. Immunohistochemically, tumor cells of both cases were positive for SALL4, AFP, pan-cytokeratin (AE1/AE3), and glypican-3. Simultaneously, a stain for EMA, OCT4, CD30, HCG, vimentin and CK20 were negative in all 2 neoplasms. The features of morphology, immunohistochemistry, laboratory examinations and imaging studies consist of the diagnosis of primary yolk sac tumor of the intestine.
肠道起源的性腺外原发性卵黄囊瘤极为罕见。通过多学科团队，对原发性肠道卵黄囊瘤的诊断和治疗有了进一步明确。我们报告2例此类病例，并进行详细的组织学和免疫组化分析。两名患者分别为一名7岁女孩和一名29岁女性。她们术前血清甲胎蛋白（AFP）水平均升高（≥1210 ng/mL）。肿瘤位于肠道，影像学检查提示直肠为原发部位。大体上，肿块呈灰白色，质地脆。显微镜检查可见网状、微囊状、大囊状、乳头状、实性及一些腺管样结构。免疫组化方面，两例肿瘤细胞SALL4、AFP、全细胞角蛋白（AE1/AE3）和磷脂酰肌醇蛋白聚糖-3均呈阳性。同时，两例肿瘤中上皮膜抗原（EMA）、八聚体结合转录因子4（OCT4）、CD30、人绒毛膜促性腺激素（HCG）、波形蛋白和细胞角蛋白20（CK20）染色均为阴性。形态学、免疫组化、实验室检查及影像学研究结果共同构成了肠道原发性卵黄囊瘤的诊断依据。
