Zeremski Vanja, Mawrin Christian, Fischer Thomas, Schalk Enrico
Department of Hematology and Oncology, Medical Center, Otto-von-Guericke University Magdeburg, D-39120 Magdeburg, Germany.
Department of Neuropathology, Medical Center, Otto-von-Guericke University Magdeburg, D-39120 Magdeburg, Germany.
Mol Clin Oncol. 2017 Jan;6(1):79-82. doi: 10.3892/mco.2016.1080. Epub 2016 Nov 11.
Yolk sac tumors (YSTs) are rare aggressive tumors, arising most commonly in the gonads and occurring mainly in young adults. We herein report a case of an extragonadal YST with hepatoid differentiation localized in the brain and lung. A 41-year old man presented to our hospital with a generalized seizure. A head computed tomography and magnetic resonance imaging examination revealed a large mass in the left occipital lobe with associated edema. Following complete resection, the histopathological examination revealed that the mass was a highly malignant epithelial tumor with a hepatoid pattern. The serum lactate dehydrogenase and α-fetoprotein levels were elevated. Additional diagnostic imaging revealed a lesion in the upper lobe of the right lung, but no other tumor manifestations. Based on the clinical and immunohistochemical characteristics, hepatocellular carcinoma and hepatoid adenocarcinoma were excluded and the diagnosis of extragonadal hepatoid YST was established. A multimodal therapeutic approach (high-dose chemotherapy with autologous stem cell transplantation, radiation and surgery) was applied; however, the patient succumbed to refractory disease 10 months after the diagnosis. Therefore, the diagnosis and treatment of hepatoid YST is an interdisciplinary challenge.
卵黄囊瘤（YSTs）是一种罕见的侵袭性肿瘤，最常见于性腺，主要发生于年轻人。我们在此报告一例发生于脑和肺的具有肝样分化的性腺外YST病例。一名41岁男性因全身性癫痫发作就诊于我院。头颅计算机断层扫描和磁共振成像检查显示左枕叶有一个大肿块并伴有水肿。完整切除后，组织病理学检查显示该肿块是一个具有肝样形态的高度恶性上皮性肿瘤。血清乳酸脱氢酶和甲胎蛋白水平升高。进一步的诊断性影像学检查显示右肺上叶有一个病灶，但无其他肿瘤表现。根据临床和免疫组化特征，排除了肝细胞癌和肝样腺癌，确立了性腺外肝样YST的诊断。采用了多模式治疗方法（大剂量化疗联合自体干细胞移植、放疗和手术）；然而，患者在诊断后10个月死于难治性疾病。因此，肝样YST的诊断和治疗是一个跨学科的挑战。
