Soriano Paolo K, Iqbal Muhammad F, Siddiqui Omar M, Wang Jeff F, Desai Meghna R
Department of Internal Medicine, Southern Illinois University, Springfield, IL, USA.
Pathology Associates of Central Illinois, Memorial Medical Center, Springfield, IL, USA.
Am J Case Rep. 2017 Aug 18;18:902-907. doi: 10.12659/ajcr.904855.
BACKGROUND Primary mediastinal non-seminomatous germ cell tumors (NSGCTs) are aggressive and carry a poor five-year disease free survival rate even with aggressive treatment. We describe a young adult male with primary mediastinal NSGCT presenting with airway obstruction and superior vena cava syndrome (SVCS). CASE REPORT The patient presented with four weeks of nonproductive cough, weight loss, and right-sided pleuritic chest pain. Chest computed topography (CT) imaging demonstrated a right-sided mediastinal mass determined as a yolk sac tumor on biopsy. The patient underwent induction chemotherapy with etoposide and cisplatin for stage III NSGCT. In the interim, he developed SVCS warranting a second cycle of chemotherapy along with intravenous steroids, with notable improvement in symptoms. However, serial alpha-fetoprotein (AFP) measurements showed progressively increasing levels up to a maximum of 18,781 ng/mL indicating treatment failure. He is currently on salvage chemotherapy. CONCLUSIONS Obstruction of the SVC by external compression is often a manifestation of a malignant process in the thorax. SVCS is a medical emergency and occurs in 6% of patients with mediastinal GCTs. Historically, irradiation was initiated without a histologic diagnosis to relieve the life-threatening obstruction. However, newer data suggest that it is acceptable to defer therapy until a full diagnostic workup is completed. This case highlights the malignant nature of primary mediastinal NSGCTs. In addition, inasmuch as SVCS is dramatic in presentation, it is important to recognize that symptomatic obstruction often develops over weeks or longer. In a hemodynamically stable patient, an accurate histologic diagnosis prior to starting treatment is essential in guiding therapy.
背景 原发性纵隔非精原细胞性生殖细胞肿瘤（NSGCTs）具有侵袭性，即使接受积极治疗，其五年无病生存率仍较低。我们描述了一名患有原发性纵隔NSGCT并出现气道梗阻和上腔静脉综合征（SVCS）的年轻成年男性。病例报告 患者出现了四周的干咳、体重减轻和右侧胸膜炎性胸痛。胸部计算机断层扫描（CT）成像显示右侧纵隔肿块，活检确定为卵黄囊瘤。患者接受了依托泊苷和顺铂的诱导化疗，用于治疗III期NSGCT。在此期间，他出现了SVCS，需要进行第二个周期的化疗以及静脉注射类固醇，症状有明显改善。然而，连续的甲胎蛋白（AFP）测量显示水平逐渐升高，最高达到18,781 ng/mL，表明治疗失败。他目前正在接受挽救性化疗。结论 上腔静脉受外部压迫导致的梗阻通常是胸部恶性病变的表现。SVCS是一种医疗急症，在纵隔生殖细胞肿瘤患者中发生率为6%。从历史上看，在没有组织学诊断的情况下就开始进行放疗以缓解危及生命的梗阻。然而，新的数据表明，在完成全面的诊断检查之前推迟治疗是可以接受的。本病例突出了原发性纵隔NSGCTs的恶性本质。此外，鉴于SVCS的表现较为显著，重要的是要认识到症状性梗阻通常会在数周或更长时间内逐渐发展。对于血流动力学稳定的患者，在开始治疗前进行准确的组织学诊断对于指导治疗至关重要。
