Ayundra Muhammad Hilmy, Laksmono Navy, Murni Tri Wahyuni
Department of Surgery, Faculty of Medicine, Padjadjaran University, Dr. Hasan Sadikin General Hospital, Bandung, Indonesia.
Division of Cardiac and Thoracic Surgery, Department of Surgery, Faculty of Medicine, Padjadjaran University, Dr. Hasan Sadikin General Hospital, Bandung, Indonesia.
Int J Surg Case Rep. 2023 Jul;108:108385. doi: 10.1016/j.ijscr.2023.108385. Epub 2023 Jun 9.
Extragonadal germ cell tumors at the mediastinum are rare and comprise of 3-4 % of all germ cell tumors. Mixed GCTs can remain asymptomatic for long periods and often present with complications. We present a case of a young male patient with a mediastinal tumor infiltrating the heart and obstructing the right ventricular outflow tract, causing cardiogenic shock.
A 16-year-old male came with chief complain of shortness of breath and underwent an echocardiogram which revealed a mass in the right atrium and right ventricle. On CT scan, a solid mass in the mediastinum, expanding and infiltrating the right atrium was found. Our patient underwent surgical treatment. Histopathology results were consistent with mixed germ cell tumor comprised of seminoma, yolk sac, and mature teratoma at the right atrial and mediastinum.
The pathogenesis of extragonadal GCTs has been linked to abnormal and/or incomplete migration of the primordial germ cells from the endoderm yolk sac to gonads. Mediastinum GCT can become clinically problematic through its growth patterns, especially its expansive profile, which can cause compression on surrounding mediastinal structures, including major vessels, which in turn diminish blood flow. Overall survival improvement is strongly linked with surgical resection of the tumor, which achieve removal of tumor tissue resistant to chemotherapy and provides sample for histological examination, which helps assessment of pathological response to chemotherapy and planning of further management.
The mediastinum is a site of different neoplasia, including germ cell tumors. Despite its low incidence, the diagnosis of a mediastinal mixed germ cell tumor should be considered in young patients with a mediastinal mass. This tumor is aggressive and often infiltrates surrounding structures and metastasis. Physicians must be aware of the difficulties and complications associated with the diagnosis.
纵隔内的性腺外生殖细胞肿瘤较为罕见，占所有生殖细胞肿瘤的3%-4%。混合性生殖细胞肿瘤（GCTs）可长期无症状，常因并发症而就诊。我们报告一例年轻男性患者，其纵隔肿瘤侵犯心脏并阻塞右心室流出道，导致心源性休克。
一名16岁男性因气短为主诉前来就诊，超声心动图显示右心房和右心室内有一肿块。CT扫描发现纵隔内有一实性肿块，肿块增大并侵犯右心房。该患者接受了手术治疗。组织病理学结果显示，右心房和纵隔的肿瘤为混合性生殖细胞肿瘤，由精原细胞瘤、卵黄囊瘤和成熟畸胎瘤组成。
性腺外GCTs的发病机制与原始生殖细胞从内胚层卵黄囊向性腺的异常和/或不完全迁移有关。纵隔GCTs因其生长方式，尤其是其膨胀性生长，可导致临床问题，可压迫周围纵隔结构，包括主要血管，进而减少血流。总体生存率的提高与肿瘤的手术切除密切相关，手术切除可去除对化疗耐药的肿瘤组织，并提供组织学检查样本，有助于评估化疗的病理反应和进一步治疗方案的制定。
纵隔是多种肿瘤的发生部位，包括生殖细胞肿瘤。尽管其发病率较低，但对于有纵隔肿块的年轻患者，应考虑诊断为纵隔混合性生殖细胞肿瘤。这种肿瘤具有侵袭性，常侵犯周围结构并发生转移。医生必须意识到诊断相关的困难和并发症。
