Department of Comprehensive Cancer Therapy, Shinshu University School of Medicine, Matsumoto, Japan.
First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
Thorac Cancer. 2021 Mar;12(6):807-813. doi: 10.1111/1759-7714.13859. Epub 2021 Jan 27.
This study was performed to clarify the treatment outcome of patients with primary mediastinal germ cell tumors (PMGCTs), focusing on the clinical manifestations and management during definitive therapy and long-term follow-up.
In this study, we retrospectively reviewed the medical records of patients with PMGCTs treated at Shinshu University School of Medicine, and examined the clinical profiles and treatment outcomes of 22 patients (mean age of 29 years) with primary mediastinal GCTs treated at our hospital between 1983 and 2019.
Five patients were diagnosed with pure seminoma and 17 had nonseminomatous GCT. A total of 21 patients were treated with cisplatin-based chemotherapy and 15 patients (68.2%) underwent thoracic surgery after chemotherapy. Although all cases of nonseminomatous GCT were negative for tumor markers after cisplatin-based chemotherapy, two cases showed variable GCT cells and two had somatic components (angiosarcoma and rhabdomyosarcoma) in resected specimens. Three relapsed soon after surgery. Growing teratoma syndrome developed during chemotherapy in four cases. Urgent thoracic surgery was performed in three patients, but one case was inoperable. The calculated 10-year overall survival rates were 100% in mediastinal seminoma and 64.7% in NSGCT. During follow-up, second non-GCT malignancies developed in three patients (colon cancer, 190 months; thyroid cancer, 260 months; non-small cell lung cancer, 250 months after the initial chemotherapy) and one patient with primary mediastinal seminoma was associated with multiple type I endocrine tumors.
Our experiences demonstrated that long-term survival and/or cure can be achieved with adequate chemotherapy followed by local surgical treatment even in patients with mediastinal GCTs. However, the clinical manifestations and biological behaviors during and/or after chemotherapy were complex and varied. In addition, the development of secondary malignancies should be taken into consideration for long-term follow-up. Clinicians should be aware of the various clinical features and secondary malignancies in primary mediastinal GCTs.
本研究旨在阐明原发性纵隔生殖细胞瘤（PMGCT）患者的治疗结果，重点关注明确治疗和长期随访期间的临床表现和管理。
本研究回顾性分析了在信州大学医学研究生院接受治疗的 PMGCT 患者的病历，并检查了我院在 1983 年至 2019 年期间治疗的 22 例原发性纵隔 GCT 患者的临床特征和治疗结果（平均年龄 29 岁）。
5 例患者被诊断为单纯精原细胞瘤，17 例患者为非精原细胞瘤生殖细胞瘤。21 例患者接受顺铂为基础的化疗，15 例患者（68.2%）在化疗后接受了胸部手术。尽管所有非精原细胞瘤生殖细胞瘤患者在顺铂为基础的化疗后肿瘤标志物均为阴性，但 2 例患者的切除标本中显示出不同的 GCT 细胞，2 例患者有体细胞成分（血管肉瘤和横纹肌肉瘤）。3 例患者在手术后很快复发。4 例患者在化疗期间发生了生殖细胞瘤综合征。3 例患者紧急行开胸手术，但 1 例患者无法手术。纵隔精原细胞瘤的 10 年总生存率为 100%，非精原细胞瘤生殖细胞瘤为 64.7%。在随访期间，3 例患者发生了第二非 GCT 恶性肿瘤（结肠癌，190 个月；甲状腺癌，260 个月；初始化疗后 250 个月发生非小细胞肺癌），1 例原发性纵隔精原细胞瘤患者合并多种 I 型内分泌肿瘤。
我们的经验表明，即使是纵隔 GCT 患者，通过充分的化疗后进行局部手术治疗，也可以实现长期生存和/或治愈。然而，化疗期间和/或之后的临床表现和生物学行为复杂多样。此外，应考虑长期随访中继发性恶性肿瘤的发生。临床医生应注意原发性纵隔 GCT 的各种临床表现和继发性恶性肿瘤。
