12-718 C.S. Mott Children's Hospital, University of Michigan, 1540 E. Hospital Drive, Ann Arbor, MI, 48109, USA,
J Neurooncol. 2014 May;118(1):93-100. doi: 10.1007/s11060-013-1306-0. Epub 2014 Apr 4.
In order to improve outcomes for CNS mixed malignant germ cell tumors (MMGCT) we sought to increase complete responses (CR) to initial therapy, through intensifying neoadjuvant chemotherapy (CHT1) with added ifosfamide, encouraging second-look surgery, and administering dose-intensive, stem cell-supported chemotherapy (CHT2) to patients with residual tumor, all prior to radiation therapy (RT). Diagnosis was confirmed by biopsy or elevated germ cell tumor markers. After tumor staging was completed, patients received four cycles of chemotherapy (cisplatin, etoposide and ifosfamide, "CHT1"). In patients with <CR, second-look surgery was encouraged. Patients with residual tumor received two cycles of high dose, sub-ablative chemotherapy with carboplatin and cyclophosphamide ("CHT2") with peripheral stem cell support. All patients then received RT: for localized tumors with CR before RT, 36 gray (Gy) whole ventricular radiation therapy (WVRT) plus 50.4 Gy boost to primary; for disseminated tumors or < CR before RT, craniospinal irradiation (CSI) plus boosts to primary site(s) and bulky metastases. 26 patients (19 M0, 7 M+) were enrolled. The diagnosis was established by histology (20) or elevated markers (6). Objective responses to CHT1 were complete in 12/22 patients with evaluable disease and partial in 10; 8 additional tumors were rendered CR prior to RT (5 surgical CRs: 3 initial, 2 second-look; 3 CRs to CHT2). Thus, 20/26 patients (77%) were free of disease (CR) prior to RT. Six-year relapse-free survival was 63 ± 10%; overall survival was 68 ± 9%. Of 16 M0 patients who received only WVRT, four relapsed in the spine, outside the radiation field. The relatively high frequency (25%) of relapse outside the initial RT volume highlights the limitations of initial staging criteria and the curative potential of conventional and high dose chemotherapy. CSI remains the standard of care for CNS MMGCT, even for patients with localized disease.
为了提高中枢神经系统混合恶性生殖细胞瘤（MMGCT）的治疗效果，我们试图通过增加新辅助化疗（CHT1）中的异环磷酰胺、鼓励二次探查手术以及对残留肿瘤患者进行剂量密集、干细胞支持的化疗（CHT2）来提高初始治疗的完全缓解率（CR），所有这些治疗均在放射治疗（RT）之前进行。诊断通过活检或升高的生殖细胞瘤标志物确认。在完成肿瘤分期后，患者接受四个周期的化疗（顺铂、依托泊苷和异环磷酰胺，“CHT1”）。对于<CR 的患者，鼓励进行二次探查手术。对于残留肿瘤的患者，接受两个周期的高剂量、亚致死性化疗，使用卡铂和环磷酰胺（“CHT2”）并进行外周干细胞支持。所有患者随后接受 RT：对于有 CR 的局限性肿瘤，在 RT 前进行 36 格雷（Gy）全脑室放射治疗（WVRT），对原发灶进行 50.4Gy 加量；对于播散性肿瘤或 RT 前<CR 的患者，进行颅脊髓照射（CSI），并对原发灶和大体积转移灶进行加量。共纳入 26 例患者（19 例 M0，7 例 M+）。诊断通过组织学（20 例）或升高的标志物（6 例）确定。有可评估疾病的 22 例患者中，CHT1 的客观缓解完全缓解 12 例，部分缓解 10 例；8 例肿瘤在 RT 前达到 CR（5 例手术 CR：3 例初始，2 例二次探查；3 例 CHT2 后达到 CR）。因此，26 例患者中有 20 例（77%）在 RT 前无疾病（CR）。6 年无复发生存率为 63±10%；总生存率为 68±9%。仅接受 WVRT 的 16 例 M0 患者中有 4 例在脊柱中复发，位于放射野外。初始 RT 体积外的复发频率相对较高（25%），突出了初始分期标准的局限性以及常规和高剂量化疗的治愈潜力。CSI 仍然是 CNS MMGCT 的标准治疗方法，即使对于局限性疾病的患者也是如此。
Zotero 插件