Breen William G, Blanchard Miran J, Rao Amulya Nageswara, Daniels David J, Buckner Jan C, Laack Nadia N Issa
Mayo Medical School, Mayo Clinic, Rochester, Minnesota.
Roger Maris Cancer Center, Sanford Health, Fargo, North Dakota.
Pediatr Blood Cancer. 2017 Nov;64(11). doi: 10.1002/pbc.26637. Epub 2017 Jul 11.
To evaluate patterns of failure after multimodality treatment of nongerminomatous germ cell tumors (NGGCTs).
We retrospectively reviewed records of 34 patients diagnosed with primary intracranial NGGCT between 1988 and 2014.
Thirty-four patients received induction chemotherapy followed by radiation with or without surgery. Median follow-up was 11.1 years (0.8-23.3). Outcomes were significantly improved in these 34 patients (5-year overall survival [OS]: 88% versus 50%, P = 0.0092), so analysis is restricted to that subset. Disease-free survival (DFS) was 67, 60, and 54% at 5, 10, and 15 years, respectively. Elevated cerebrospinal fluid-α-fetoprotein (CSF-AFP) at diagnosis was associated with poorer DFS (37 vs. 89% at 10 years; P = 0.01). There was no statistically significant difference in OS, or DFS, or patterns of failure for limited radiotherapy volumes versus larger volumes; however, patients receiving initial local radiotherapy had 32% distant central nervous system (CNS) recurrence at 10 years compared to 0% for those receiving initial larger field irradiation (P = 0.09). Fifteen patients recurred. All four patients who relapsed in the spine had received local radiotherapy and had elevated serum and CSF-AFP at baseline. All three patients with ventricular relapse received local radiation therapy.
NGGCT patients continue to relapse beyond 5 years. Late ventricular relapse occurred even in patients without clear evidence of germinoma component. Elevated CSF-AFP at diagnosis is associated with poor DFS and risk for distant CNS relapse. Patients with residual radiographic disease after chemotherapy or residual malignant histologies after second-look surgery have inferior outcomes. Our data support consideration of treatment intensification for these patients.
评估非生殖细胞瘤性生殖细胞肿瘤(NGGCTs)多模式治疗后的失败模式。
我们回顾性分析了1988年至2014年间34例诊断为原发性颅内NGGCT的患者记录。
34例患者接受了诱导化疗,随后接受放疗,部分患者还接受了手术。中位随访时间为11.1年(0.8 - 23.3年)。这34例患者的预后有显著改善(5年总生存率[OS]:88%对50%,P = 0.0092),因此分析仅限于该亚组。无病生存率(DFS)在5年、10年和15年分别为67%、60%和54%。诊断时脑脊液α-甲胎蛋白(CSF-AFP)升高与较差的DFS相关(10年时为37%对89%;P = 0.01)。对于有限放疗体积与较大体积放疗,OS、DFS或失败模式无统计学显著差异;然而,接受初始局部放疗的患者在10年时有32%发生远处中枢神经系统(CNS)复发,而接受初始较大野照射的患者为0%(P = 0.09)。15例患者复发。所有4例脊柱复发的患者均接受了局部放疗,且基线时血清和CSF-AFP升高。所有3例脑室复发的患者均接受了局部放疗。
NGGCT患者在5年后仍会复发。即使在没有明确生殖细胞瘤成分证据的患者中也会发生晚期脑室复发。诊断时CSF-AFP升高与较差的DFS及远处CNS复发风险相关。化疗后有残留影像学疾病或二次探查手术后有残留恶性组织学的患者预后较差。我们的数据支持对这些患者考虑强化治疗。