Hardenbergh P H, Golden J, Billet A, Scott R M, Shrieve D C, Silver B, Loeffler J S, Tarbell N J
Joint Center for Radiation Therapy, and Dana Farber Cancer Institute, Boston, MA, USA.
Int J Radiat Oncol Biol Phys. 1997 Sep 1;39(2):419-26. doi: 10.1016/s0360-3016(97)00330-1.
To determine the optimal dose and treatment outcome of patients treated with radiation for intracranial germinoma.
Between 1975 and 1995, 40 patients with the diagnosis of intracranial germinoma were treated with radiation (RT) to the central nervous system. All patients received whole-brain (WB) RT (median dose: 32.4 Gy, range: 15-44.37 Gy) and a boost to the tumor volume (median total tumor volume dose: 52 Gy, range: 45-59.5 Gy). Thirty patients received RT to the spine (median dose: 26 Gy, range: 18.75-37.5 Gy). Four patients were treated with cisplatin-based chemotherapy and WB RT with a boost to the tumor volume (dose range: 51-54 Gy). A low-dose RT only group was defined as < or = 25.5 Gy to the WB (9 patients); < 50 Gy to the primary site (14 patients); and < 22 Gy to the spine (9 patients). Seventeen tumors were biopsy-proven germinoma, and 17 patients presented with multiple midline germinomas (MMG). Among 26 patients who had tumor markers measured, 27% had elevation of beta-human chorionic gonadotropin and by definition, no patient had an elevation of AFP. Twenty-four percent of 26 patients who had spine imaging or cerebral spinal fluid cytology had evidence of tumor seeding at diagnosis. The male to female ratio was 1.9:1. Median age at diagnosis was 14 years for male patients and 9.5 years for female patients (p = 0.02), (overall age ranges: 0.5-31 years). Median follow-up was 62 months (range: 3-226 months). Late effects of 29 patients with follow-up of > or = 20 months and adequate documentation in their medical records were analyzed.
The 5-year actuarial rate of disease-free survival (DFS) and overall survival (OS) for biopsy-proven germinomas and presumed germinomas was 97%. No patient died of germinoma. There were no local failures regardless of the dose of RT, elevation of HCG tumor marker, or CSF dissemination at presentation. At presentation 22 patients had evidence of at least one endocrine abnormality. At follow-up there were no new patients diagnosed with an endocrine abnormality; however, 13 out of 22 patients had an increase in the number of endocrine deficiencies requiring hormone replacement. At presentation, 14 patients showed evidence of growth retardation. At follow-up there were no new cases of growth failure in the remaining patients.
Germinomas are highly curable with RT alone. Lower doses of RT to the craniospinal axis without chemotherapy appear to produce equally effective DFS and OS as do higher doses of RT or combination chemotherapy and RT. Craniospinal RT may be indicated for patients with MMG or patients with evidence of spinal seeding. Long-term effects of growth retardation, and other endocrine deficiencies appear to be correlated with disease at presentation rather than solely with treatment.
确定接受颅内生殖细胞瘤放射治疗患者的最佳剂量和治疗结果。
1975年至1995年间，40例诊断为颅内生殖细胞瘤的患者接受了中枢神经系统放射治疗（RT）。所有患者均接受全脑（WB）放疗（中位剂量：32.4 Gy，范围：15 - 44.37 Gy），并对肿瘤体积进行加量照射（中位总肿瘤体积剂量：52 Gy，范围：45 - 59.5 Gy）。30例患者接受了脊柱放疗（中位剂量：26 Gy，范围：18.75 - 37.5 Gy）。4例患者接受了以顺铂为基础的化疗及全脑放疗，并对肿瘤体积进行加量照射（剂量范围：51 - 54 Gy）。低剂量放疗组定义为全脑放疗剂量≤25.5 Gy（9例患者）；原发部位放疗剂量<50 Gy（14例患者）；脊柱放疗剂量<22 Gy（9例患者）。17例肿瘤经活检证实为生殖细胞瘤，17例患者表现为多发中线生殖细胞瘤（MMG）。在26例检测了肿瘤标志物的患者中，27%的患者β-人绒毛膜促性腺激素升高，按照定义，无患者甲胎蛋白升高。26例进行了脊柱成像或脑脊液细胞学检查的患者中，24%在诊断时有肿瘤播散的证据。男女比例为1.9:1。男性患者诊断时的中位年龄为14岁，女性患者为9.5岁（p = 0.02），（总体年龄范围：0.5 - 31岁）。中位随访时间为62个月（范围：3 - 226个月）。对29例随访时间≥20个月且病历中有充分记录的患者的晚期效应进行了分析。
经活检证实的生殖细胞瘤和疑似生殖细胞瘤的5年无病生存率（DFS）和总生存率（OS）为97%。无患者死于生殖细胞瘤。无论放疗剂量、HCG肿瘤标志物升高情况或诊断时脑脊液播散情况如何，均无局部复发。诊断时22例患者有至少一种内分泌异常的证据。随访时无新诊断为内分泌异常的患者；然而，22例患者中有13例内分泌缺乏症数量增加，需要激素替代治疗。诊断时，14例患者有生长发育迟缓的证据。随访时其余患者无新的生长发育失败病例。
单纯放疗可使生殖细胞瘤获得高度治愈。在不进行化疗的情况下，较低剂量的颅脊柱轴放疗似乎能产生与较高剂量放疗或联合化疗及放疗同样有效的DFS和OS。对于MMG患者或有脊柱播散证据的患者，可能需要进行颅脊柱放疗。生长发育迟缓及其他内分泌缺乏症的长期影响似乎与诊断时的疾病相关，而非仅与治疗相关。
