Wolff J E A, Westphal S, Mölenkamp G, Gnekow A, Warmuth-Metz M, Rating D, Kuehl J
St. Hedwigs Klinik, Hämato/Onkologie, Steinmetzstr. 1-3, 93049 Regensburg, Germany.
Br J Cancer. 2002 Oct 21;87(9):945-9. doi: 10.1038/sj.bjc.6600552.
To evaluate the overall survival of paediatric patients with pontine gliomas treated with oral trophosphamide and etoposide. Patients between 3 and 17 years of age with either typical diffuse pontine glioma on MRI or histologically proven anaplastic astrocytoma/glioblastoma multiforme located in the pons, were eligible. Treatment consisted of oral trophosphamide 100 mg x m(-2) x day(-1) combined with oral etoposide at 25 mg x m(-2) x day(-1) starting simultaneously with conventional radiation. Twenty patients were enrolled (median age 6 years, male : female=9 : 11). Surgical procedures included: no surgery: five, open biopsy: three, stereotactic biopsy: six, partial resection: three, and sub-total resection: three. Histological diagnoses included pilocytic astrocytoma: one, astrocytoma with no other specification: three, anaplastic astrocytoma: three, glioblastoma multiforme: eight, no histology: five. The most frequent side effects were haematologic and gastrointestinal. There was no toxic death. The response to combined treatment in 12 evaluable patients was: complete response: 0, partial response: three, stable disease: four, and progressive disease: five. All tumours progressed locally and all patients died. The overall median survival was 8 months. The overall survival rates at 1 and 4 years were: 0.4 and 0.05 respectively. This was not different from a control group of patients documented in the same population. Oral trophosphamide in combination with etoposide did not improve survival of pontine glioma patients. The treatment was well tolerated and should be evaluated for more chemoresponsive paediatric malignancies.
评估口服环磷酰胺和依托泊苷治疗小儿脑桥胶质瘤患者的总生存期。年龄在3至17岁之间，MRI显示为典型弥漫性脑桥胶质瘤或组织学证实为位于脑桥的间变性星形细胞瘤/多形性胶质母细胞瘤的患者符合条件。治疗方案为口服环磷酰胺100 mg x m(-2) x 天(-1)，联合口服依托泊苷25 mg x m(-2) x 天(-1)，与传统放疗同时开始。共纳入20例患者（中位年龄6岁，男:女 = 9:11）。手术方式包括：未手术：5例，开放活检：3例，立体定向活检：6例，部分切除：3例，次全切除：3例。组织学诊断包括毛细胞型星形细胞瘤：1例，未明确其他类型的星形细胞瘤：3例，间变性星形细胞瘤：3例，多形性胶质母细胞瘤：8例，无组织学诊断：5例。最常见的副作用是血液学和胃肠道方面的。无毒性死亡。12例可评估患者联合治疗的反应为：完全缓解：0例，部分缓解：3例，病情稳定：4例，病情进展：5例。所有肿瘤均局部进展，所有患者均死亡。总中位生存期为8个月。1年和4年的总生存率分别为：0.4和0.05。这与同一人群中记录的对照组患者无差异。口服环磷酰胺联合依托泊苷并未改善脑桥胶质瘤患者的生存期。该治疗耐受性良好，应针对更具化疗反应性的小儿恶性肿瘤进行评估。
