Episcleral plaque brachytherapy for retinoblastoma

BACKGROUND: The purpose of this study was to report our experience using episcleral plaque brachytherapy (EPBRT) to treat retinoblastoma and to demonstrate its applicability in multimodality treatment. PROCEDURE: We treated 26 tumors in 25 eyes from a group of 21 children with unilateral (n = 4) or bilateral (n = 17) retinoblastoma. The group comprised 8 girls and 13 boys; the median age was 25 months (range: 2-64 months) at the time of EPBRT. Iodine-125 ((125)I) was used for all applications. The median dose was 44 Gy (range: 35-47.6 Gy). EPBRT was administered primarily at the time of relapse after primary chemotherapy or radiation therapy. RESULTS: For eyes treated with EPBRT, the eye preservation rate was 15/25 with a median follow-up of 47 months (range: 2-198 months); the lesion control rate was 25/26 with a median follow-up of 13 months (range: 1-140 months). The median time to additional whole-eye treatment after EPBRT was 12 months (range: 2-105 months). CONCLUSIONS: Similar to previously reported series, EPBRT shows a high rate of successful tumor control as a primary treatment for retinoblastoma, as well as a secondary therapy at the time of relapse. EPBRT also allows for a clinically significant delay in the time to additional measures for the affected eye. Therefore, EPBRT should be considered as a form of local ophthalmic therapy that avoids or delays the use of external-beam radiotherapy, especially for patients primarily treated with chemotherapy who might require consolidation therapy.     

Optimum radiation technique for retinoblastoma in conserved eyes

The study evaluated 121 consecutive unoperated cases of retinoblastoma in children. They received radiation as the primary treatment for their ocular disease. The stage grouping was done according to Reese classification. Ninety four children had their disease evaluated by CT scan. All except one had imaged lesions in both eyes. In addition, twenty six had thickened optic nerves and twenty one showed chiasmal infiltration. Radiation field selection was based on clinical and CT imaged disease. A tumor dose of 4500c Gy in 16 to 19 fractions over 18 to 24 days was delivered by cobalt teletherapy. The target volume encompassed ora serrata anteriorly and optic chiasma posteriorly in all cases. Tumor response and patient survival was correlated with the initial stage and radiation factors utilised amongst them. Encouraging results were obtained using three field radiation technique with an open anterior field and a TDF between 75 and 90.     

Combined chemotherapy and local treatment in the management of intraocular retinoblastoma

BACKGROUND: To assess the efficacy of chemotherapy (chemoreduction) plus local treatments as an alternative to external beam and enucleation for intraocular retinoblastoma. MATERIALS AND METHODS: A prospective study was performed on 21 patients with retinoblastoma treated in our institution from September 1997 to December 2000 to study the ocular outcome of those 33 eyes. RESULTS: There were 9 unilateral and 12 bilateral retinoblastoma cases. There were 12 eyes with Reese-Ellsworth group I-IV and 21 eyes with group V. Among 33 eyes, nine eyes (27%) were initially managed by enucleation. The remaining 24 eyes (73%) were initially treated with chemoreduction (maximum of six cycles of carboplatin, vincristine, etoposide) or chemothermotherapy. Among those 24 eyes, 20 were successfully treated with local treatments (thermotherapy plus cryotherapy in 16 eyes and thermotherapy plus cryotherapy plus (125)I plaque radiotherapy in 4 eyes), enucleation eventually underwent in two eyes and was proposed but refused in one child with bilateral group V retinoblastoma. With a median follow-up of 21 months, conservative management without external beam radiation was successful in all 12 eyes with group I-IV and in a total of 20/33 eyes (60%). Among the nine cases of unilateral retinoblastoma, eight were enucleated but among the 24 eyes with bilateral retinoblastoma, 19 (79%) were successfully treated with conservative therapy. CONCLUSIONS: It may be possible to eradicate viable tumor in all eyes with Reese-Ellsworth group I-IV retinoblastoma by chemoreduction followed by local treatments. Although 8 out of 21 eyes (38%) with group V retinoblastoma may be salvaged after chemoreduction and local therapies, enucleation remained the treatment of choice in those eyes with total retinal detachment and diffuse vitreous seeding.     

VEC方案治疗眼内期视网膜母细胞瘤的效果及安全性分析

目的探讨VEC方案治疗儿童眼内期视网膜母细胞瘤的效果并评价其安全性。方法对2006年1月至2008年2月间在复旦大学附属儿科医院血液肿瘤科门诊接受化疗的眼内期视网膜母细胞瘤患儿进行临床观察分析。结果共36例(48只眼),其中单侧24例,双侧12例,首次诊断平均年龄21.3月(2~93月),中位随访时间25.8月(15~36月)。12只眼IIRC分期为AC期,应用化疗结合局部治疗,1只眼(8.3%)经化疗仍发生进展,其余11只眼(91.7%)随访均持续缓解。36只眼IIRC分期为D~E期,应用眼球摘除术结合化疗,1例对侧眼出现新发病灶,1眼进展再次手术摘除眼球,其余34只眼(94.4%)随访均持续缓解。所有病例都能耐受化疗,仅1例(2.8%)发生轻度肝功能损害。结论化疗与局部治疗、手术相结合能对眼内期视网膜母细胞瘤进行有效的综合治疗。化疗的应用能避免外放疗的使用,降低眼球摘除术率,提高患儿生存质量,且安全可行,并发症少。     

Ocular preservation after 36 Gy external beam radiation therapy for retinoblastoma

PURPOSE: The purpose of this study was to document the ocular preservation rate after 36 Gy external beam radiation therapy (EBRT) for retinoblastoma. PATIENTS AND METHODS: Forty-nine eyes of 38 patients were treated with a median dose of 36 Gy EBRT. The patient population included 7 unilateral and 31 bilateral presentations, with a median age at diagnosis of 4 months. Eyes enucleated at the time of diagnosis or treated with other measures were not included in the analysis of ocular preservation. The median age at EBRT was 8 months. Patients were monitored for progression of disease after EBRT and second malignant neoplasms. RESULTS: The median follow-up was 88.6 months, with an estimated ocular preservation rate of 82.0% +/- 5% at 10 years. There was a difference in the ocular preservation rates for patients with advanced disease (Reese-Ellsworth group III-V) compared with early disease. Metastatic disease developed in two patients, and a second malignant neoplasm developed in three. Patients treated with en face electrons experienced a lower 5-year estimate of ocular preservation than those treated with photons, although patients treated with electrons were more likely to have advanced disease. CONCLUSIONS: The use of low-dose EBRT (36 Gy) results in ocular preservation rates that are comparable to those of high-dose EBRT. The use of electrons requires careful treatment planning and computerized dosimetry.     

Incidence of Anterior Pituitary Deficiency after Radiotherapy at an Early Age: Study in Retinoblastoma

ABSTRACT. Thirty-one patients treated for retinoblastoma in the first few years (3 months to 3 years and 6 months) of life were studied 2 to 15 years later. Radiotherapy delivered 1300 to 6 500 rads to the hypothalamo-pituitary area. Growth deficiency was documented in 30 % of all cases. Other pituitary deficiencies were the exception. The critical dose for GH insufficiency is between 2000 and 3000 rads, as in older children or adults. Our study does not support the hypothesis that the hypothalamo-pituitary area is more sensitive to radiation at an early age. Furthermore, conservative therapy of retinoblastoma leads to double lateral irradiation and will increase the number of GH deficient children after retinoblastoma.     

Incidence of anterior pituitary deficiency after radiotherapy at an early age: study in retinoblastoma

Thirty-one patients treated for retinoblastoma in the first few years (3 months to 3 years and 6 months) of life were studied 2 to 15 years later. Radiotherapy delivered 1 300 to 6 500 rads to the hypothalamo-pituitary area. Growth deficiency was documented in 30% of all cases. Other pituitary deficiencies were the exception. The critical dose for GH insufficiency is between 2 000 and 3 000 rads, as in older children or adults. Our study does not support the hypothesis that the hypothalamo-pituitary area is more sensitive to radiation at an early age. Furthermore, conservative therapy of retinoblastoma leads to double lateral irradiation and will increase the number of GH deficient children after retinoblastoma.     

Retinoblastoma in children older than 5 years of age

BACKGROUND: Retinoblastoma is a malignant tumor of the embryonic neural retina. About 80% of cases are diagnosed before age 4, with a median age at diagnosis of 2 years. OBJECTIVE: To determine characteristics and prognosis of retinoblastoma in children older than 5 years. PROCEDURES: From 1986 to 2002, medical records of 16 patients out of 453 cases referred to Hospital do Cancer AC Camargo, S?o Paulo, Brazil. RESULTS: Median age at diagnosis was 73.7 months (range 65-144) and there was an equal gender distribution. Fifteen patients presented with unilateral disease. The mean time between first symptoms and diagnosis was 9.6 months (range 0-48). Most cases were diagnosed in advanced stages and 15 eyes were enucleated. Eleven patients presented with intraocular tumor (1 Reese II and 10 Reese V) and five presented with extraocular disease (one CCG II and four CCG III). Twelve patients are still alive with a median follow-up of 92 months (range 65-199). CONCLUSIONS: Because of its low incidence at this age, diagnosis of retinoblastoma is usually delayed due to low level of suspicion. Therefore, it is important that physicians are aware of this disease in order to perform an earlier diagnosis, and decrease treatment-related morbidity.     

Clinical outcomes and toxicity following palliative radiotherapy for childhood cancers

1 Background

Few reports of palliative radiotherapy (RT) for pedialltric malignancies have been published. We described clinical indications, outcomes, and toxicities for children who received palliative RT.

2 Procedure

Pediatric patients (age ≤18 years) treated with palliative RT for incurable cancer from January 1 2008 to February 26, 2014 were included. Diagnosis, details of RT, treatment response, toxicity, and survival were retrospectively reviewed.

3 Results

Forty‐six patients received 76 RT courses. Fifteen patients (33%) had ≥2 courses. Median age at palliative RT was 10.3 years; 54% were male. The most common diagnoses were neuroblastoma (20%) and diffuse intrinsic pontine glioma (17%). The most common indications for RT were oligometastatic disease in asymptomatic patients (39%) and pain (25%). The most common treatment sites were brain (32%) and bone (29%). Median RT dose was 30 Gy. Median number of RT fractions was 12. Sixty‐five treatment courses (86%) were delivered with fraction sizes ≥2.5 Gy. Twenty‐seven treatment courses (36%) were given under general anesthesia. Median follow‐up was 3.9 months. Grade 1–2 RT‐related toxicity occurred in 21% of treatment courses and 4–8% up to 12 months after RT. Two patients had Grade 3 toxicity during RT (esophagitis). Of symptomatic patients, 91%, 73%, 58%, and 43% had improved or stable symptoms during RT and 0–3, 3–6, and 6–12 months afterwards, respectively. Median survival after palliative RT was 4.2 months. Four of 21 surviving patients (19%) had hospice care at last follow‐up.

4 Conclusions

Palliative RT was well tolerated in children with incurable malignancies, with most cases associated with acceptable toxicity, and improved or stable symptoms.     

Treatment outcome and dose-response relationship in infants younger than 1 year treated for retinoblastoma with primary irradiation

Thirty-three infants (<1 year at diagnosis) were treated for retinoblastoma with primary irradiation at St. Jude Children's Research Hospital (SJCRH) between 1963 and June 1992. Staging of the 44 treated eyes was as follows: Reese-Ellsworth (R-E) Groups I (n = 20), Group II (n = 9), Group III (n = 6), Group IV (n = 2), Group V (n = 7). Irradiation was delivered using either a single anterior field (31 eyes) or lens-sparing techniques (13 eyes). Total doses ranged from 21–45 Gy (median = 36 Gy) in fractions of 150–180 cGy (n = 34) or >180 cGy (n = 10). One child died of metastatic disease at 42 months. Three patients have developed second malignant neoplasms; two have succumbed at 88 and 125 months post-RB diagnosis; the remaining patients are alive at 6–259 months postdiagnosis (median follow-up = 127 months). Local control with irradiation alone and supplemented cryotherapy given within 2 months (n = 2) was maintained in 29 eyes, with no statistical difference seen for total doses ≤36 Gy (21/28 eyes) vs. >36 Gy (8/16). Of 15 eyes that required salvage therapy, tumor control has been maintained in 13. Enucleation was required for four patients, two with recurrent retinoblastoma and one with a massive retinal hemorrhage, one with a phthisical eye. Cataract formation was documented in 23 eyes (87.5%) treated with anterior field. Three patient treated with lens-sparing techniques developed cataract. At last follow-up, 23 of 30 patients tested (77%) had visual acuity of 20/100 or better. This experience confirms early observations in that doses ≥36 Gy do not appear to improve local control with irradiation alone in infants (<365 days) with retinoblastoma. © 1996 Wiley-Liss, Inc.     

24例儿童高危神经母细胞瘤调强适形放疗的近期疗效分析

目的 总结分析高危神经母细胞瘤患儿调强适形放疗（intensity-modulated radiotherapy,IMRT）疗效、安全性及相关经验。方法 回顾性选取2018年4月至2020年12月于湖南省人民医院儿童血液肿瘤科进行诊治并完成IMRT的高危组神经母细胞瘤患儿24例,收集相关资料,包括年龄、放疗剂量、放疗次数、实验室检查结果、不良反应、生存情况,分别进行归纳总结。结果 24例患儿均接受IMRT,其中男14例、女10例;平均年龄（65±23）个月,中位年龄59个月;原发瘤灶23例位于腹部,1例位于纵隔。放疗时患儿中位年龄41.5个月。放疗辐射剂量范围为14.4~36.0 Gy,平均剂量为（22±3）Gy,每日剂量为1.8~2.0 Gy,总次数8~20次,平均次数11.9次。其中6例接受了残留灶或转移灶放疗。放疗过程中3例出现咳嗽,2例出现腹泻,1例出现呕吐。放疗后2周,血肌酐范围值2.3~70.1 μmol/L,丙氨酸氨基转移酶范围值9.1~65.3 U/L,放疗开始后1~2周出现骨髓抑制Ⅲ度10例,Ⅳ度2例;放疗开始后3~4周出现骨髓抑制Ⅲ度4例,Ⅳ度1例。随访中位时间为13.5个月,其中23例（96%）疾病稳定,1例死亡,截至随访日期,未发现第2次恶性肿瘤及脏器功能异常。结论 IMRT能有效提高神经母细胞瘤局部控制率。IMRT治疗神经母细胞瘤患儿,短期内无明显不良反应,安全性高。     

Conservative therapy in intraocular retinoblastoma: response/recurrence rate

PURPOSE: To evaluate the response/recurrence rate and the outcome in intraocular retinoblastoma treated with chemoreduction and focal therapy, the authors performed a retrospective review of their patients. METHODS: The series included 46 newly diagnosed patients with unilateral or bilateral intraocular retinoblastoma (58 eyes) receiving carboplatin/etoposide chemotherapy associated with focal therapy (laser or cryotherapy). The mean follow-up was 53 months (range 11-125). RESULTS: Fifty-one eyes (88%) presented with complete response after four to eight courses of chemotherapy combined with focal treatment. The response rate was 100% in group 1, 94% in group 2, 100% in group 3, 83% in group 4, and 70% in group 5 (5 vs. 1-4, P < 0.03; 5-4 vs. 1-3, P < 0.025). Twenty-nine eyes (57%) relapsed after a mean of 7 months (range 2-36). The relapse rate was 30% in group 1, 27.% in group 2, 67% in group 3, 80% in group 4, and 100% in group 5 (5 vs. 1-4, P < 0.001; 4-5 vs. 1-3, P < 0.001). Seven of 18 cases achieved a second complete response with further conservative treatment (total courses 8-14). Twenty-nine eyes (50%) were treated without external-beam radiotherapy or enucleation: 90% in group 1, 69% in group 2, 67% in group 3, 33% in group 4, and 6% in group 5 (5 vs. 1-4, P < 0.01; 5-4 vs. 1-3, P < 0.001). Ten eyes (17%) required external-beam radiotherapy and 21 eyes (36%) enucleation. The ocular salvage rate was 67%. CONCLUSIONS: Although all groups of patients with intraocular retinoblastoma responded to carboplatin/etoposide chemotherapy associated with focal therapy, all the cases in group 5 relapsed. This approach is questionable in group 5, in which could be justified to delay aggressive treatment in a very young child.     

Long-term morbidity in children treated with fractionated high-dose-rate brachytherapy for soft tissue sarcomas

BACKGROUND: The purpose of this study was to determine the long-term local control, disease-free survival, and morbidity of fractionated high-dose-rate brachytherapy (F-HDR) in infants and children with soft tissue sarcomas. PATIENTS AND METHODS: Fifteen children (13 girls and 2 boys, ages 5-101 months) with soft tissue sarcomas were treated with chemotherapy, organ-preserving surgery, and F-HDR (36 Gy in 12 fractions) to post-chemotherapy volumes. External beam radiotherapy was not part of the primary treatment, although four patients (27%) subsequently received salvage external beam radiotherapy after treatment failure. Chemotherapy was administered to all patients based on their tumor histology and stage. RESULTS: After a median follow-up of 10 years (range 32-154 months), 12 patients (80%) were alive without evidence of disease. Ten-year overall survival and local control rates were both 80% (12/15 children). The overall survival was better (91%) for children with microscopic residual versus gross residual disease (75%). With longer follow-up, grade 3 to 4 brachytherapy-related late morbidities increased from 8% (1/12) to 20% (3/15) and included trismus/osteonecrosis, vaginal stenosis, and periurethral fibrosis. There were two late complications associated with puberty that occurred 8 to 10 years after the initial treatments. Acute toxicity occurred in five patients (38%) and consisted primarily of grade 1 to 3 skin and mucosal reactions. CONCLUSIONS: As the sole radiation modality, F-HDR achieved excellent local control and disease-free survival in properly selected children with soft tissue sarcomas while preserving normal bone and organ development. A significant percentage of patients experience adverse late sequelae as a result of this treatment.     

Use of Irradiation for Therapy of Retinoblastoma in Children More Than 1 Year Old: The St. Jude Children's Research Hospital Experience and Review of Literature

Fifteen children (>1 year old at diagnosis) were treated for retinoblastoma (RB) with primary irradiation at St. Jude Children's Research Hospital between January 1963 and January 1992. Staging of the 19 treated eyes was as follows: Reese-Ellsworth (R-E) Groups I and II, n = 7; Group III, n = 3; Group IV, n = 1; Group V, n = 7; information on one globe is incomplete. Total dosage ranged from 25 to 45 Gy (median = 40 Gy) in fractions of 170–225 cGy delivered 5x/week (n = 13) or 225–265 cGy delivered 4x/week (n = 4 eyes) or x5/week (n = 2 eyes). Treatment techniques included anterior field (n = 11) or lens-sparing technique (n = 8). One patient has died of progressive central nervous system (CNS) disease at 13 months; one patient succumbed to second malignant neoplasm within the irradiated field at 194 months; the remaining 13 patients remain alive from 27 to 301 months (median = 178 months). Local control with irradiation alone has been maintained in 12 eyes. Four eyes with local recurrence were salvaged using cryotherapy or reirradiation. Enucleation was required for three eyes for progressive disease at 4, 7, and 7 months postirradiation. Cataract formation was documented in nine eyes treated with anterior fields and in one patient treated with lens-sparing technique. At last follow-up, 7 patients had visual acuity of 20/100 or better. Radiation dose of 40 Gy appears to be adequate for local control of early stage RB (R-E Groups I-III and VB whose tumors are R-E Groups I-III) in patients >1 year old. The results of this limited series which has lengthy follow-up is compared with the results of previously published data in an effort to define the benefits and disadvantages of the different treatment techniques which have been reported in the treatment of RB in children >1 year old. © 1995 Wi1ey-Liss, Inc.     

Childhood retinoblastoma

Childhood retinoblastoma was studied in a period of January 1980 to December 1988. Forty six patients were included consisting of 21 males (45.65%) and 25 females (54.35%). Most of those patients (76.08%) were in the age group of 0-5 years. The main complaint was protrusion of eyes, found in 42 patients (91.30%). Bone marrow examination was done in 24 patients, and 52.17% of them showed sign of metastasis. Thirty seven cases had unilateral retinoblastoma (80.43%). The statistical analysis indicated no significant differences in sex and age, site of tumor (left or right eye), abnormality of the eyes, bone marrow involvement, protrusion of the eyes either unilateral or bilateral. Heredity was found in one patient (2.17%). The main treatment was radiation in 25 patients (50.43%).     

Combined radiation and chemotherapy for advanced undifferentiated nasopharyngeal carcinoma in children

Five children, 11–16 years of age at diagnosis, with advanced (stage IV) undifferentiated nasopharyngeal carcinoma, are reviewed. All had radiotherapy and chemotherapy, four at first treatment, are disease free in 8 months, 6, 10, and 13 years from diagnosis. One patient who had radiotherapy only as primary treatment and chemotherapy for metastases, died 15 months from diagnosis. One patient who received 60 Gy and chemotherapy according to the BACON protocol had severe early toxicity and severe late sequelae of treatment. Three patients who received chemotherapy according to a modified UICC-2 protocol for nasopharyngeal carcinoma and 50 to 54 Gy to the primary site had only mild early toxicity and mild late effects of treatment. With lower radiation doses, adjusted to the effect of preradiation chemotherapy, complete tumor control was achieved and acute and long-term morbidity reduced. Med. Pediatr. Oncol. 28:366–369, 1997. © 1997 Wiley-Liss, Inc.     

Retinoblastoma in Turkey: survival and clinical characteristics 1981–2004

BACKGROUND: In this study, the authors aim to describe the survival and clinical characteristics of 141 retinoblastoma cases treated at Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, between 1981 and 2004. METHOD: The authors retrospectively analyzed the clinical records of 141 children (177 eyes) diagnosed with retinoblastoma and treated between 1981 and 2004. Information on gender, laterality, age at diagnosis, presenting signs, spread of tumor, treatment modality, survival rate, and family history were collected. RESULTS: A total of 105 cases (74.5%) were unilateral and 36 cases (25.5%) were bilateral. The mean age overall at the time of diagnosis was 25 months; in unilateral cases, 29 months; and in bilateral cases, 16 months. The most common presenting signs were leukocoria (116 cases, 82%), strabismus (14 cases, 10%) and proptosis (11 cases, 8%). A total of 28 cases had orbital extension, nine patients had central nervous system invasion, and five cases exhibited bone marrow involvement. In total, 16 patients (11%) had a family history of retinoblastoma. One case developed a secondary neoplasm. The 3 year cumulative survival rate of 141 patients was 89.69% (unilateral, 90.74%; bilateral 87.35% P = 0.9371, P > 0.05, log rank test). CONCLUSION: The study's survival rate was similar to developed countries. The success in higher survival rates is based on the authors multidisciplinary team approach done by the same group and the support of the authors' clinic and government in sponsoring the medical insurance of all patients.     

Robotically guided radiosurgery for children

BACKGROUND: A robotically guided linear accelerator has recently been developed which provides frameless radiosurgery with high precision. Potential advantages for the pediatric population include the avoidance of the cognitive decline associated with whole brain radiotherapy, the ability to treat young children with thin skulls unsuitable for frame-based methods, and the possible avoidance of general anesthesia. We report our experience with this system (the "Cyberknife") in the treatment of 21 children. PROCEDURES: Cyberknife radiosurgery was performed on 38 occasions for 21 patients, age ranging from 8 months to 16 years (7.0 +/- 5.1 years), with tumors considered unresectable. Three had pilocytic astrocytomas, two had anaplastic astrocytomas, three had ependymomas (two anaplastic), four had medulloblastomas, three had atypical teratoid/rhabdoid tumors, three had craniopharyngiomas, and three had other pathologies. The mean target volume was 10.7 +/- 20 cm(3), mean marginal dose was 18.8 +/- 8.1 Gy, and mean follow-up is 18 +/- 11 months. Twenty-seven (71%) of the treatments were single-shot and eight (38%) patients did not require general anesthesia. RESULTS: Local control was achieved in the patients with pilocytic and anaplastic astrocytoma, three of the patients with medulloblastoma, and the three with craniopharyngioma, but not for those with ependymoma. Two of the patients with rhabdoid tumors are alive 16 and 35 months after this diagnosis. There have been no procedure related deaths or complications. CONCLUSION: Cyberknife radiosurgery can be used to achieve local control for some children with CNS tumors without the need for rigid head fixation.     

HIT-LGG: effectiveness of carboplatin-vincristine in progressive low-grade gliomas of childhood--an interim report

BACKGROUND: The rationale of the HIT-LGG protocol is to delay standard radiotherapy through administration of chemotherapy for children with progressive low grade glioma at an age under 5 years and in older children upon individual decision. PATIENTS AND METHOD: Until October 10th., 1999, 402 patients from 69 hospitals were registered. 130 children with progressive tumors were treated after a median observation time of 5 months: 46 patients received primary radiotherapy and 84 primary chemotherapy. A ten week induction period with weekly Vincristine and pulses of Carboplatin at weeks 1, 4, 7 and 10 is followed by consolidation with simultaneous application of both drugs every 4 weeks until week 53. RESULTS: Of 84 patients in the chemotherapy arm of the study (49 male, 35 female, 23 NF I, median age 2.99 years) 36 received treatment at diagnosis and 43 after a median observation time of 19.7 months. 94.3% achieved a clinical and neuroradiological response according to protocol criteria (5 CR, 30 PR/OR, 31 SD) after a median of 5.1 months. 4 tumors showed primary progression (9 too early, 5 not known). Only 6 of 84 children received radiation therapy for progressive disease during (2) or after termination (4) of chemotherapy, after a median delay of 25.6 months at a median age of 6.0 years. At a median observation time of 21.0 months, 6 children are in CR, 11 in PR, 48 have SD, 4 tumors are progressive and 3 children died of their tumor. (9 too early, 3 not known). PFS is at 72% after 36 months. 24 of 27 children experiencing allergic reactions to Carboplatin had to interrupt chemotherapy prematurely. CONCLUSIONS: Combination therapy with Carboplatin and Vincristine can effectively delay the start of radiotherapy in children with progressive low-grade glioma. The high rate of hypersensitivity reactions has to prompt future modifications of treatment.