Medulloblastoma – an overview (original) (raw)
Related papers
Medulloblastoma. Results of a sequential combined treatment
Cancer, 1984
Actuarial progression-free survival rate at 5 years of a series of 34 patients with medulloblastoma treated by combined surgery, radiotherapy, and chemotherapy was 71%. No relapses were observed in 14 patients followed for more than 5 years. Treatment consisted of a short postoperative course of vincristine (VCR) and intrathecal (IT) methotrexate (MTX) followed by irradiation to the entire cranio spinal axis. Maintenance chemotherapy (CCNU, VCR, and IT MTX) was then continued to encompass 2 years from surgery. Failure occurred in nine patients: four had local recurrence, four dissemination within the central nervous system, and one widespread skeletal metastases. Poor prognostic factors such as presence of malignant cells in the cerebrospinal fluid, non-radical surgery, young age, and radiation doses less than 50 Gy to the tumor bed, did not adversely affect the outcome of patients in this series. Long-term sequelae from the treatment program could be observed in all patients, and in 58% they were severe enough to interfere with normal, active life.
South Asian Journal of Cancer, 2015
Objectives: Adult medulloblastoma (AMB) is a rare central nervous system tumor. We aimed to analyze the treatment outcomes of AMB treated at our institute with surgery followed by craniospinal irradiation (CSI) and adjuvant chemotherapy. Methods: We retrospectively evaluated the treatment charts of 31 patients of AMB treated from 2003-2011. The patient demography, treatment details and survival data were collected in a predesigned proforma. Kaplan Meier method was used to analyze disease free survival (DFS) and the impact of prognostic factors was determined by univariate analysis (log rank test). Results: Male: Female ratio was 21:10. Cerebrospinal fluid dissemination was noted in 16% cases. CSI (36 Gray at 1.8 Gray/fraction to entire neuraxis and 20 Gray at 2 Gray/fraction boost to posterior fossa) was used in all cases. 26 patients received adjuvant chemotherapy (carboplatin plus etoposide). Median follows up was 26.85 months (9.47-119.73 months). The estimated 3 and 5 years DFS ...
Treatment results for 149 medulloblastoma patients from one institution
International Journal of Radiation Oncology Biology Physics, 1996
Purpose: Retrospective analysis of patients with medulloblastoma to determine the effectiveness of previous treatments for medulloblastoma and plan for future management strategies. Methods and Materials: During the period March 1976 to December 1991, 172 patients with cerebellar medulloblastoma were referred to King Faisal Specialist Hospital and Research Center. One hundred and forty-nine patients were treated with curative intent. There were six postoperative deaths, and 10 patients planned for radiotherapy treatment failed to complete the prescribed course. One hundred and thirty-three patients completed a course of radiotherapy after surgery. Adjuvant chemotherapy was not used routinely (six patients only). Tumors were staged retrospectively according to the Chang staging system. There were no T1 patients, 32 patients had T2 tumors, 76 had T3 tumors, and 29 had T4 tumors. The T stage could not be allocated in 12 patients. Ninety-nine patients required a shunting procedure either pre-or postoperatively. Forty-six patients had complete resection of tumor, 91 had incomplete resection, and 6 patients had biopsy only. The extent of resection could not be determined in six patients. The median radiation dose for the whole brain was 34 Gy, spine 32.5 Gy, and posterior fossa 52.8 Gy. Fraction sizes ranged from 1.7-1.8 Gy for craniospinal fields and 2 Gy for the posterior fossa boost. Seventy percent completed the prescribed course within 7 weeks. Results: Actuarial survival for the whole group of 149 patients was 53% at 5 years and 38% at 10 years. On univariate analysis, patients with T2 tumors did significantly better as compared to patients with T3 and T4 tumors. Survival of patients who had clinical and radiological complete resection of tumor at surgery was significantly better than patients with incomplete tumor removal. The presence of a ventriculoperitoneal (VP) shunt had a significant negative impact on survival. Treatment failure by site was analyzed with respect to the radiation dose. Doses greater than 50 Gy for the posterior fossa, and greater than 30 Gy for craniospinal axis, resulted in significantly better survival. On multivariate analysis, the only significant prognostic factor was the presence of a VP shunt in patients with T2 tumors. Conclusion: T stage, VP shunt, radiation doses and extent of surgery were important prognostic factors. In this study, radiation doses of more than 50 Gy to the posterior fossa and 30 Gy to the craniospinal axis resulted in improved survival.
Cancer, 1983
For 43 medulloblastoma patients who had five-and ten-year actuarial survival rates of 56%, prognostic factors of statistical significance included: T-stage (82% versus 46% T3.4; P < 0.02), M-stage (63% Mo.l ;hiw~s 0% M2,3; P < 0.03), and histopathologic tumor score (TS, based upon necrosis, desmoplasia, cytoplasmic processes, and mitoses) (81% TS I 5 versus 41% TS 2 6; P < 0.05). Posterior fossa local control rates were also function of T-stage (90% versus 38% T3.4) and TS (83% TS I 5 versus 38% TS 2 6). Combining TS with T-stake, patients fell into three prognostic and local control groups, which may have different future management implications: Small tumors of favorable (TS < 5) histology had a 92% ten-year actuarial survival rate with 100% (8/8) local control; no change from current management is suggested. For the intermediate prognosis group (T1.2-TS L 6 or T3.4-TS I 5 with 67% and 70% survival, respectively), increasing the irradiation dose alone may improve survival because these tumors exhibited an irradiation dose-response relatiodship. However, it is the poor prognosis group (T3,4-TS 2 6 with 42% survival) which might be suitable for future adjuvant chemotherapy or radiosensitizer trials since there is no evidence that higher irradiation doses improve local control. This article identifies prognostic subgroups based on histologic type and T M staging in medulloblastoma patients which potentially may be utilized to improve therapeutic results, and confirms the value of staging patients with central nervous system malignancies.
Medulloblastoma in adults: clinical characteristics and treatment
Cancer Treatment Reviews, 1999
Long-term survival is possible in adults with medulloblastoma with rates of approximately 50-60% at 5 years, and 40-50% at 10 years. As the literature data are based on retrospective studies, treatments are neither randomized nor uniform, however, some treatment cornerstones have been identified. The first step is surgery, which should be as radical as possible; adjuvant radiotherapy must be 55 Gy on the posterior fossa, and 36 Gy on the remaining cranial-spinal axis; adjuvant chemotherapy may be useful in patients at high risk of recurrence provided it is administered before radiotherapy in moderate-high dosages and includes cisplatin, etoposide and cyclophosphamide.This chemotherapy program should not overly delay the start of radiotherapy, be recycled as soon as blood count permits and not exceed two or three cycles. Adjuvant chemotherapy after radiotherapy, even if indicated in cases with persistent tumour, may have an adverse effect due to the poor marrow reserves of these patients. At recurrence, the prospects of cure are very poor due to the deficient hematopoietic reserve, but in very young patients high dose chemotherapy with marrow rescue might be usefully employed.
Medulloblastoma in adults: treatment results and prognostic factors
International Journal of Radiation Oncology*Biology*Physics, 2002
Purpose: To investigate the treatment outcome and prognostic factors of adult medulloblastoma patients who received postoperative craniospinal irradiation (RT). Methods and Materials: Between 1983 and 2000, 30 adult patients (17 men and 13 women, age >16 years, median 27, range 16-45) underwent postoperative RT. The median duration of symptoms was 2 months (range 1-9). The tumor location was lateral in 16 (53%). A desmoplastic variant was seen in 12 (40%). Tumor resection was complete in 20 (67%) and incomplete in 10 (33%). All patients received craniospinal RT. The median dose to the whole brain was 40 Gy (range 36-51), to the posterior fossa 54 Gy (range 49-56), and to the spinal axis 36 Gy (range 24-40). The median interval between surgery and the start of RT was 31 days (range 12-69), and the median duration of RT was 45 days (range 34-89). Ten patients (33%) received adjuvant chemotherapy. The median follow-up was 51 months (range 5-215). Results: The 5-and 8-year overall survival and disease-free survival rates were 65% and 51% and 63% and 50%, respectively. Twelve patients (40%) developed relapse, with a median follow-up of 51 months. The posterior fossa was the most common site of relapse (6 patients). The median time to relapse was 26 months (range 4-78). Fifty percent of the relapses occurred after 2 years, 17% after 5 years. In univariate analysis, M stage and the interval between surgery and the start of RT were significant prognostic factors for disease-free survival. At 5 years, 70% of M0 patients were estimated to be disease-free, but none of the 3 M3 patients reached 5 years without recurrence (p ؍ 0.0002). The 5-year disease-free survival rate for the patients whose interval between surgery and the start of RT was <3 weeks, between 3 and 6 weeks, and >6 weeks was 0%, 85%, and 75%, respectively (p ؍ 0.002). The 5-year posterior fossa control rate for patients who received >54 Gy or <54 Gy to the posterior fossa was 91% and 33%, respectively (p ؍ 0.05). Conclusion: The survival results for medulloblastomas in adults compare favorably with those in children. However, late relapses, lateral tumor location, and desmoplastic histologic features are more frequent in adults. Spinal seeding at presentation is a poor prognostic factor for disease-free survival. A minimal dose of 54 Gy to the posterior fossa is essential for adequate tumor control. The interval between surgery and the start of RT, which was found to be a significant prognostic factor, is an interesting issue that requires further study.
Medulloblastoma in adults: Survival and prognostic factors
Radiotherapy and Oncology, 1993
The authors report a series of 30 adults with medulloblastoma treated after surgery between 1975 and 1990. Half the patients presented a classical medulloblastoma and the other half presented desmoplastic medulloblastoma. Brainstem infiltration was noted in 3 patients, cerebellar peduncle infiltration in 6 cases and 4 patients had cerebrospinal fluid (CSF) involvement. Tumor resection was achieved in all patients except 1, and in 5 surgery was incomplete. All patients but 2 received postoperative cerebral radiotherapy. Six patients did not receive chemotherapy before radiotherapy, 7 patients received CCNU-Vincristine concomitantly with and after irradiation, 1 patient was given a CHOP regimen, and the remaining 16 patients received the 8 drugs in a l-day (8/1) regimen before irradiation. The median follow-up time was 104 months. The 5-and 10-year overall survival rates were 58.5% and 41%, respectively. Two toxic deaths occurred under the 8/1 regimen, and no better survival was observed for this group of patients in comparison with those receiving other regimens. Persistance of malignant cells in the CSF, brainstem involvement, cerebellar peduncle infiltration, non-radical surgery and a postoperative performance status (PS) of more than 2 were significantly correlated with an adverse outcome for overall survival in adult patients with medulloblastoma.
Medulloblastoma—A retrospective analysis
Indian Journal of Pediatrics, 1997
A retrospective review of 45 patients was undertaken at the All India Institute of Medical Sciences to assess the outcome and prognostic factors for these patients who received post operative radiotherapy with or without chemotherapy for medulloblastoma. The median age at diagnosis was 11 years, with 34 males and 11 female patients. Thirty four tumours were confined to midline structures, and 11 were localised to one cerebellar hemisphere or involved midllne and lateral structures. Complete macroscopic removal was achieved in 24 patients and subtotal removal in 21 patients. Forty one patients underwent craniospinal irradiation and 27 patients received adjuvant chemotherapy. Median overall and disease free survival was 57 and 31 months respectively and 3 year overall, survival was 76%. The addition of adjuvant chemotherapy was a significant factor for disease free survival (p = 0.01) whereas extent of surgery (total vs subtotal, p = 0.01) was a significant factor for overall survival only. Eleven patients developed recurrent disease, with ten relapsing first in the posterior fossa.
Local control in non-metastatic medulloblastoma
Acta Oncol, 2014
Background. A single-institution review of long-term outcomes and factors affecting local control (LC) following radiotherapy for non-metastatic medulloblastoma. Material and methods. From 1963 to 2008, 50 children (median age, 7.3 years; range 1.2-18.5) with stage M0 medulloblastoma were treated with radiotherapy; half underwent a gross total resection (no visible residual tumor) or near-total resection (Ͻ 1.5 cm 3 of gross disease remaining after resection). Median craniospinal dose was 28.8 Gy (range 21.8-38.4 Gy). Median total dose to the posterior fossa was 54.3 Gy (range 42.4-64.8 Gy). Eighteen patients (36%) received chemotherapy as part of multimodality management, including 11 who received concurrent chemotherapy. Results. Median follow-up was 15.7 years (range 0.3-44.4 years) for all patients and 26.6 years (range 7.3-44.4 years) for living patients. The 10-year overall survival, cancer-specifi c survival, and progression-free survival rates were 65%, 65%, and 69%. The 10-year LC rate was 84% and did not signifi cantly change across eras. Four percent of patients experienced local progression fi ve years after treatment. On univariate analysis, chemotherapy and overall duration of radiotherapy Յ 45 days were associated with improved LC. Patients receiving chemotherapy had a 10-year 100% LC rate versus 76% in patients not receiving chemotherapy (p ϭ 0.0454). When overall radiotherapy treatment lasted Յ 45 days, patients experienced a superior 95% 10-year LC rate (vs. 73% in patients treated Ͼ 45 days; p ϭ 0.0419). Three patients (6%) died from treatment complications, including radionecrosis/cerebellar degeneration, severe cerebral edema leading to herniation, and secondary malignancy. Conclusions. While we cannot draw defi nitive conclusions given the retrospective nature of our study, our long-term data suggest that reductions in craniospinal dose and boost target volume to reduce toxicity have not compromised disease control in the modern era. Our data also support analyses that implicate duration of radiotherapy, rather than interval between surgery and radiotherapy, as a factor in LC. Chemotherapy in multimodality management of medulloblastoma may have an underappreciated role in improving LC rates.
Current therapy and new perspectives in the treatment of medulloblastoma
Pediatric Neurology, 1998
Medulloblastoma, a malignant tumor arising from the medullary velum, is the most common malignant brain tumor of childhood. Local extension into the cerebellar hemisphere, infiltration of the floor of the fourth ventricle, and seeding into the subarachnoid space are common. Early diagnosis and improved treatment consisting of surgery followed by radiation and chemotherapy for selected high-risk patients has contributed to a dramatic change in survival. This article reviews current treatment strategies and describes new therapies that have the potential to improve the outlook of children with medulloblastoma.