Adult Medulloblastoma Associated with Syringomyelia: A Case Report

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Abstract

The association between cerebellar medulloblastoma and syringomyelia is uncommon and only found in pediatric patients. To date, adult medulloblastoma associated with syringomyelia has not been reported in the literature. Paroxysmal bradycardia is an uncommon clinical manifestation in posterior fossa tumors and likely to be vagally mediated via brainstem preganglionic cardiac motor neurons. This report introduces the diagnosis and treatment of a case of adult medulloblastoma associated with syringomyelia, which presented with paroxysmal bradycardia.

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Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children's Cancer Group 921 randomized phase III study.

P Zeltzer, J Boyett, J L Finlay … (1999)

From 1986 to 1992, "eight-drugs-in-one-day" (8-in-1) chemotherapy both before and after radiation therapy (XRT) (54 Gy tumor/36 Gy neuraxis) was compared with vincristine, lomustine (CCNU), and prednisone (VCP) after XRT in children with untreated, high-stage medulloblastoma (MB). Two hundred three eligible patients with an institutional diagnosis of MB were stratified by local invasion and metastatic stage (Chang T/M) and randomized to therapy. Median time at risk from study entry was 7.0 years. Survival and progression-free survival (PFS) +/- SE at 7 years were 55%+/-5% and 54%+/-5%, respectively. VCP was superior to 8-in-1 chemotherapy, with 5-year PFS rates of 63%+/-5% versus 45%+/-5%, respectively (P = .006). Upon central neuropathology review, 188 patients were confirmed as having MB and were the subjects for analyses of prognostic factors. Children aged 1.5 to younger than 3 years had inferior 5-year estimates of PFS, compared with children 3 years old or older (P = .0014; 32%+/-10% v 58%+/-4%, respectively). For MB patients 3 years of age or older, the prognostic effect of tumor spread (MO v M1 v M2+) on PFS was powerful (P = .0006); 5-year PFS rates were 70%+/-5%, 57%+/-10%, and 40%+/-8%, respectively. PFS distributions at 5 years for patients with M0 tumors with less than 1.5 cm2 of residual tumor, versus > or = 1.5 cm2 of residual tumor by scan, were significantly different (P = .023; 78%+/-6% v 54%+/-11%, respectively). VCP plus XRT is a superior adjuvant combination compared with 8-in-1 chemotherapy plus XRT. For patients with M0 tumors, residual tumor bulk (not extent of resection) is a predictor for PFS. Patients with M0 tumors, > or = 3 years with < or = 1.5 cm2 residual tumor, had a 78%+/-6% 5-year PFS rate. Children younger than 3 years old who received a reduced XRT dosage had the lowest survival rate.

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Management of and prognosis with medulloblastoma: therapy at a crossroads.

Gilbert Vezina, Roger Packer (2008)

Medulloblastoma is the most common malignant childhood brain tumor and, although relatively uncommon in older patients, poses a therapeutic challenge in adults. With current means of therapy, children with nondisseminated medulloblastoma have a high likelihood of long-term survival; 80% or more will be alive 5 years after diagnosis and treatment, with many free of the disease. Even in children with disseminated disease, intensified therapy has been associated with improved survival rates, although some of this improvement may be more apparent than real. The quality of life in long-term survivors is a major issue, and most children who survive have substantial neurologic and cognitive sequelae. The outcome in infants and younger children with medulloblastoma is suboptimal, although there is some evidence to suggest that intensification of therapy has improved the likelihood of disease control. A better understanding of the biological characteristics of medulloblastoma including the cell or cells of origin and the aberrant cellular signaling pathways involved has the promise of dramatically changing tumor stratification and treatment in the near future. However, these biological advances have yet to be integrated into the treatment of medulloblastoma in children or adults.

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Adult medulloblastoma: prognostic factors and patterns of relapse.

Peter Chan, Aaron D. Black, J-P Loeffler … (2000)

To determine the patterns of relapse and the prognostic factors for adult medulloblastomas treated in the magnetic resonance imaging era. Between 1986 and 1996, 32 adult patients (age, > or =16 yr) with medulloblastomas confined to the craniospinal axis were treated in our institutions. Twenty cases involved classic histological features and 12 involved the desmoplastic variant. The Chang staging distribution was as follows: T1, 2; T2, 17; T3, 10; T4, 3; M0, 24; M1, 1; M2, 4; M3, 3. Brainstem invasion was present in nine patients. Lesions were midline in 13 cases and lateral in 19. Resection was complete in 17 cases, subtotal in 6, and partial in 5, with biopsy only in 4 cases. All patients received postoperative radiotherapy, with median doses of 36 Gy to the entire craniospinal axis and 55 Gy to the posterior fossa. Twenty-four patients received chemotherapy (20 before radiotherapy, 3 after radiotherapy, and 1 before and after radiotherapy). With a median follow-up period of 5.4 years, 17 patients experienced recurrences. At 5 and 8 years, overall survival rates were 83 and 45% and disease-free survival rates were 57 and 40%, respectively. The 5- and 8-year posterior fossa control rates were 67 and 59%, respectively. Twenty-nine percent of all relapses occurred more than 5 years after treatment. The posterior fossa was the most common site of relapses. In univariate analyses, factors adversely affecting posterior fossa control were less than complete resection (P<0.001), the presence of brainstem invasion (P = 0.02), and the use of chemotherapy (P = 0.03). The overall radiotherapy duration was marginally significant in predicting posterior fossa control, with 5-year posterior fossa control rates of 81 and 49% for durations of less than 48 days and 48 days or more, respectively (P = 0.06). In a multivariate analysis, complete resection was predictive of improved posterior fossa control (P = 0.02) and disease-free survival (P = 0.02) rates. Of the eight low-risk patients who received radiotherapy alone, three experienced recurrences in the bone as the only site of relapse. Late relapse is common among adult patients with medulloblastomas, and long-term follow-up monitoring is important. Because of the high risk of systemic failure among the low-risk patients treated with radiotherapy alone, the role of chemotherapy for this group of patients needs to be further investigated. Complete resection, the absence of brainstem invasion, and an overall radiotherapy duration of less than 48 days are important prognostic factors.

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Author and article information

Journal

Journal ID (nlm-ta): Cancer Biol Med

Journal ID (iso-abbrev): Cancer Biol Med

Journal ID (publisher-id): CBM

Title: Cancer Biology & Medicine

Publisher: Chinese Anti-Cancer Association

ISSN (Print): 2095-3941

Publication date (Print): June 2012

Volume: 9

Issue: 2

Pages: 137-140

Affiliations

[1]Nepean Hospital, Kingswood NSW 2747, Australia

Author notes

Correspondence to: Ching-Chun Wang

E-mail: cwan7975@ 123456uni.sydney.edu.au

Tel: 61-(0)423531859

Article

Publisher ID: cbm-09-02-137

DOI: 10.3969/j.issn.2095-3941.2012.02.011

PMC ID: 3643653

PubMed ID: 23691470

SO-VID: 47f514ee-b23a-4721-88e4-1896ad4afe48

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This work is licensed under a Creative Commons Attribution 3.0 Unported License. To view a copy of this license, visit http://creativecommons.org/licenses/by/3.0/

Adult Medulloblastoma Associated with Syringomyelia: A Case Report

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Most cited references 10

Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children's Cancer Group 921 randomized phase III study.

Management of and prognosis with medulloblastoma: therapy at a crossroads.

Adult medulloblastoma: prognostic factors and patterns of relapse.

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