Adult Medulloblastoma: Prognostic Factors and Patterns of Relapse

Chan, Annie W.; Tarbell, Nancy J.; Black, Peter McL.; Louis, David N.; Frosch, Matthew P.; Ancukiewicz, Marek; Chapman, Paul H.; Loeffler, Jay S.

doi:10.1097/00006123-200009000-00018

Cited by 120 publications

(128 citation statements)

References 35 publications

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“…It was stated that 29% of the recurrences occurred 5 years after treatment. [25] In our study, recurrence was detected in 3 patients at 39 th , 47 th , and 57 th months, found at the primary tumor location. Those patients died at 2 nd and 26 th months after recurrence.…”

Section: Discussionsupporting

confidence: 46%

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Outcomes of Surgery and Craniospinal Radiotherapy for Adult Patients with Medulloblastoma

Güzelöz¹

2017

TJ Oncol

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OBJECTIVEThe aim of the present study was to evaluate treatment outcomes for adult patients with medulloblastoma who received craniospinal irradiation following surgery at Dokuz Eylül University Radiation Oncology Department. METHODSTwelve patients were evaluated retrospectively. Median age was 31 years (range: 18-55 years). According to Chang staging system, 3 (25%) of the patients were T1, 2 (17%) were T2, 5 (42%) were T3, and 1 (8%) was T4; 1 patient could not be staged due to inadequate preoperative imaging. Tumor location, stage, presence of residual disease, duration between surgery and radiotherapy (RT), age, and sex were evaluated as prognostic factors. Statistical analyses were conducted using Kaplan-Meier method and SPSS for Windows, Version 15.0 (SPSS Inc., Chicago, IL, USA). RESULTSMedian dose was 36 Gy (range: 32-44 Gy) to craniospinal field and 54 Gy (range: 50-56 Gy) to primary tumor location. Median follow-up time was 62 months (range: 4-212 months). Five-, 10-, and 15-year overall survival rates were 79%, 63%, and 63%, and 5-, 10-and 15-year progression-free survival rates were 63%, 63%, and 63%, respectively. No statistically significant prognostic factor was found for survival rate. Three (25%) patients developed serious hematological toxicity during RT course. No grade 3 or 4 late side effect was observed. CONCLUSIONDespite the limited number of cases, results are consistent with the literature. Evaluation of features of this rare disease requires studies with larger number of.

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Section: Discussionsupporting

confidence: 46%

“…There was no recurrence or distant metastasis in the follow-up after adjuvant RT or CT. Recurrences develop mostly in posterior fossa. [23][24][25] A review of recurrence patterns indicated that recurrences occur mostly within 2 years in pediatric group. [26,27] This duration is longer for adult patients.…”

Section: Discussionmentioning

confidence: 99%

Outcomes of Surgery and Craniospinal Radiotherapy for Adult Patients with Medulloblastoma

Güzelöz¹

2017

TJ Oncol

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“…However, adult medulloblastoma is arguably a biologically distinct challenge in that it exhibits a higher proportion of desmoplastic histological characteristics, shows more proclivity toward cerebellar hemispheric origin, possesses different proliferative and apoptotic indices, and demonstrates a notorious tendency for late relapse. 1,13 Metastatic disease spread via the cerebrospinal fluid is commonly present when this malignant embryonal tumor or the histologically related supratentorial PNET is diagnosed. 5 Thus, medulloblastoma demands a broader armamentarium than that provided by open resection.…”

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confidence: 99%

Boost Gamma Knife surgery during multimodality management of adult medulloblastoma

Germanwala

Jeffrey

Tomycz

et al. 2008

JNS

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Object. The aim of this paper was to determine prognostic factors for adult medulloblastoma treated with boost Gamma Knife surgery (GKS) following resection and craniospinal irradiation.Methods. The authors performed a retrospective analysis of 12 adult patients with histologically proven medulloblastoma or supratentorial primitive neuroectodermal tumor who between February 1991 and December 2004 underwent Ն 1 sessions of GKS for posttreatment residual or recurrent tumors (6 tumors in each group). Before GKS, all patients had undergone a maximal feasible resection followed by craniospinal irradiation. Nine patients also received systemic chemotherapy. Stereotactic radiosurgery was applied to residual and recurrent posterior fossa tumor as well as to foci of intracranial medulloblastoma metastases. The median time interval from initial diagnosis and resection to the first GKS treatment was 24 months (range 2-37 months). The mean GKS-treated tumor volume was 9.4 cm 3 (range 0.5-39 cm 3 ). Results. Following adjunctive radiosurgery, 5 patients had no evidence of tumor on magnetic resonance (MR) imaging, 3 patients had stable tumor burden on MR imaging, and 4 patients had evidence of tumor progression locally with or without intracranial metastases. All patients with tumor progression died. Eight patients survive with a mean cumulative follow-up of 72.4 months (range 21-152 months). No acute radiation toxicity or delayed radiation necrosis was observed among any of the 12 patients. The majority of patients who achieved tumor eradication (80%) and tumor stabilization (67%) after GKS had residual tumor as the reason for their referral for GKS. The best outcomes were attained in patients with residual disease who were younger, had smaller tumor volumes, had no evidence of metastatic disease, and had received higher cumulative GKS doses.Conclusions. Single or multiple GKS sessions were a well-tolerated, feasible, and effective adjunctive treatment for posterior fossa residual or recurrent medulloblastoma as well as intracranial metastatic medulloblastoma in adult patients.

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“…MBs are overwhelmingly found in pediatric patients, but can rarely occur within adult brain, where the tumor characteristics become very atypical. Adult MB is arguably a biologically distinct challenge in that it exhibits a higher proportion of desmoplastic histological characteristics, shows more proclivity toward cerebellar hemispheric origin, possesses different proliferative and apoptotic indices, and demonstrates a notorious tendency for late relapse with respect to the pediatric variants (Chan et al, 2000;Sarkar et al, 2002). MBs are thought to arise within the cerebellum, with approximately 25% originating from granule neuron precursor cells (GNPCs) (Gibson et al, 2010) after aberrant activation of the Sonic Hedgehog (Shh) pathway ( Figure 1B).…”

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confidence: 99%