Évolution de la prise en charge des médulloblastomes de l’enfant et de l’adulte

Vigneron, C.; Entz‐Werlé, Natacha; Lutz, Pierre G.; Spiegel, A.; Jannier, Sarah; Helfre, Sylvie; Alapetite, Claire; Coca, Andrés; Kehrli, P.; Noël, G.

doi:10.1016/j.canrad.2015.03.010

Cited by 3 publications

(2 citation statements)

References 129 publications

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“…Four molecular subgroups exist in medulloblastoma: Wingless (WNT), Sonic Hedgehog (SHH), Group 3, and Group 4. Compared to children, adults comprise a greater proportion of SHH (60 vs. 30%) and relatively fewer Group 3 (0–5 vs. 25%) subgroup patients [4–8].…”

Section: Introductionmentioning

confidence: 99%

Long-term outcomes of adult medulloblastoma patients treated with radiotherapy

Beal

Braganca

et al. 2017

J Neurooncol

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Medulloblastoma (MB) is rare in adults and treatment guidelines are consequently not well-established. Few modern series have reported long-term follow-up and treatment sequelae. We examined long-term outcomes of adult MB patients at one institution. Records of 29 consecutive patients (18 male, 11 female) aged ≥ 18 years who received radiotherapy (RT) for primary MB from 1990 to 2016 were reviewed. Median age at diagnosis was 28 years (range 18-72 years). Seventeen patients were standard risk and 12 were high risk. Nineteen patients had gross total resection, seven had subtotal resection, and three had biopsy only. Median craniospinal irradiation and boost doses were 36 Gy (range 23.4-39.6 Gy) and 55.8 Gy (range 54-59.4 Gy), respectively. Of 24 patients receiving chemotherapy, 20 received concurrent + adjuvant and 4 received adjuvant only. At median follow-up of 9.0 years (range 1.1-20.5 years), five patients recurred: four in the posterior fossa and one in both the posterior fossa and above the tentorium. Five patients died: two of disease progression and three after possible treatment complications (seizure, lobar pneumonia, and multifactorial sepsis). At last follow-up, 23 patients were alive with no evidence of disease. Long-term effects include executive dysfunction (n = 17), weakness/ataxia (n = 16), and depression/anxiety (n = 13). Kaplan-Meier estimates of 10-year overall survival and failure-free survival are 83% (95% confidence interval [CI] 59-93%) and 79% (CI 55-91%), respectively. Despite encouraging disease control in this cohort, long-term sequelae may limit quality of life. Multimodality pediatric regimens using lower RT doses may be considered to reduce treatment-related morbidity.

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Section: Introductionmentioning

confidence: 99%

Long-term outcomes of adult medulloblastoma patients treated with radiotherapy

Beal

Braganca

et al. 2017

J Neurooncol

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“…In adults, radiotherapy is the consensus and must be initiated within 90 days after the surgery. [ 20 ]…”

Section: Discussionmentioning

confidence: 99%

Adult hemispheric cerebellar medulloblastoma

Oliveira¹,

Landeiro²,

Castro³

2018

Surg Neurol Int

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Background:Medulloblastoma is an embryonal neoplasm and accounts for 1% of all adult intracranial tumors. It is associated with many familiar cancer syndromes, but there is no known cause for medulloblastoma. Many studies have documented differences between childhood and adult medulloblastomas in terms of location, proliferation, and apoptotic indices. There are four histological groups – classic and the variant forms (desmoplastic/nodular, anaplasic, and large cell). There are four major subgroups according to molecular configuration: wingless (WNT), sonic hedgehog (SHH), group 3, and group 4 with differences between them according to prognostic outcomes.Case Description:We present the case of a 19-year-old female who complained of headache and vomiting. On neurological exam, she was awake, conscious, and had mild truncal ataxia, dysmetria, and intentional tremor. Brain magnetic resonance imaging (MRI) showed an intra-axial left hemisphere cerebellar lesion causing midline shift tonsilar herniation. She was submitted for posterior fossa craniotomy and microsurgical resection of cerebellar tumor and then to 18 Gy adjuvant radiotherapy to the tumor bed and 23 Gy to the neuroaxis.Conclusion:This article briefly discusses the newest points in classification, diagnosis, and treatment of medulloblastoma. This case illustrates the diagnostic workup and treatment of a rare tumor in adults showing the importance of molecular and histological studies for the treatment and counseling of the patient. Medulloblastoma has different prognosis depending on the histological and molecular feature. Accessing these different features is essential to better plan the treatment as well as inform the patient regarding the disease and its prognosis.

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Posterior Fossa Intra-Axial Tumors in Adults

Grossman

Ram

2016

World Neurosurgery

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Évolution de la prise en charge des médulloblastomes de l’enfant et de l’adulte

Cited by 3 publications

References 129 publications

Long-term outcomes of adult medulloblastoma patients treated with radiotherapy

Long-term outcomes of adult medulloblastoma patients treated with radiotherapy

Adult hemispheric cerebellar medulloblastoma

Posterior Fossa Intra-Axial Tumors in Adults

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