scholarly journals MB-43REDUCED DOSE CRANIOSPINAL IRRADIATION (CSI) IS FEASIBLE FOR STANDARD RISK ADULT MEDULLOBLASTOMA (MBL) PATIENTS SIMILARLY TO PEDIATRIC POPULATION

2016 ◽  
Vol 18 (suppl 3) ◽  
pp. iii106.3-iii106
Author(s):  
Maura Massimino ◽  
Marie Pierre Sunyach ◽  
Lorenza Gandola ◽  
Filippo Spreafico ◽  
Alice Bonneville Levard ◽  
...  
Keyword(s):  
Pediatric Population ◽  
Craniospinal Irradiation ◽  
Adult Medulloblastoma ◽  
Standard Risk

scholarly journals P14.77 Reduced dose craniospinal irradiation (CSI) is feasible for standard risk adult medulloblastoma (MBL) patients similarly to pediatric population

2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii85-iii86
Author(s):  
M Massimino ◽  
M Sunyach ◽  
L Gandola ◽  
E Pecori ◽  
F Spreafico ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Pediatric Population ◽  
Pediatric Brain Tumor ◽  
Molecular Classification ◽  
Adult Brain ◽  
Adult Patients ◽  
Current Therapy ◽  
Published Data ◽  
Adult Medulloblastoma ◽  
Standard Risk

Abstract BACKGROUND MBL is the most common malignant pediatric brain tumor but represents 1% of adult brain tumors. Recent molecular classification suggests that MBL is not the same disease in children and adults. For standard risk pediatric medulloblastomas current therapy includes CSI at reduced doses (23.4Gy) associated with chemotherapy. Most adult patients with similar risk factors still receive CSI at 36 Gy±chemotherapy (CT): in the adult series treated according to the HIT protocol (CSI 35.2 Gy + boost to 55.2 Gy to posterior fossa followed in most patients by maintenance CT with lomustine, vincristine and cisplatin) a 73% 5-year PFS was reported {Friedrich, Eur J Cancer 2013}, so far the best published data. Hence retrospective experience of 23.4 Gy together with CT for adult patients in some institutions is worthwhile reporting. MATERIAL AND METHODS We gathered M0 patients, aged over 18 years with medulloblastomas and no/minimal post-surgical residues/no biological negative factor, between 1996–2018 in Centre Léon Bérard of-Lyon and Fondazione IRCCS Istituto Nazionale dei Tumori-Milan. RESULTS Forty-four patients were included, median age 26 (18–48) years,20 females. Median follow-up 90 months(10–227). Thirty-six and 8 received 23.4Gy and 30Gy CSI, respectively, + posterior fossa/tumor bed boost and CT in all: pre-RT (carbo/VPx2 courses or 8 drugs-in-one day x 2 courses (13 patients)) and/or post-RT (carbo/VPx2 courses in 11 and 8 drugs-in-one day x 2 courses in 10; CDDP/VCR/CCNU x 8courses in other 22). The 5/10 year PFS and OS were respectively 80.9±6.5%/76.8±7.4% and 88.4±5.5%/73.1±8.5%. Median progression time was 44 months. Relapses (8) were local (4), local+CSF or spine or bone in one instance each and bone only in one. Among variable considered, higher CSI dose than 23.4 Gy, pre-RT CT did not influence PFS, while females had a trend to better PFES and OS (P=0.07). CONCLUSION These combined series present results comparable to - or even better than- those obtained after high CSI doses highlighting the need for treatment redefinition in adults.

Reduced-dose craniospinal irradiation is feasible for standard-risk adult medulloblastoma patients

2020 ◽  
Vol 148 (3) ◽  
pp. 619-628
Author(s):  
Maura Massimino ◽  
Marie Pierre Sunyach ◽  
Francesco Barretta ◽  
Lorenza Gandola ◽  
Anna Garegnani ◽  
...  
Keyword(s):  
Craniospinal Irradiation ◽  
Reduced Dose ◽  
Adult Medulloblastoma ◽  
Standard Risk

scholarly journals MPC-08 Molecular risk stratification using genome-wide DNA methylation data of standard-risk medulloblastomas treated with 18-Gy craniospinal irradiation

2020 ◽  
Vol 2 (Supplement_3) ◽  
pp. ii12-ii12
Author(s):  
Tomoya Irikura ◽  
Kohei Fukuoka ◽  
Makiko Mori ◽  
Koichi Oshima ◽  
Yuki Arakawa ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Disease Onset ◽  
Low Risk ◽  
Craniospinal Irradiation ◽  
Diagnostic Tools ◽  
Late Relapse ◽  
Mycn Amplification ◽  
Group 4 ◽  
Radiation Induced ◽  
Standard Risk

Abstract A novel risk stratification of medulloblastoma has been proposed based on retrospective data from patients undergoing standard treatment. However, it remains unclear whether the classification is applicable to patients receiving reduced-dose craniospinal irradiation (CSI). We performed molecular diagnosis and copy number analysis using methylation array on patients with standard-risk medulloblastoma treated with 18 Gy CSI at our institution. Nine tumor samples were available for analysis from seven patients who had a median age of 7.4 years at disease onset and a median observation period of 73 months. Three patients had recurrence, and another patient developed radiation-induced glioblastoma. From the three recurrent cases, one was molecularly diagnosed as SHH subtype with MYCN amplification; another case was a Group 4 tumor without favorable prognostic chromosomal aberrations, and the remaining patient experienced a very late relapse despite low-risk stratification. Of the recurrence-free cases, one was classified as WNT subtype, and another was a Group 4 tumor with chromosome 7 gain, and loss of chromosomes 8 and 11, both of which were associated with good prognosis. Methylation analysis also unveiled the fact that the recurrent tumor diagnosed as relapsing medulloblastoma by conventional diagnostic tools was in fact a radiation-induced glioblastoma. Our data suggested that the new risk stratification may be useful for cases treated with CSI reduced to 18 Gy. However, due to the presence of the late-relapsed case stratified to low risk, further investigations with a larger cohort should be required to confirm the data.

scholarly journals SEOM clinical guideline for management of adult medulloblastoma (2020)

2021 ◽  
Author(s):  
R. Luque ◽  
M. Benavides ◽  
S. del Barco ◽  
L. Egaña ◽  
J. García-Gómez ◽  
...  
Keyword(s):  
Residual Tumor ◽  
Large Cell ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Molecular Alterations ◽  
Adult Medulloblastoma ◽  
Group 4 ◽  
Risk Patients ◽  
Standard Risk

AbstractRecent advances in molecular profiling, have reclassified medulloblastoma, an undifferentiated tumor of the posterior fossa, in at least four diseases, each one with differences in prognosis, epidemiology and sensibility to different treatments. The recommended management of a lesion with radiological characteristics suggestive of MB includes maximum safe resection followed by a post-surgical MR < 48 h, LCR cytology and MR of the neuroaxis. Prognostic factors, such as presence of a residual tumor volume > 1.5 cm2, presence of micro- or macroscopic dissemination, and age > 3 years as well as pathological (presence of anaplastic or large cell features) and molecular findings (group, 4, 3 or p53 SHH mutated subgroup) determine the risk of relapse and should guide adjuvant management. Although there is evidence that both high-risk patients and to a lesser degree, standard-risk patients benefit from adjuvant craneoespinal radiation followed by consolidation chemotherapy, tolerability is a concern in adult patients, leading invariably to dose reductions. Treatment after relapse is to be considered palliative and inclusion on clinical trials, focusing on the molecular alterations that define each subgroup, should be encouraged. Selected patients can benefit from surgical rescue or targeted radiation or high-dose chemotherapy followed by autologous self-transplant. Even in patients that are cured by chemorradiation presence of significant sequelae is common and patients must undergo lifelong follow-up.

Boost Gamma Knife surgery during multimodality management of adult medulloblastoma

2008 ◽  
Vol 108 (2) ◽  
pp. 204-209 ◽  
Author(s):  
Anand V. Germanwala ◽  
Jeffrey C. Mai ◽  
Nestor D. Tomycz ◽  
Ajay Niranjan ◽  
John C. Flickinger ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Tumor Progression ◽  
Posterior Fossa ◽  
Gamma Knife ◽  
Gamma Knife Surgery ◽  
Adult Patients ◽  
Craniospinal Irradiation ◽  
Time Interval ◽  
Radiation Toxicity ◽  
Adult Medulloblastoma

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 ≥ 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 cm3 (range 0.5–39 cm3). 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.

Pattern of blood count change during craniospinal irradiation and the effect on progression free survival in medulloblastoma adult patients.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e13500-e13500
Author(s):  
Khaled Nabil Dibs ◽  
Imad A. Jaradat ◽  
Jamal Khader ◽  
Laith Al Khairi ◽  
Nasim Sarhan ◽  
...  
Keyword(s):  
Blood Count ◽  
Progression Free Survival ◽  
Primary Treatment ◽  
Craniospinal Irradiation ◽  
P Value ◽  
Significant Drop ◽  
Free Survival ◽  
Adult Medulloblastoma ◽  
Grade 3 ◽  
Minimum Interval

e13500 Background: The primary treatment of medulloblastoma in adults is surgery followed by radiotherapy. Craniospinal irradiation in such patients holds a clinical significant effect on blood count drop, as more than 50% of bone marrow is irradiated during CSI. Precise guidelines on how often a patient's peripheral blood count should be monitored are yet to be established. Methods: A retrospective analysis of blood count data on 42 adult medulloblastoma patients who underwent surgical resection followed by CSI as 36-39.6Gy over 20-22 fractions followed by boost to posterior cranial fossa up to 54Gy and spinal boost up to 45Gy. The manner of blood count cells during radiotherapy and the correlation between WBC drop and outcome were examined. Results: Our study showed a drop in WBCs to 4 and below in 64% of patients during CSI. Eight patients (19%) had Hb drop below 12. There was no grade 3 or 4 anemia. Significant drop in neutrophils below 1500 was observed in 8 patients (20%), with 2.4% had grade 3 neutropenia. In 26 patients (62%) who developed platelets drop, three patients (7%) had platelets of less than 50000 (grade 3 and 4 thrombocytopenia). The Hb, lymphocyte, neutrophils, and platelets nadirs were 8.7 g/dL, 42 cells /microL, 720 cells /microL, and 1000 platelets/microL, respectively. The minimum interval between the nadir and starting radiotherapy for Hb, WBCs, and platelets were 13, 6, and 11 days, respectively. The median interval between the nadir and starting radiotherapy for all cells was 3 weeks. The median WBCs percentage drop was 71%. In 23 patients who had non-metastatic disease, the estimated three year progression free survival was 70%. In those who had WBC drop below 2.7 the PFS was 100% vs. 46%. (P value = 0.042). Conclusions: Some institutions reduced monitoring of CBC during conventional radiotherapy may result in financial saving; however, our study suggests the need for weekly CBC for patients receiving CSI irradiation. Fortunately, no one developed serious infection during radiotherapy, but a precaution should be there. A WBCs change during radiotherapy could reflect the outcome and may suggest more aggressive treatment in patients who had a nadir of 2.7 and above.

scholarly journals P14.03 Adult medulloblastoma: Influence of risk stratification and molecular subtypes on chemotherapy efficacy

2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii66-iii67
Author(s):  
O Absalyamova ◽  
G Kobiakov ◽  
G Agabekyan ◽  
M Ryzhova ◽  
N Kobiakov ◽  
...  
Keyword(s):  
High Risk ◽  
Molecular Genetic ◽  
Craniospinal Irradiation ◽  
Disease Stage ◽  
Molecular Subgroups ◽  
Molecular Subgroup ◽  
Treatment Protocols ◽  
Adult Medulloblastoma ◽  
Radiotherapy Alone ◽  
Radio Chemotherapy

Abstract BACKGROUND Incidence of medulloblastoma (MB) in adults is about 1%. Optimal treatment for adult medulloblastoma has not been defined. Published datasets were acquired over a long period of time, during which there were changes in surgical technique, radiotherapy, chemotherapy regimens; often disease stage was not considered in the analyses. Recently discovered molecular subgroups are not taken into account in studies concerning treatment of medulloblastoma in adults. MATERIAL AND METHODS Study includes 64 patients with medulloblastoma (median age 26.5 years (15–62)), who were first admitted to Burdenko Neurosurgery Institute for surgery from 2000 to 2017. 100 % patients included in the study received radiotherapy - craniospinal irradiation, 36 Gy (20 x 1.8 Gy) with boost to primary site (2 cm around tumor bed) up to 55 Gy total dose. 41 patients (64%) received chemotherapy (ChT). Chemotherapy was started 4–6 weeks after finishing craniospinal irradiation, the regimen included cisplatin 25 mg/m2 days 1–4+etoposide 100 mg/m2 days 1–4+cyclophosphamide 600 mg/m2 days 1–4; the cycles repeating every 28 days, 6 cycles in total. Treatment volume didn’t correspond with risk group and/or molecular genetic subgroup (these parameters were determined retrospectively). Median follow-up time is 4.45 years. RESULTS 53% patients had classic histology MB, 27% - desmoplastic, 20% - large cell/anaplastic. High risk (HR), standard risk (SR) and unspecified risk (UR) groups included 23, 16 and 25 patients respectively. Molecular subgroup testing was performed in 39 cases: SHH-activated - 59%, WNT-activated - 18%, GROUP4 - 23%. Median time to tumor progression (MTTP) in patients who received radio-chemotherapy versus radiotherapy alone was 4.3 and 2.64 years, respectively (p=0.264). The differences in MTTP were the most pronounced in SR and UR patients who received radio-chemotherapy (5.5 and 6.0 months, respectively) versus radiotherapy alone (1.5 and 2.0 months, respectively). HR patients showed the least difference in MTTP for radiotherapy (2.0 years) versus radio-chemotherapy (2.5 years), p=0.093. The SHH-subgroup patients had MTTP of 3.1 years (3.1 and 3.0 years for ChT+ and ChT-, respectively). GROUP4 patients had MTTP of 4.3 years (4.3 and 1.5 years for ChT+ and ChT-, respectively). All WNT-subgroup patients received chemotherapy, MTTP was 9.0 years. Median overall survival cannot be defined in any of the subgroups. CONCLUSION Chemotherapy in adult medulloblastoma patients increases MTTP in SR and UR patients. Low efficacy of chemotherapy for high risk medulloblastoma urges search for more efficacious treatment protocols. WNT-activated adult medulloblastoma has the highest MTTP among other molecular subgroups.

scholarly journals P14.04 10 years of weekly web conference for brain tumor of Adolescent/Young Adult (AYA) on behalf of ANOCEF, GO-AJA and SFCE societies

2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii67-iii67
Author(s):  
D Frappaz ◽  
C Bronnimann ◽  
C Faure Conter ◽  
E Vauleon ◽  
D Meyronnet ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumors ◽  
Embryonal Tumors ◽  
Web Based ◽  
Pineal Tumors ◽  
Adult Medulloblastoma ◽  
Multidisciplinary Meetings ◽  
Inclusion Rate ◽  
Adolescent And Young Adults ◽  
Standard Risk

Abstract BACKGROUND The skills of adult versus pediatric neuro-oncologists are not completely similar though additive. Because the tumors and their protocols are different and the tolerance and expected sequelae are specific. Multidisciplinary meetings including adult and pediatric neuro oncologists are warranted to share expertise. MATERIAL AND METHODS Since 2008, on behalf of ANOCEF, GO-AJA and SFCE societies, a weekly national web based conference was held in France. Any patient with the following criteria could be discussed: Adolescent and Young Adults aged between 15 and 25 years, and any adult with a pediatric type pathology, including medulloblastoma, germ cell tumors, embryonic tumors, ependymoma, pilocytic astrocytoma. RESULTS Number of cases discussed increased from 8 to 202/year, with a mean number of 3 cases (1 to 7) discussed at each meeting. In 2018, 36 meetings were held, with a mean of 8 participants (2 to 14) issued from various French centers. 168 cases were discussed in 2018. Mean age was 30 years old (7 to 67). 45% were discussed at diagnosis; The patients had mostly medulloblastomas (n=47), ependymomas (n=24), low (n=21) or high grade gliomas (n=11), pineal tumors (n=20), germ cell tumors (n=9) and embryonal tumors (n=6). The rate of inclusion in protocols was increased since the opening of this web conference, especially for the germ cell tumor GCT SIOP protocol that is opened without age restriction, and in RSMA standard risk or MEVITEM relapse adult medulloblastoma protocols. CONCLUSION Multidisciplinary Web conference for AYAs is feasible and fruitful. It shares knowledge and increases the inclusion rate in protocols. It should be developped further.

scholarly journals 06 Prognostic factors for survival and recurrence in adult medulloblastoma

2018 ◽  
Vol 45 (S3) ◽  
pp. S1-S1
Author(s):  
D. Yusuf ◽  
A. Krauze ◽  
J. Easaw ◽  
A. Murtha ◽  
J. Amanie ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Posterior Fossa ◽  
Treatment Options ◽  
Recurrence Risk ◽  
Subtotal Resection ◽  
Limited Evidence ◽  
Adult Medulloblastoma ◽  
Standard Risk ◽  
4Th Ventricle

BACKGROUND: Adult medulloblastomas account for less than 1% of adult neoplasms. They are challenging to treat due to their rarity and the heterogeneity of treatment options, all of which have limited evidence. In this retrospective review, we examined cases of adult medulloblastoma diagnosed in Alberta during a 70-year period. METHODS: We reviewed the charts of patients diagnosed with medulloblastoma between 1944 and 2014. We performed Cox and logistic regression analysis to elucidate features that may influence recurrence risk and survival. RESULTS: We found 86 and analyzed 78 cases. The median age at diagnosis was 27 (range 16 to 71). Most were male (68%). Most had surgery (92%). By COG risk stratification, 54% were standard risk while 21% were poor risk. RT was administered to 85% of patients, and craniospinal irradiation (CSI) to 81%. Chemotherapy was administered to 48%. Median survival was 4.4 years from diagnosis (range 0 to 20). At last follow-up, 39% were alive and recurrence-free. Patients who had CSI and posterior fossa boost had longer survival (p=0.047 and<0.01, respectively) and were less likely to recur (p=0.041 and<0.01). Chemotherapy was also associated with decreased recurrence (p=0.025). CONCLUSIONS: Medulloblastomas carry a significant recurrence risk, especially for patients who had subtotal resection. CSI and posterior fossa boost were associated with fewer recurrences and improved survival. COG risk stratification, Chang staging, desmoplastic histology, vermian location, 4th ventricle involvement, tumor enhancement, presence of hydrocephalus and cerebrospinal fluid (CSF) involvement are not significantly prognostic.

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