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 ◽  
...  

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.

2008 ◽  
Vol 108 (2) ◽  
pp. 204-209 ◽  
Author(s):  
Anand V. Germanwala ◽  
Jeffrey C. Mai ◽  
Nestor D. Tomycz ◽  
Ajay Niranjan ◽  
John C. Flickinger ◽  
...  

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.


2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i51-i51
Author(s):  
Aashim Bhatia

Abstract Pediatric glioma response to novel targeted therapy can be heterogeneous on conventional proton (1H) MRI. Sodium concentration, as measured with 23Na MRI in adult brain tumors can provide complementary assessment of tumor proliferation to conventional MRI. However, 23Na MRI pediatric brain tumor studies are lacking. Determine the technical feasibility of performing sodium23Na MRI on pediatric glioma patients. Prospective study of an immunotherapy trial for newly diagnosed and recurrent gliomas (high-grade gliomas, low-grade gliomas, brainstem gliomas) in which participants were imaged with 23Na MRI at 3.0 Tesla. The participants (n=26, 14 males) with median age of 11 years (range = 4–23 years of age) were prospectively evaluated with sodium. 23Na MRI is technically feasible in the pediatric population and can distinguish different types of pediatric gliomas at baseline.


Author(s):  
D. Yusuf ◽  
A. Krauze ◽  
J. Easaw ◽  
A. Murtha ◽  
J. Amanie ◽  
...  

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.


2016 ◽  
Vol 18 (suppl 3) ◽  
pp. iii106.3-iii106
Author(s):  
Maura Massimino ◽  
Marie Pierre Sunyach ◽  
Lorenza Gandola ◽  
Filippo Spreafico ◽  
Alice Bonneville Levard ◽  
...  

2019 ◽  
Vol 23 (4) ◽  
pp. 498-506 ◽  
Author(s):  
Tofey J. Leon ◽  
Elizabeth N. Kuhn ◽  
Anastasia A. Arynchyna ◽  
Burkely P. Smith ◽  
R. Shane Tubbs ◽  
...  

OBJECTIVEThere are sparse published data on the natural history of “benign” Chiari I malformation (CM-I)—i.e., Chiari with minimal or no symptoms at presentation and no imaging evidence of syrinx, hydrocephalus, or spinal cord signal abnormality. The purpose of this study was to review a large cohort of children with benign CM-I and to determine whether these children become symptomatic and require surgical treatment.METHODSPatients were identified from institutional outpatient records using International Classification of Diseases, 9th Revision, diagnosis codes for CM-I from 1996 to 2016. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, 3) had previously undergone posterior fossa decompression, or 4) had imaging evidence of syringomyelia at their first appointment. To include only patients with benign Chiari (without syrinx or classic Chiari symptoms that could prompt immediate intervention), any patient who underwent decompression within 9 months of initial evaluation was excluded. After a detailed chart review, patients were excluded if they had classical Chiari malformation symptoms at presentation. The authors then determined what changes in the clinical picture prompted surgical treatment. Patients were excluded from the multivariate logistic regression analysis if they had missing data such as race and insurance; however, these patients were included in the overall survival analysis.RESULTSA total of 427 patients were included for analysis with a median follow-up duration of 25.5 months (range 0.17–179.1 months) after initial evaluation. Fifteen patients had surgery at a median time of 21.0 months (range 11.3–139.3 months) after initial evaluation. The most common indications for surgery were tussive headache in 5 (33.3%), syringomyelia in 5 (33.3%), and nontussive headache in 5 (33.3%). Using the Kaplan-Meier method, rate of freedom from posterior fossa decompression was 95.8%, 94.1%, and 93.1% at 3, 5, and 10 years, respectively.CONCLUSIONSAmong a large cohort of patients with benign CM-I, progression of imaging abnormalities or symptoms that warrant surgical treatment is infrequent. Therefore, these patients should be managed conservatively. However, clinical follow-up of such individuals is justified, as there is a low, but nonzero, rate of new symptom or syringomyelia development. Future analyses will determine whether imaging or clinical features present at initial evaluation are associated with progression and future need for treatment.


Author(s):  
R. Luque ◽  
M. Benavides ◽  
S. del Barco ◽  
L. Egaña ◽  
J. García-Gómez ◽  
...  

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.


2001 ◽  
Vol 37 ◽  
pp. S87-S88
Author(s):  
A. Le Cesne ◽  
M.C. Le Deley ◽  
L. Brugières ◽  
C. Kalifa ◽  
G. Missenard ◽  
...  

Blood ◽  
2009 ◽  
Vol 113 (6) ◽  
pp. 1375-1382 ◽  
Author(s):  
Jan J. Cornelissen ◽  
Bronno van der Holt ◽  
Gregor E. G. Verhoef ◽  
Mars B. van 't Veer ◽  
Marinus H. J. van Oers ◽  
...  

Abstract While commonly accepted in poor-risk acute lymphoblastic leukemia (ALL), the role of allogeneic hematopoietic stem cell transplantation (allo-SCT) is still disputed in adult patients with standard-risk ALL. We evaluated outcome of patients with ALL in first complete remission (CR1), according to a sibling donor versus no-donor comparison. Eligible patients (433) were entered in 2 consecutive, prospective studies, of whom 288 (67%) were younger than 55 years, in CR1, and eligible to receive consolidation by either an autologous SCT or an allo-SCT. Allo-SCT was performed in 91 of 96 patients with a compatible sibling donor. Cumulative incidences of relapse at 5 years were, respectively, 24 and 55% for patients with a donor versus those without a donor (hazard ratio [HR], 0.37; 0.23-0.60; P < .001). Nonrelapse mortality estimated 16% (± 4) at 5 years after allo-SCT. As a result, disease-free survival (DFS) at 5 years was significantly better in the donor group: 60 versus 42% in the no-donor group (HR: 0.60; 0.41-0.89; P = .01). After risk-group analysis, improved outcome was more pronounced in standard-risk patients with a donor, who experienced an overall survival of 69% at 5 years (P = .05). In conclusion, standard-risk ALL patients with a sibling donor may show favorable survival following SCT, due to both a strong reduction of relapse and a modest nonrelapse mortality. This trial is registered with http://www.trialregister.nl under trial ID NTR228.


2020 ◽  
Vol 25 (5) ◽  
pp. 459-464 ◽  
Author(s):  
Jelte Kelchtermans ◽  
Jessica Chang ◽  
Wendy Glaberson ◽  
Marissa DeFreitas ◽  
Monica Alba-Sandoval ◽  
...  

Sirolimus is an immunosuppressive medication often used in solid organ transplantation. It has been associated with severe side effects, including pulmonary toxicity. In adult patients, a single center study found that 14% of those treated with sirolimus developed pulmonary pneumonitis; however, the incidence in the pediatric population is not known. Most reports in adult patients indicate that elevated drug concentrations and a prolonged duration of use are associated with pulmonary toxicity. We report a case of a 17-year-old male kidney transplant recipient who developed rapid-onset respiratory failure, necessitating mechanical ventilation and acute renal replacement therapy for ultrafiltration secondary to sirolimus-induced pneumonitis. He had been treated for acute rejection with corticosteroids 17 days prior to the development of pneumonitis. His symptoms developed within 1 week of initiation of sirolimus and with a serum concentration of 1.1 ng/mL. Sirolimus was discontinued, and, following aggressive diuresis and ventilatory support, his respiratory status returned to baseline. Sirolimus-induced pneumonitis is an important diagnosis to be considered in any transplant recipient receiving sirolimus with new onset fever, cough, or dyspnea without an identifiable source, especially if there is a preceding history of treatment with high-dose corticosteroids.


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