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4. Follow-up evaluation of a web-based pediatric brain tumor board in Latin America

Author

Rosabal-Obando M, Osorio DS, Lassaletta A, Morales-La Madrid A, Bartels U, Finlay JL, Qaddoumi I, Rutkowski S, and Mynarek M

Subjects
virtual tumor boards, tele-oncology, childhood brain tumors, low- and middle-income countries, high-income countries, neuro-oncology, outreach
Abstract

BACKGROUND: Since 2013, pediatric oncologists from Central and South America discuss neuro-oncology cases with experts from North America and Europe in a web-based "Latin American Tumor Board" (LATB). Here, we evaluate the feasibility of recommendations rendered by the Board. METHODS: An electronic questionnaire was distributed to physicians who had received recommendations between October 2017 and October 2018. Physicians were asked regarding the feasibility of each recommendation given during the LATB discussion. Baseline case characteristics of all presented cases were obtained from anonymized minutes. RESULTS: Of the 142 patients discussed, data on 103 patients from 15 countries were available, corresponding to 283 recommendations. Physicians followed 60% of diagnostic procedural recommendations and 69% of therapeutic recommendations. The most difficult recommendations to follow were genetic and molecular testing, pathology review, chemotherapy, surgery, and molecular targeted therapies. Histological diagnoses changed in eight of 18 cases in which a pathology review was undertaken. Fifty-four percent of the recommendations that could not be implemented were considered not feasible in the specific context of the patient, while 31% were not implemented due to a decision of the medical staff or the parents (15% not specified). However, 96% of respondents considered the recommendations useful. CONCLUSION: Recommendations were frequently perceived as useful, and were applicable in the participating institutions. Nevertheless, limitations in availability of diagnostic procedures and treatment modalities affected the feasibility of some recommendations. Tele-oncology tumor boards offer physicians from low- and middle-income countries access to real-time, high-level subspecialist expertise and provide a valuable platform for worldwide information exchange.

Published
2021

5. Survival of retinoblastoma in less-developed countries impact of socioeconomic and health-related indicators

Author

Canturk, S., Qaddoumi, I., Khetan, V., Ma, Z., Furmanchuk, A., Antonell, C.B.G., Sultan, I., Kebudi, R., Sharma, T., Rodriguez-Galindo, C., Abramson, D.H., and Chantada, G.L.

Subjects
Retinoblastoma -- Care and treatment, Retinoblastoma -- Patient outcomes, Retinoblastoma -- Demographic aspects, Retinoblastoma -- Research, Developing countries -- Health aspects, Developing countries -- Research, Cancer -- Care and treatment, Cancer -- Patient outcomes, Cancer -- Social aspects, Cancer -- Economic aspects, Cancer -- Research, Health
Published
2010

10. Pattern of ABCC Transporter Gene Expression in Pediatric Patients with Relapsed Acute Lymphoblastic Leukemia

Author

Mehrvar, N., Abolghasemi, H., Rezvany, M. R., Akbari, M. E., Saberynejad, J., Mehrvar, A., Ehsani, M. A., Nourian, M., Qaddoumi, I., and abolfazl movafagh

Subjects
Original Article
Abstract

BACKGROUND: Abnormal expression of ABCC transporter genes has been associated with treatment failure in pediatric patients with acute lymphoblastic leukemia (ALL). The aim of this study was to evaluate the expression pattern of ABCC1-6 and ABCC10 genes in Iranian pediatric patients with ALL relapse and determine the potential predictive value of determining ALL relapse from ABCC expression. METHODS: Patients with ALL were divided into two separate groups, either the case group with relapsed ALL or the control group in which ALL patients have been in progression-free survival for at least 3 years A total of thirty-nine participants (23 with relapsed ALL; 16 controls) were enrolled over 26 months. To determine the levels of ABCC1-6 and ABCC10 transporter gene expression RT-PCR was used. Cumulative doses of the chemotherapy drugs, VCR, DNR and L-ASP, were calculated for each patient. RESULTS: Our findings showed elevated expression of ABCC2-6 and decreased expression of ABCC1 and ABCC10 to be associated with an increased risk of ALL relapse. The mean-fold expression of ABCC2 was significantly increased in the ALL relapse group. Additionally, the expression pattern of the ABCC transporter genes was associated with high doses of three chemotherapy drugs, VCR, DNR and L-ASP. CONCLUSION: Evaluating the expression pattern of ABCC transporter genes may be a potential biomarker for predicting the occurrence of ALL relapse in Iranian pediatric patients and improve cancer prognosis.

Published
2019

11. The Latin American Brain Tumor Board teleconference: results of a web-based survey to evaluate participant experience utilizing this resource

Author

Abu Arja MH, Stanek JR, Morales-La Madrid A, Lassaletta A, Bartels U, Qaddoumi I, Finlay JL, and Osorio DS

Subjects
Lower and middle income countries, Childhood brain tumors, Neuro-oncology, Telemedicine, Global oncology, Central nervous system tumors
Abstract

PURPOSE: The Latin American Brain Tumor Board (LATB) is a weekly teleconference connecting pediatric neuro-oncologists from referral centers in high-income countries with pediatric subspecialists from 20 Latin American countries since 2013. This survey explored the participants' experience utilizing this resource. METHODS: A cross-sectional electronic questionnaire was distributed to 159 participants through email and Cure4Kids. RESULTS: Ninety-five respondents (60%) from all the participating countries completed the survey. Sixty-one reported frequent-attendance (= 1 per month), 23 reported infrequent-attendance (< 1 per month), and 11 never participated. The most frequently reported attendance-barriers were the subspecialist's workload (64%), the timing of the teleconference (38%), and Internet connectivity problems (29%). Subspecialist's workload was more frequently reported as a barrier compared with other barriers, in both the frequent- and infrequent-attendance groups (p < 0.05), with the exception of the timing of the meeting in the infrequent-attendance group. More than 80% of attendees found the frequency and duration of the teleconference were sufficient. Utilizing Spanish as the primary language was reported to enhance the recommendations by 93% of the attendees. Moreover, 84% reported that the recommendations (almost) always fit the local circumstances. Furthermore, 99% of attendees found the teleconference provided a continuing medical education opportunity. Finally, 96% of attendees (almost) always found that the provided recommendations helped to improve the outcomes/quality of life of the patients. CONCLUSIONS: The LATB teleconference provided a valuable tool for the management of pediatric brain tumors in Latin America as it provided a feasible and easy to access continued medical education opportunity for the participants.

Published
2019

13. Patients with Retinoblastoma and chromosome 13q deletions have increased chemotherapy-related toxicities

Author

Brennan, RC, Qaddoumi, I, Billups, CA, Kaluzny, T, Furman, WL, and Wilson, MW

Subjects
Adult, Male, Adolescent, Chromosomes, Human, Pair 13, Retinal Neoplasms, Retinoblastoma, Infant, Chromosome Disorders, Middle Aged, Article, Child, Preschool, Antineoplastic Combined Chemotherapy Protocols, Humans, Female, Chromosome Deletion, Child, Aged
Abstract

A total of 5-10% of patients with retinoblastoma (RB) harbor deletion of the long arm (q) chromosome 13 (13q-). The treatment-related toxicities in this population have not been described.Sixty-eight RB patients on a single institutional protocol (RET5) from 2005 to 2010 were reviewed. Genetic screening identified 11 patients (seven female) with 13q-. Patients with early (Reese-Ellsworth [R-E] group I-III) disease (6/23 with 13q-) received eight courses of vincristine/carboplatin (VC). Patients with advanced (R-E group IV-V) bilateral disease (2/27 with 13q-) received two courses of vincristine/topotecan (VT) followed by nine courses of alternating VT/VC. Patients undergoing upfront enucleation received histopathology-based chemotherapy: intermediate risk (2/8 with 13q-) or high risk (1/10 with 13q-). Dose reductions were mandated for7 day delay in two consecutive courses following hematologic toxicity. Grades 3 and 4 hematologic, infectious, and gastrointestinal toxicities were compared between RET5 patients with and without 13q-.Demographics were similar between groups. When present, prolonged neutropenia (median 7 days, range 0-14 days) delayed chemotherapy and resulted in more frequent dose reductions among 13q- patients (5/11) than non-13q- patients (4/57) (P0.01). GI toxicity was similar between groups (5/11 13q- vs. 13/57 non-13q-; P = 0.14), but halted chemotherapy in one 13q- patient. Infectious complications and disease outcomes were similar between groups. At follow-up, all patients are alive (median 6.1 years, range 7.6 months-9.5 years).13q- RB patients had a higher incidence of neutropenia requiring chemotherapy dose reductions, but did not have increased treatment failure.

Published
2016

21. CLINICAL TRIALS

Author

Tippelt, S., primary, Mikasch, R., additional, Warmuth-Metz, M., additional, Pietsch, T., additional, Hilger, R. A., additional, Kwiecien, R., additional, Faldum, A., additional, Rutkowski, S., additional, Bode, U., additional, Siegler, N., additional, Fleischhack, G., additional, Dufour, C., additional, Delisle, M.-B., additional, Geoffray, A., additional, Laplanche, A., additional, Frappaz, D., additional, Icher, C., additional, Bertozzi, A.-I., additional, Leblond, P., additional, Doz, F., additional, Andre, N., additional, Schneider, P., additional, De Carli, E., additional, Berger, C., additional, Lejars, O., additional, Chastagner, P., additional, Soler, C., additional, Entz-Werle, N., additional, Valteau-Couanet, D., additional, Burzynski, S., additional, Janicki, T., additional, Burzynski, G., additional, Marszalek, A., additional, Deiss, A., additional, Korshunov, A., additional, Capper, D., additional, Witt, H., additional, van Tilburg, C., additional, von Deimling, A., additional, Kulozik, A. E., additional, Pfister, S. M., additional, Witt, O., additional, Milde, T., additional, Dhall, G., additional, Haley, K., additional, Finlay, J., additional, Rushing, T., additional, Sposto, R., additional, Seeger, R., additional, Lulla, R. R., additional, Goldman, S., additional, Beattie, C., additional, DasGupta, T. K., additional, Pollack, I., additional, Fisher, P. G., additional, Wu, S., additional, Boyett, J. M., additional, Fouladi, M., additional, Meijer, L., additional, Veal, G., additional, Walker, D., additional, Grundy, R., additional, Konczalik, W., additional, Ivanov, D., additional, Garnett, M., additional, Parker, T., additional, Kearns, P., additional, Rahman, R., additional, Smith, S., additional, Kimpo, M., additional, Yan, B., additional, Ning, C., additional, Villegas, M., additional, Alcasabas, A. P., additional, Juh, Y. E., additional, Chong, Q. T., additional, Lin, T. P., additional, Dewire, M., additional, Drissi, R., additional, Chow, L., additional, Pai, A., additional, Leach, J., additional, Lane, A., additional, Backus, L., additional, Grimme, L., additional, Tabares, J., additional, Kumar, S., additional, Sobo, M., additional, Hummel, T. R., additional, Alharbi, M., additional, Abdullah, S., additional, Alharbi, Q., additional, Alshahrani, M., additional, Mosleh, O., additional, Balbaid, A., additional, Alkofide, A., additional, Alkhayat, N., additional, AlFar, K., additional, Banyhamdan, A., additional, Ahmed, O., additional, El-Badawy, S., additional, Bouffet, E., additional, Jiang, M.-w., additional, Zhou, R.-h., additional, Zhou, Q., additional, Yuan, X.-j., additional, Ma, J., additional, Turner, D., additional, Wright, K., additional, Broniscer, A., additional, Robinson, G., additional, Qaddoumi, I., additional, Armstrong, G., additional, Gajjar, A., additional, Stewart, C., additional, Misra, S. N., additional, Misra, A. K., additional, Michalski, A., additional, and Stiller, C., additional

Published
2014
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22. EPENDYMOMA

Author

Hoffman, L. M., primary, Donson, A. M., additional, Nakachi, I., additional, Griesinger, A. M., additional, Birks, D. K., additional, Amani, V., additional, Hemenway, M. S., additional, Liu, A. K., additional, Wang, M., additional, Hankinson, T. C., additional, Handler, M. H., additional, Foreman, N. K., additional, Zakrzewska, M., additional, Zakrzewski, K., additional, Fendler, W., additional, Stefanczyk, L., additional, Liberski, P. P., additional, Massimino, M., additional, Gandola, L., additional, Ferroli, P., additional, Valentini, L., additional, Biassoni, V., additional, Garre, M. L., additional, Sardi, I., additional, Genitori, L., additional, Giussani, C., additional, Massimi, L., additional, Bertin, D., additional, Mussano, A., additional, Viscardi, E., additional, Modena, P., additional, Mastronuzzi, A., additional, Barra, S., additional, Scarzello, G., additional, Cinalli, G., additional, Peretta, P., additional, Giangaspero, F., additional, Boschetti, L., additional, Schiavello, E., additional, Calareso, G., additional, Antonelli, M., additional, Pecori, E., additional, Di Meco, F., additional, Migliorati, R., additional, Taborelli, A., additional, Witt, H., additional, Sill, M., additional, Wani, K., additional, Mack, S. C., additional, Capper, D., additional, Pajtler, K., additional, Lambert, S., additional, Tzaridis, T., additional, Milde, T., additional, Northcott, P. A., additional, Kulozik, A. E., additional, Witt, O., additional, Collins, V. P., additional, Ellison, D. W., additional, Taylor, M. D., additional, Kool, M., additional, Jones, D. T. W., additional, Korshunov, A., additional, Ken, A., additional, Pfister, S. M., additional, Makino, K., additional, Nakamura, H., additional, Kuroda, J.-i., additional, Kuratsu, J.-i., additional, Toledano, H., additional, Margolin, Y., additional, Ohali, A., additional, Michowiz, S., additional, Johann, P., additional, Tabori, U., additional, Walker, E., additional, Hawkins, C., additional, Taylor, M., additional, Yaniv, I., additional, Avigad, S., additional, Hoffman, L., additional, Plimpton, S. R., additional, Stence, N. V., additional, Vibhakar, R., additional, Lourdusamy, A., additional, Rahman, R., additional, Ward, J., additional, Rogers, H., additional, Grundy, R., additional, Punchihewa, C., additional, Lee, R., additional, Lin, T., additional, Orisme, W., additional, Dalton, J., additional, Aronica, E., additional, Smith, A., additional, Gajjar, A., additional, Onar, A., additional, Pounds, S., additional, Tatevossian, R., additional, Merchant, T., additional, Ellison, D., additional, Parker, M., additional, Mohankumar, K., additional, Weinlich, R., additional, Phoenix, T., additional, Thiruvenkatam, R., additional, White, E., additional, Gupta, K., additional, Boop, F., additional, Ding, L., additional, Mardis, E., additional, Wilson, R., additional, Downing, J., additional, Gilbertson, R., additional, Speed, D., additional, Gould, T., additional, Consortium, t. I. E., additional, Hoffman, L. M., additional, Griesinger, A., additional, Donson, A., additional, Birks, D., additional, Ohe, N., additional, Yano, H., additional, Nakayama, N., additional, Iwama, T., additional, Wright, K., additional, Hassall, T., additional, Bowers, D. C., additional, Crawford, J., additional, Bendel, A., additional, Fisher, P. G., additional, Klimo, P., additional, Armstrong, G., additional, Qaddoumi, I., additional, Robinson, G., additional, Wetmore, C., additional, Broniscer, A., additional, Chapman, R., additional, Mayne, C., additional, Duane, H., additional, Kilday, J.-P., additional, Coyle, B., additional, Graul-Conroy, A., additional, Hartsell, W., additional, Bragg, T., additional, Goldman, S., additional, Rebsamen, S., additional, Puccetti, D., additional, Salamat, S., additional, Patel, N. J., additional, Gomi, A., additional, Oguma, H., additional, Hayase, T., additional, Kawahara, Y., additional, Yagi, M., additional, Morimoto, A., additional, Wilbur, C., additional, Dunham, C., additional, Mabbott, D., additional, Carret, A.-S., additional, Lafay-Cousin, L., additional, McNeely, P. D., additional, Eisenstat, D., additional, Wilson, B., additional, Johnston, D., additional, Hukin, J., additional, Mynarek, M., additional, Kortmann, R. D., additional, Kaatsch, P., additional, Pietsch, T., additional, Timmermann, B., additional, Fleischhack, G., additional, Benesch, M., additional, Friedrich, C., additional, von Bueren, A. O., additional, Gerber, N. U., additional, Muller, K., additional, Tippelt, S., additional, Warmuth-Metz, M., additional, Rutkowski, S., additional, von Hoff, K., additional, Murugesan, M. K., additional, Poppleton, H., additional, Currle, S., additional, Kranenburg, T., additional, Eden, C., additional, Boulos, N., additional, Dapper, J., additional, Patel, Y., additional, Freeman, B., additional, Shelat, A., additional, Stewart, C., additional, Guy, R., additional, Adamski, J., additional, Huang, A., additional, Bartels, U., additional, Ramaswamy, V., additional, Krishnatry, R., additional, Laperriere, N., additional, Bouffet, E., additional, Araki, A., additional, Chocholous, M., additional, Gojo, J., additional, Dorfer, C., additional, Czech, T., additional, Dieckmann, K., additional, Slavc, I., additional, Haberler, C., additional, Doerner, E., additional, Muehlen, A. z., additional, Kortmann, R., additional, von Buehren, A., additional, Ottensmeier, H., additional, Resch, A., additional, Kwiecien, R., additional, Faldum, A., additional, Kuehl, J., additional, Sabnis, D., additional, Storer, L., additional, Simmonds, L., additional, Blackburn, S., additional, Lowe, J., additional, Kerr, I., additional, Wohlers, I., additional, Goschzik, T., additional, Dreschmann, V., additional, Denkhaus, D., additional, Rahmann, S., additional, Klein-Hitpass, L., additional, Iglesias, M. J. L., additional, Riet, F. G., additional, Dhermain, F. D., additional, Canale, S., additional, Dufour, C., additional, Rose, C. S., additional, Puget, S., additional, Grill, J., additional, Bolle, S., additional, Parkes, J., additional, Davidson, A., additional, Figaji, A., additional, Pillay, K., additional, Kilborn, T., additional, Padayachy, L., additional, Hendricks, M., additional, Van Eyssen, A., additional, Piccinin, E., additional, Lorenzetto, E., additional, Brenca, M., additional, Aldape, K., additional, Cho, Y.-J., additional, Weiss, W., additional, Phillips, J., additional, Jabado, N., additional, Mora, J., additional, Fan, X., additional, Jung, S., additional, Lee, J. Y., additional, Zitterbart, K., additional, French, P., additional, Kros, J. M., additional, Hauser, P., additional, Faria, C., additional, and Pfister, S., additional

Published
2014
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23. Feasibility and Efficacy of a Computer-Based Intervention Aimed at Preventing Reading Decoding Deficits Among Children Undergoing Active Treatment for Medulloblastoma: Results of a Randomized Trial

Author

Palmer, S. L., primary, Leigh, L., additional, Ellison, S. C., additional, Onar-Thomas, A., additional, Wu, S., additional, Qaddoumi, I., additional, Armstrong, G. T., additional, Wright, K., additional, Wetmore, C., additional, Broniscer, A., additional, and Gajjar, A., additional

Published
2013
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24. Abstracts from the 2012 BNOS Conference

Author

Birks, S., primary, Altinkaya, M., additional, Altinkaya, A., additional, Pilkington, G., additional, Kurian, K. M., additional, Crosby, C., additional, Hopkins, K., additional, Williams, M., additional, Donovan, L., additional, Birks, S., additional, Eason, A., additional, Bosak, V., additional, Holliday, J., additional, Corbett, I., additional, Keeling, M., additional, Bambrough, J., additional, Simpson, J., additional, Higgins, S., additional, Dogra, H., additional, Zhang, Y., additional, Bradley, M., additional, Schmidberger, C., additional, Hafizi, S., additional, Noorani, I., additional, Price, S., additional, Dubocq, A., additional, Jaunky, T., additional, Chatelain, C., additional, Evans, L., additional, Gaissmaier, T., additional, Pilkington, G. J., additional, An, Q., additional, Hurwitz, V., additional, Logan, J., additional, Bhangoo, R., additional, Ashkan, K., additional, Gullan, A., additional, Beaney, R., additional, Brazil, L., additional, Kokkinos, S., additional, Blake, R., additional, Singleton, A., additional, Shaw, A., additional, Iyer, V., additional, Jeyapalan, J. N., additional, Morley, I. C., additional, Hill, A. A., additional, Mumin, M. A., additional, Tatevossian, R. G., additional, Qaddoumi, I., additional, Ellison, D. W., additional, Sheer, D., additional, Frary, A., additional, Jefferies, S., additional, Harris, F., additional, Burnet, N., additional, Jena, R., additional, Watts, C., additional, Haylock, B., additional, Leow-Dyke, S., additional, Rathi, N., additional, Wong, H., additional, Dunn, J., additional, Baborie, A., additional, Crooks, D., additional, Husband, D., additional, Shenoy, A., additional, Brodbelt, A., additional, Walker, C., additional, Bahl, A., additional, Larsen, J., additional, Craven, I., additional, Metherall, P., additional, McKevitt, F., additional, Romanowski, C., additional, Hoggard, N., additional, Jellinek, D. A., additional, Bell, S., additional, Murray, E., additional, Muirhead, R., additional, James, A., additional, Hanzely, Z., additional, Jackson, R., additional, Stewart, W., additional, O'Brien, A., additional, Young, A., additional, Shepherd, S., additional, Cavers, D., additional, Wallace, L., additional, Hacking, B., additional, Scott, S., additional, Bowyer, D., additional, Elmahdi, A., additional, Frary, A. J., additional, O'Donovan, D. G., additional, Price, S. J., additional, Kia, A., additional, Przystal, J. M., additional, Nianiaris, N., additional, Mazarakis, N. D., additional, Mintz, P. J., additional, Hajitou, A., additional, Karakoula, K., additional, Phipps, K., additional, Harkness, W., additional, Hayward, R., additional, Thompson, D., additional, Jacques, T., additional, Harding, B., additional, Darling, J., additional, Warr, T., additional, Jenkinson, M., additional, Zhou, L., additional, Ercolano, E., additional, Ammoun, S., additional, Schmid, M. C., additional, Barczyk, M., additional, Hanemann, C. O., additional, Rowther, F., additional, Dawson, T., additional, Ashton, K., additional, Maherally, Z., additional, Hatherell, K. E., additional, Kroese, K., additional, Singh, P., additional, McQuaid, S., additional, Al-Rashid, S., additional, Prise, K., additional, Herron, B., additional, Healy, E., additional, Shoakazemi, A., additional, Donnelly, M., additional, McConnell, R., additional, Harney, J., additional, Conkey, D., additional, McGrath, E., additional, Lunsford, L., additional, Kondziolka, D., additional, Niranjan, A., additional, Kano, H., additional, Hamilton, R., additional, Flannery, T., additional, Majani, Y., additional, Smith, S., additional, Grundy, R., additional, Rahman, R., additional, Saini, S., additional, Hall, G., additional, Davis, C., additional, Lawson, T., additional, Potter, N., additional, Goessl, E., additional, Wilkins, S., additional, Smith, T., additional, Petinou, V., additional, Nicholl, I., additional, Singh, J., additional, Lea, R., additional, Welsby, P., additional, Spiteri, I., additional, Sottoriva, A., additional, Marko, N., additional, Tavare, S., additional, Collins, P., additional, Su, Z., additional, Gerhard, A., additional, Hinz, R., additional, Roncaroli, F., additional, Coope, D., additional, Thompson, G., additional, Karabatsou, K., additional, Sofat, A., additional, Leggate, J., additional, du Plessis, D., additional, Turkheimer, F., additional, Jackson, A., additional, Das, K., additional, Herholz, K., additional, Whittle, I. R., additional, Grundy, P., additional, Cruickshank, G., additional, Berry, V., additional, Elder, D., additional, Cohen, N., additional, Tavare, J., additional, Zilidis, G., additional, Tibarewal, P., additional, Spinelli, L., additional, Leslie, N. R., additional, Coope, D. J., additional, Green, S., additional, Wall, G., additional, Brennan, P., additional, Baily, J., additional, Diaz, M., additional, Ironside, J., additional, Sansom, O., additional, Brunton, V., additional, Frame, M., additional, Thomas, O., additional, Mohsen, L., additional, Lupson, V., additional, McLean, M., additional, Arora, M., additional, Shaw, L., additional, Lawrence, C., additional, Alder, J., additional, Rada, L., additional, Chen, K., additional, Shivane, A., additional, Parkinson, D., additional, Hanemann, C., additional, Pangeni, R. P., additional, Warr, T. J., additional, Morris, M. R., additional, Mackinnon, M., additional, Williamson, A., additional, Chalmers, A., additional, Beckett, V., additional, Joannides, A., additional, Brock, R., additional, McCarthy, K., additional, Singh, A., additional, Kardooni, H., additional, Morris, M., additional, Syed, N., additional, Janczar, K., additional, O'Neil, K., additional, Nigro, C. L., additional, Lattanzio, L., additional, Coley, H., additional, Hatzimichael, E., additional, Bomalaski, J., additional, Szlosarek, P., additional, Crook, T., additional, Pullen, N. A., additional, Anand, M., additional, Van Meter, T., additional, Williams, S., additional, Boissinot, M., additional, Steele, L., additional, Chiocca, E. A., additional, Lawler, S., additional, Al Rashid, S. T., additional, Mashal, S., additional, Taggart, L., additional, Clarke, E., additional, and Prise, K. M., additional

Published
2012
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27. HIGH GRADE GLIOMAS

Author

Leonard, A., primary, Wolff, J., additional, Sengupta, R., additional, Marassa, J., additional, Piwnica-Worms, D., additional, Rubin, J., additional, Pollack, I., additional, Jakacki, R., additional, Butterfield, L., additional, Okada, H., additional, Fangusaro, J., additional, Warren, K. E., additional, Mullins, C., additional, Jurgen, P., additional, Julia, S., additional, Friedrich, C. C., additional, Keir, S., additional, Saling, J., additional, Roskoski, M., additional, Friedman, H., additional, Bigner, D., additional, Moertel, C., additional, Olin, M., additional, Dahlheimer, T., additional, Gustafson, M., additional, Sumstad, D., additional, McKenna, D., additional, Low, W., additional, Nascene, D., additional, Dietz, A., additional, Ohlfest, J., additional, Sturm, D., additional, Witt, H., additional, Hovestadt, V., additional, Quan, D. A. K., additional, Jones, D. T. W., additional, Konermann, C., additional, Pfaff, E., additional, Korshunov, A., additional, Rizhova, M., additional, Milde, T., additional, Witt, O., additional, Zapatka, M., additional, Collins, V. P., additional, Kool, M., additional, Reifenberger, G., additional, Lichter, P., additional, Lindroth, A. M., additional, Plass, C., additional, Jabado, N., additional, Pfister, S. M., additional, Pizer, B., additional, Salehzadeh, A., additional, Brodbelt, A., additional, Mallucci, C., additional, Brassesco, M., additional, Pezuk, J., additional, Morales, A., additional, de Oliveira, J., additional, Roberto, G., additional, Umezawa, K., additional, Valera, E., additional, Rego, E., additional, Scrideli, C., additional, Tone, L., additional, Veringa, S. J. E., additional, Van Vuurden, D. G., additional, Wesseling, P., additional, Vandertop, W. P., additional, Noske, D. P., additional, Wurdinger, T., additional, Kaspers, G. J. L., additional, Hulleman, E., additional, Wright, K., additional, Broniscer, A., additional, Bendel, A., additional, Bowers, D., additional, Crawford, J., additional, Fisher, P., additional, Hassall, T., additional, Armstrong, G., additional, Baker, J., additional, Qaddoumi, I., additional, Robinson, G., additional, Wetmore, C., additional, Klimo, P., additional, Boop, F., additional, Onar-Thomas, A., additional, Ellison, D., additional, Gajjar, A., additional, Cruz, O., additional, de Torres, C., additional, Sunol, M., additional, Rodriguez, E., additional, Alonso, L., additional, Parareda, A., additional, Cardesa, T., additional, Salvador, H., additional, Celis, V., additional, Guillen, A., additional, Garcia, G., additional, Muchart, J., additional, Trampal, C., additional, Martin, M. L., additional, Rebollo, M., additional, Mora, J., additional, Piotrowski, A., additional, Kowalska, A., additional, Coyle, P., additional, Smith, S., additional, Rogers, H., additional, Macarthur, D., additional, Grundy, R., additional, Puccetti, D., additional, Salamat, S., additional, Kennedy, T., additional, Patel, N., additional, Bradley, K., additional, Casey, K., additional, Iskandar, B., additional, Nakano, Y., additional, Okada, K., additional, Osugi, Y., additional, Yamasaki, K., additional, Fujisaki, H., additional, Fukushima, H., additional, Inoue, T., additional, Matsusaka, Y., additional, Sakamoto, H., additional, Hara, J., additional, De Vleeschouwer, S., additional, Ardon, H., additional, Van Calenbergh, F., additional, Sciot, R., additional, Wilms, G., additional, Van Loon, J., additional, Goffin, J., additional, Van Gool, S., additional, Rusinak, D., additional, Knight, P., additional, Onel, K., additional, Wargowski, D., additional, Stettner, A., additional, Al-Ghafari, A., additional, Punjaruk, W., additional, Coyle, B., additional, Kerr, I., additional, Xipell, E., additional, Rodriguez, M., additional, Gonzalez-Huarriz, M., additional, Tunon, M. T., additional, Zazpe, I., additional, Tejada-Solis, S., additional, Diez-Valle, R., additional, Fueyo, J., additional, Gomez-Manzano, C., additional, Alonso, M. M., additional, Pastakia, D., additional, McCully, C., additional, Murphy, R., additional, Bacher, J., additional, Thomas, M., additional, Steffen-Smith, E., additional, Saleem, K., additional, Waldbridge, S., additional, Widemann, B., additional, Warren, K., additional, Miele, E., additional, Buttarelli, F., additional, Arcella, A., additional, Begalli, F., additional, Po, A., additional, Baldi, C., additional, Carissimo, G., additional, Antonelli, M., additional, Donofrio, V., additional, Morra, I., additional, Nozza, P., additional, Gulino, A., additional, Giangaspero, F., additional, Ferretti, E., additional, Elens, I., additional, Pauwels, F., additional, Fritzell, S., additional, Eberstal, S., additional, Sanden, E., additional, Visse, E., additional, Darabi, A., additional, Siesjo, P., additional, McDonald, P., additional, Wrogemann, J., additional, Krawitz, S., additional, Del Bigio, M., additional, Eisenstat, D., additional, Kwiecien, R., additional, Pietsch, T., additional, Faldum, A., additional, Kortmann, R.-D., additional, Warmuth-Metz, M., additional, Rutkowski, S., additional, Slavc, I., additional, Kramm, C. M., additional, Uparkar, U., additional, Geyer, R., additional, Ermoian, R., additional, Ellenbogen, R., additional, Leary, S., additional, Triscott, J., additional, Hu, K., additional, Fotovati, A., additional, Yip, S., additional, Kast, R., additional, Toyota, B., additional, Dunn, S., additional, Hegde, M., additional, Corder, A., additional, Chow, K., additional, Mukherjee, M., additional, Ashoori, A., additional, Brawley, V., additional, Heslop, H., additional, Gottschalk, S., additional, Yvon, E., additional, Ahmed, N., additional, Wong, T.-T., additional, Yang, F.-Y., additional, Lu, M., additional, Liang, H.-F., additional, Wang, H.-E., additional, Liu, R.-S., additional, Teng, M.-C., additional, Yen, C.-C., additional, Agnihotri, S., additional, Ternamian, C., additional, Jones, C., additional, Zadeh, G., additional, Rutka, J., additional, Hawkins, C., additional, Filipek, I., additional, Drogosiewicz, M., additional, Perek-Polnik, M., additional, Swieszkowska, E., additional, Baginska, B. D., additional, Jurkiewicz, E., additional, Perek, D., additional, Kuehn, A., additional, Falkenstein, F., additional, Gnekow, A., additional, Kramm, C., additional, Brooks, M. D., additional, Jackson, E., additional, Mitra, R. D., additional, Rubin, J. B., additional, Liu, X.-Y., additional, Schwartzentruber, J., additional, Fontebasso, A. M., additional, Quang, D.-A. K., additional, Albrecht, S., additional, Dong, Z., additional, Siegel, P., additional, Von Diemling, A., additional, Faury, D., additional, Tabori, U., additional, Majewski, J., additional, Lulla, R., additional, Echevarria, M., additional, Alden, T., additional, DiPatri, A., additional, Tomita, T., additional, Goldman, S., additional, Lin, T., additional, Merchant, T. E., additional, Kocak, M., additional, Panandiker, A. P., additional, Armstrong, G. T., additional, Gielen, G. H., additional, Muehlen, A. z., additional, Hubert, C., additional, Ding, Y., additional, Toledo, C., additional, Paddison, P., additional, Olson, J., additional, Nandhabalan, M., additional, Bjerke, L., additional, Bax, D., additional, Carvalho, D., additional, Bajrami, I., additional, Ashworth, A., additional, Lord, C., additional, Hargrave, D., additional, Reis, R., additional, Workman, P., additional, Little, S., additional, Popov, S., additional, Jury, A., additional, Burford, A., additional, Doey, L., additional, Al-Sarraj, S., additional, Jurgensmeier, J., additional, Chen, L., additional, Kozarewa, I., additional, Baker, S., additional, Perryman, L., additional, Box, G., additional, Raynaud, F., additional, Eccles, S., additional, Viana-Pereira, M., additional, Pereira, M., additional, Forshew, T., additional, Tatevossian, R., additional, Sheer, D., additional, Pimental, J., additional, Pires, M., additional, Sarkar, C., additional, Jha, P., additional, Patrick, I. R. P., additional, Somasundaram, K., additional, Pathak, P., additional, Sharma, M. C., additional, Suri, V., additional, Suri, A., additional, Gerges, N., additional, Haque, T., additional, Nantel, A., additional, Lee, C., additional, Chen, J., additional, Venugopal, C., additional, Singhal, A., additional, Dunham, C., additional, Kerr, J., additional, Verreault, M., additional, Wakimoto, H., additional, Jayanthan, A., additional, Narendran, A., additional, Singh, S., additional, Giraud, G., additional, Holm, S., additional, Gustavsson, B., additional, Kizyma, R., additional, Kizyma, Z., additional, Dvornyak, L., additional, Kotsay, B., additional, Epari, S., additional, Sharma, P., additional, Gurav, M., additional, Gupta, T., additional, Shetty, P., additional, Moiyadi, A., additional, Kane, S., additional, and Jalali, R., additional

Published
2012
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28. EPIDEMIOLOGY

Author

Khatua, S., primary, Brown, R., additional, Pearlman, M., additional, Vats, T., additional, Satge, D., additional, Stiller, C., additional, Rutkowski, S., additional, von Bueren, A. O., additional, Lacour, B., additional, Sommelet, D., additional, Nishi, M., additional, Massimino, M., additional, Garre, M.-L., additional, Moreno, F., additional, Hasle, H., additional, Jakab, Z., additional, Greenberg, M., additional, von der Weid, N., additional, Kuehni, C., additional, Zurriaga, O., additional, Vicente, M.-L., additional, Peris-Bonet, R., additional, Benesch, M., additional, Vekemans, M., additional, Sullivan, S., additional, Rickert, C., additional, Fisher, P. G., additional, Von Behren, J., additional, Nelson, D. O., additional, Reynolds, P., additional, Fukuoka, K., additional, Yanagisawa, T., additional, Suzuki, T., additional, Koga, T., additional, Wakiya, K., additional, Adachi, J.-i., additional, Mishima, K., additional, Fujimaki, T., additional, Matsutani, M., additional, Nishikawa, R., additional, Gidding, C., additional, Schieving, J., additional, Wesseling, P., additional, Ligtenberg, M., additional, Hoogerbrugge, N., additional, Jongmans, M., additional, Crosier, S., additional, Nicholson, S. L., additional, Robson, K., additional, Jacques, T., additional, Wharton, S., additional, Bown, N., additional, Michalski, A., additional, Pizer, B., additional, Clifford, S., additional, Sanden, E., additional, Visse, E., additional, Siesjo, P., additional, Darabi, A., additional, Nousome, D., additional, Lupo, P. J., additional, Scheurer, M. E., additional, Nulman, I., additional, Barrera, M., additional, Maxwell, C., additional, Koren, G., additional, Gorelyshev, S., additional, Matuev, K., additional, Lubnin, A., additional, Laskov, M., additional, Lemeneva, N., additional, Mazerkina, N., additional, Khuhlaeva, E., additional, Muller, K., additional, Bruns, F., additional, Pietsch, T., additional, Kortmann, R.-D., additional, Krishnatry, R., additional, Shirsat, N., additional, Kunder, R., additional, Epari, S., additional, Gupta, T., additional, Kurkure, P., additional, Vora, T., additional, Arora, B., additional, Moiyadi, A., additional, Jalali, R., additional, Swieszkowska, E., additional, Dembowska-Baginska, B., additional, Drogosiewicz, M., additional, Filipek, I., additional, Perek-Polnik, M., additional, Grajkowska, W., additional, Perek, D., additional, Johnston, D., additional, Cyr, J., additional, Strother, D., additional, Lafay-Cousin, L., additional, Fryer, C., additional, Scheinemann, K., additional, Carret, A.-S., additional, Fleming, A., additional, Larouche, V., additional, Bouffet, E., additional, Friedrich, C., additional, Gnekow, A. K., additional, Fleischhack, G., additional, Kramm, C. M., additional, Fruehwald, M. C., additional, Muller, H. L., additional, Calaminus, G., additional, Kordes, U., additional, Faldum, A., additional, Warmuth-Metz, M., additional, Kortmann, R. D., additional, Jung, I., additional, Kaatsch, P., additional, Caretti, V., additional, Bugiani, M., additional, Boor, I., additional, Schellen, P., additional, Vandertop, W. P., additional, Noske, D. P., additional, Kaspers, G., additional, Wurdinger, T., additional, Robinson, G., additional, Chingtagumpala, M., additional, Adesina, A., additional, Dalton, J., additional, Santi, M., additional, Sievert, A., additional, Wright, K., additional, Armstrong, G., additional, Boue, D., additional, Olshefski, R., additional, Scott, S., additional, Huang, A., additional, Cohn, R., additional, Gururangan, S., additional, Bowers, D., additional, Gilbertson, R., additional, Gajjar, A., additional, Ellison, D., additional, Chick, E., additional, Donson, A., additional, Owens, E., additional, Smith, A. A., additional, Madden, J. R., additional, Foreman, N. K., additional, Bakry, D., additional, Aronson, M., additional, Durno, C., additional, Hala, R., additional, Farah, R., additional, Amayiri, N., additional, Alharbi, Q., additional, Shamvil, A., additional, Ben-Shachar, S., additional, Constantini, S., additional, Rina, D., additional, Ellise, J., additional, Keiles, S., additional, Pollet, A., additional, Qaddoumi, I., additional, Gallinger, S., additional, Malkin, D., additional, Hawkins, C., additional, Tabori, U., additional, Trivedi, M., additional, Goodden, J., additional, Chumas, P., additional, Tyagi, A., additional, O'kane, R., additional, O'Kane, R., additional, Crimmins, D., additional, Picton, S., additional, and Elliott, M., additional

Published
2012
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29. PEDIATRICS CLINICAL RESEARCH

Author

Murray, J. C., primary, Rainusso, N., additional, Roberts, R. A., additional, Gomez, A. M., additional, Egler, R., additional, Russell, H., additional, Okcu, M. F., additional, Gururangan, S., additional, Fangusaro, J., additional, Young-Poussaint, T., additional, Lesh, S., additional, Onar, A., additional, Gilbertson, R., additional, Packer, R., additional, McLendon, R., additional, Friedman, H. S., additional, Boyett, J., additional, Kun, L. E., additional, Venkatramani, R., additional, Haley, K., additional, Gilles, F., additional, Sposto, R., additional, Ji, L., additional, Olshefski, R., additional, Garvin, J., additional, Tekautz, T., additional, Kennedy, G., additional, Rassekh, R., additional, Moore, T., additional, Gardner, S., additional, Allen, J., additional, Shore, R., additional, Moertel, C., additional, Atlas, M., additional, Lasky, J., additional, Finlay, J., additional, Valera, E. T., additional, Brassesco, M. S., additional, Scrideli, C. A., additional, Oliveira, R. S., additional, Machado, H. R., additional, Tone, L. G., additional, Finlay, J. L., additional, Kreimer, S., additional, Dagri, J., additional, Grimm, J., additional, Bluml, S., additional, Britt, B., additional, Dhall, G., additional, Brown, R. J., additional, Shah, A., additional, Kapoor, N., additional, Abdel-Azim, H., additional, Rao, A. A. N., additional, Wallace, D., additional, Gajjar, A., additional, Packer, R. J., additional, Pearlman, M. L., additional, Sadighi, Z., additional, Bingham, R., additional, Vats, T., additional, Khatua, S., additional, Ko, R. H., additional, O'Neil, S., additional, Lavey, R. S., additional, Davidson, T. B., additional, Tovar, J., additional, Wong, K., additional, Olch, A., additional, Murray, J. C., additional, Honeycutt, J. H., additional, Donahue, D. J., additional, Head, H. W., additional, Alles, A. J., additional, Ray, A., additional, Pearlman, M., additional, Baskin, J., additional, Qaddoumi, I., additional, Ahchu, M. S., additional, Alabi, S. F., additional, Arambu, I. C., additional, Castellanos, M., additional, Gamboa, Y., additional, Martinez, R., additional, Montero, M., additional, Ocampo, E., additional, Howard, S. C., additional, Broniscer, A., additional, Baker, S. D., additional, Baker, J. N., additional, Panandiker, A. P., additional, Onar-Thomas, A., additional, Chin, T. K., additional, Merchant, T. E., additional, Davidoff, A., additional, Kaste, S. C., additional, Stewart, C. F., additional, Espinoza, J., additional, Patel, N., additional, Jeffrey, A., additional, Torkildson, J., additional, Cornelius, A., additional, Bedros, A., additional, Etzl, M., additional, Pradhan, K., additional, Corbett, R., additional, Sullivan, M., additional, McGowage, G., additional, Puccetti, D., additional, Stein, D., additional, Jasty, R., additional, Antony, R., additional, Patel, M., additional, Wong, K. E., additional, Krieger, M., additional, McComb, G., additional, Sanchez-Lara, P. A., additional, Randolph, L. M., additional, Krieger, M. D., additional, Wu, S., additional, Panigrahy, A., additional, Shimada, H., additional, Erdreich-Epstein, A., additional, Puccetti, D. M., additional, Kennedy, T., additional, Salamat, S., additional, Bradfield, Y., additional, Park, H. J., additional, Yoon, J. H., additional, Ahn, H. S., additional, Shin, H. Y., additional, Kim, S. K., additional, Im, H. J., additional, Ra, Y. S., additional, Won, S. C., additional, Baek, H. J., additional, Sung, K. W., additional, Hah, J. O., additional, Lim, Y. T., additional, Lee, G. S., additional, Lee, Y. H., additional, Kim, H. S., additional, Park, J. K., additional, Kim, M. K., additional, Park, J. E., additional, Chung, N. G., additional, Choi, H. S., additional, Campen, C. J., additional, Fisher, P. G., additional, Ruge, M. I., additional, Simon, T., additional, Suchorska, B., additional, Lehrke, R., additional, Hamisch, C., additional, Koerber, F., additional, Treuer, H., additional, Berthold, F., additional, Sturm, V., additional, Voges, J., additional, Kirsch, M., additional, Lindner, C., additional, and Schackert, G., additional

Published
2011
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33. Dysembryoplastic neuroepithelial tumors and cognitive outcome: cure at a price?

Author

Qaddoumi I, Ellison DW, Morris EB, Broniscer A, Boop F, Merchant T, Palmer SL, Gajjar A, Qaddoumi, Ibrahim, Ellison, David W, Morris, E Brannon, Broniscer, Alberto, Boop, Frederick, Merchant, Thomas, Palmer, Shawna L, and Gajjar, Amar

Abstract

Background: Dysembryoplastic neuroepithelial tumors (DNETs) are benign glioneuronal tumors that occur in children. These tumors are characterized by seizures, lack of neurologic deficits, and a seemingly benign course after resection.Methods: A retrospective review was conducted of data relating to 11 children diagnosed with DNETs between January 1988 and December 2007 at St. Jude Children's Research Hospital. This report documented the clinical features, neurocognitive function, and treatment outcomes in this institutional series.Results: The patient cohort included 8 boys and 3 girls (median age at diagnosis, 10 years); all patients presented with seizures: 4 complex partial, 3 generalized tonic-clonic, 2 absence, 1 partial simple, and 1 not classified. Of the 11 patients, 1 died of cardiac fibrosis, and tumors recurred or progressed in 4 (36%) patients. Seizure control was achieved in all patients but 1. Of the 9 patients who completed neuropsychologic testing, only 3 (33%) functioned at or above the expected level of same-age peers.Conclusions: The high recurrence and progression rates of DNETs and the high rate of abnormal neurocognitive test results noted in the current study highlight the need for regular follow-up and appropriate academic counseling of children with these tumors. [ABSTRACT FROM AUTHOR]

Published
2010
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34. Outcome and prognostic features in pediatric gliomas: a review of 6212 cases from the Surveillance, Epidemiology, and End Results database.

Author

Qaddoumi I, Sultan I, Gajjar A, Qaddoumi, Ibrahim, Sultan, Iyad, and Gajjar, Amar

Abstract

Background: Pediatric gliomas are rare and heterogeneous tumors. The Surveillance, Epidemiology, and End Results (SEER) database allows a large-scale analysis of the clinical characteristics and prognostic features of these tumors.Methods: The authors analyzed available SEER data on 6212 patients younger than 20 years at diagnosis of glioma (1973-2005), according to 4 age categories: <1 year, 1-3 years, 3-5 years, and 5-20 years.Results: The overall 5- and 10-year survival estimates were 71%+/-0.62% (standard error) and 68%+/-0.67%, respectively. Forty-one percent of gliomas were cerebral; the frequency of cerebellar tumors (22%-32% of gliomas) increased sharply after the first year of life. Of the tumors for which grade was available, 77% were low grade (grade I or II). Tumor grade emerged as the most significant independent prognostic factor in all age groups except the youngest age group, in which extent of resection was most significant. Surgery other than gross total resection was an adverse prognostic factor (hazard ratio, 2.18; 95% confidence interval, 1.78-2.67). Age<3 years predicted a greater likelihood of survival in patients with high-grade gliomas and brainstem tumors. Conversely, age<3 years predicted a lower likelihood of survival in patients with low-grade gliomas. Children aged<1 year received less radiotherapy than older patients (P<.0001) and were less likely to undergo gross total resection (P<.0001).Conclusions: The survival of children with gliomas is influenced by histologic subtype, age, and extent of resection. Despite its limitations, the SEER database provides a useful tool for studies of rare tumors such as pediatric gliomas. [ABSTRACT FROM AUTHOR]

Published
2009
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37. A Proposal for Future Modifications on Clinical TNM Staging System of Retinoblastoma Based on the American Joint Committee on Cancer Staging Manual, 7 th and 8 th Editions

Author

Yacoub A. Yousef, Ibrahim Qaddoumi, Ibrahim Al-Nawaiseh, Mona Mohammad, Dalia AlRimawi, Mario Damiano Toro, Sandrine Zweifel, Robert Rejdak, Rashed Nazzal, Mustafa Mehyar, Imad Jaradat, Iyad Sultan, Maysa Al-Hussaini, Yousef, Ya, Qaddoumi, I, Al-Nawaiseh, I, Mohammad, M, Alrimawi, D, Toro, Md, Zweifel, S, Rejdak, R, Nazzal, R, Mehyar, M, Jaradat, I, Sultan, I, and Al-Hussaini, M

Subjects
Oncology
Published
2022

38. Therapeutic and Prognostic Implications of BRAF V600E in Pediatric Low-Grade Gliomas

Author

Didier Frappaz, Shiyang Wang, Matija Snuderl, Catriona Ling, Rahul Krishnatry, Romain Perbet, Elizabeth Finch, David Sumerauer, Alexandre Vasiljevic, Nataliya Zhukova, Annie Huang, A. T. Chan, Matthew Mistry, Zhi Feng Shi, Cecile Faure Conter, Adam Fleming, Jean Mulcahy-Levy, Nicholas K. Foreman, Matthias A. Karajannis, Ibrahim Qaddoumi, Vijay Ramaswamy, Amulya A. Nageswara Rao, Julie H. Harreld, Anne Sophie Carret, Roger J. Packer, Samantha Mascelli, Cheng-Ying Ho, Theodore Nicolaides, Eric Bouffet, Shayna Zelcer, David W. Ellison, Mark W. Kieran, Keith L. Ligon, Sarah Leary, Ute Bartels, Tara McKeown, Sabine Mueller, Maria Luisa Garrè, Scott Ryall, Bev Wilson, Peter B. Dirks, Michael D. Taylor, Peter Hauser, James T. Rutka, Lenka Krskova, Michal Zapotocky, Courtney A. Crane, Ho Keung Ng, Ofelia Cruz, Carmen de Torres, Ying Mao, Uri Tabori, Alvaro Lassaletta, Marion Honnorat, Anthony Arnoldo, Paolo Nozza, David D. Eisenstat, Valerie Larouche, Alessandro Raso, Shiyi Chen, Nada Jabado, Karen Silva, Ruth G. Tatevossian, Cynthia Hawkins, Ana Guerreiro Stucklin, Jim Loukides, Caterina Giannini, James Dalton, Lassaletta A., Zapotocky M., Mistry M., Ramaswamy V., Honnorat M., Krishnatry R., Stucklin A.G., Zhukova N., Arnoldo A., Ryall S., Ling C., McKeown T., Loukides J., Cruz O., De Torres C., Ho C.-Y., Packer R.J., Tatevossian R., Qaddoumi I., Harreld J.H., Dalton J.D., Mulcahy-Levy J., Foreman N., Karajannis M.A., Wang S., Snuderl M., Rao A.N., Giannini C., Kieran M., Ligon K.L., Garre M.L., Nozza P., Mascelli S., Raso A., Mueller S., Nicolaides T., Silva K., Perbet R., Vasiljevic A., Conter C.F., Frappaz D., Leary S., Crane C., Chan A., Ng H.-K., Shi Z.-F., Mao Y., Finch E., Eisenstat D., Wilson B., Carret A.S., Hauser P., Sumerauer D., Krskova L., Larouche V., Fleming A., Zelcer S., Jabado N., Rutka J.T., Dirks P., Taylor M.D., Chen S., Bartels U., Huang A., Ellison D.W., Bouffet E., Hawkins C., and Tabori U.

Subjects
Oncology, Male, Cancer Research, Pathology, medicine.medical_treatment, Pediatrics, Cohort Studies, 0302 clinical medicine, CDKN2A, Brain Stem Neoplasms, Child, Brain Neoplasms, Glioma, Prognosis, 030220 oncology & carcinogenesis, Child, Preschool, Cohort, Female, Human, Cohort study, Proto-Oncogene Proteins B-raf, medicine.medical_specialty, Adolescent, Prognosi, Brain Neoplasm, 03 medical and health sciences, Internal medicine, Original Reports, medicine, Adjuvant therapy, Humans, Diencephalon, Preschool, neoplasms, Brain Stem Neoplasm, Chemotherapy, business.industry, Infant, medicine.disease, digestive system diseases, BRAF V600E, Mutation, Cohort Studie, Neoplasm Grading, business, human activities, 030217 neurology & neurosurgery, Progressive disease
Abstract

Purpose BRAF V600E is a potentially highly targetable mutation detected in a subset of pediatric low-grade gliomas (PLGGs). Its biologic and clinical effect within this diverse group of tumors remains unknown. Patients and Methods A combined clinical and genetic institutional study of patients with PLGGs with long-term follow-up was performed (N = 510). Clinical and treatment data of patients with BRAF V600E mutated PLGG (n = 99) were compared with a large international independent cohort of patients with BRAF V600E mutated-PLGG (n = 180). Results BRAF V600E mutation was detected in 69 of 405 patients (17%) with PLGG across a broad spectrum of histologies and sites, including midline locations, which are not often routinely biopsied in clinical practice. Patients with BRAF V600E PLGG exhibited poor outcomes after chemotherapy and radiation therapies that resulted in a 10-year progression-free survival of 27% (95% CI, 12.1% to 41.9%) and 60.2% (95% CI, 53.3% to 67.1%) for BRAF V600E and wild-type PLGG, respectively ( P < .001). Additional multivariable clinical and molecular stratification revealed that the extent of resection and CDKN2A deletion contributed independently to poor outcome in BRAF V600E PLGG. A similar independent role for CDKN2A and resection on outcome were observed in the independent cohort. Quantitative imaging analysis revealed progressive disease and a lack of response to conventional chemotherapy in most patients with BRAF V600E PLGG. Conclusion BRAF V600E PLGG constitutes a distinct entity with poor prognosis when treated with current adjuvant therapy.

Published
2017

40. Development of the pediatric neuro-oncology services assessment aid: An assessment tool for pediatric neuro-oncology service delivery capacity.

Author

Rajagopal R, Diaz Coronado R, Hamid SA, Navarro Martin Del Campo R, Boop F, Bag A, Benito Reséndiz AE, Bhat K V, Campos D, Chang K, Cirt R, Dhyani Rahmartani L, Foo JC, Hoveyan J, Lucas JT Jr, Ngcana T, Ul Ain R, Omran N, Osorio DS, Qureshi BM, Sabin ND, Schandorf E, Bankah P, Dadzie MA, Gbadamos H, Sharafeldin H, Somathilaka M, Yang P, Atteby Jean-Jacques Y, Zhang A, Salman Z, Gonzalez M, Friedrich P, Rodriguez-Galindo C, Qaddoumi I, and Moreira DC

Abstract

Background: To enhance the quality of care available for children with central nervous system (CNS) tumors across the world, a systematic evaluation of capacity is needed to identify gaps and prioritize interventions. To that end, we created the pediatric neuro-oncology (PNO) resource assessment aid (PANORAMA) tool., Methods: The development of PANORAMA encompassed 3 phases: operationalization, consensus building, and piloting. PANORAMA aimed to capture the elements of the PNO care continuum through domains with weighted assessments reflecting their importance. Responses were ordinally scored to reflect the level of satisfaction. PANORAMA was revised based on feedback at various phases to improve its relevance, usability, and clarity., Results: The operationalization phase identified 14 domains by using 252 questions. The consensus phase involved 15 experts (6 pediatric oncologists, 3 radiation oncologists, 2 neurosurgeons, 2 radiologists, and 2 pathologists). The consensus phase validated the identified domains, questions, and scoring methodology. The PANORAMA domains included national context, hospital infrastructure, organization and service integration, human resources, financing, laboratory, neurosurgery, diagnostic imaging, pathology, chemotherapy, radiotherapy, supportive care, and patient outcomes. PANORAMA was piloted at 13 institutions in 12 countries, representing diverse patient care contexts. Face validity was assessed by examining the correlation between the estimated score by respondents and calculated PANORAMA scores for each domain ( r  = 0.67, P  < .0001)., Conclusions: PANORAMA was developed through a systematic, collaborative approach, ensuring its relevance to evaluate core elements of PNO service capacity. Distribution of PANORAMA will enable quantitative service evaluations across institutions, facilitating benchmarking and the prioritization of interventions., Competing Interests: The authors declare no competing interests., (© The Author(s) 2024. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)

Published
2024
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41. Comprehensive analysis of MYB/MYBL1-altered pediatric-type diffuse low-grade glioma.

Author

Moreira DC, Qaddoumi I, Spiller S, Bouldin TW, Davidson A, Saba-Silva N, Sullivan DV, Tanaka R, Wagner AS, Wood M, Klimo P, Job G, Devidas M, Li X, Gajjar A, Robinson GW, and Chiang J

Subjects
Child, Child, Preschool, Female, Humans, Male, Biomarkers, Tumor genetics, Follow-Up Studies, Neoplasm Grading, Prognosis, Proto-Oncogene Proteins, Retrospective Studies, Survival Rate, Trans-Activators genetics, Brain Neoplasms pathology, Brain Neoplasms genetics, Glioma pathology, Glioma genetics, Proto-Oncogene Proteins c-myb genetics
Abstract

Background: Pediatric-type diffuse low-grade gliomas (pLGG) harboring recurrent genetic alterations involving MYB or MYBL1 are closely related tumors. Detailed treatment and outcome data of large cohorts are still limited. This study aimed to comprehensively evaluate pLGG with these alterations to define optimal therapeutic strategies., Methods: We retrospectively reviewed details of pLGG with MYB or MYBL1 alterations from patients treated or referred for pathologic review at St. Jude Children's Research Hospital. Tumor specimens were centrally reviewed, and clinical data were collated., Results: Thirty-three patients (18 male; median age, 5 years) were identified. Two tumors had MYBL1 alterations; 31 had MYB alterations, MYB::QKI fusion being the most common (n = 10, 30%). Most tumors were in the cerebral hemispheres (n = 22, 67%). Two patients (6%) had metastasis at diagnosis. The median follow-up was 6.1 years. The 5-year event-free survival (EFS) rate was 81.3% ± 8.3%; the 5-year overall survival (OS) rate was 96.4% ± 4.1%. Patients receiving a near-total or gross-total resection had a 5-year EFS of 100%; those receiving a biopsy or subtotal resection had a 5-year EFS rate of 56.6% ± 15.2% (P < .01). No difference in EFS was observed based on location, histology, or molecular alterations. However, the tumors that progressed or metastasized may have distinct methylation profiles with evidence of activation of the MAPK and PI3K/AKT/mTOR pathways., Conclusions: pLGG with MYB/MYBL1 alterations have good outcomes. Our findings suggest that surgical resectability is a crucial determinant of EFS. Further characterization is required to identify optimal treatment strategies for progressive tumors., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.)

Published
2024
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43. [11C]-methionine positron emission tomography in the evaluation of pediatric low-grade gliomas.

Author

Kim EY, Vavere AL, Snyder SE, Chiang J, Li Y, Patni T, Qaddoumi I, Merchant TE, Robinson GW, Holtrop JL, Shulkin BL, and Bag AK

Abstract

Background: [ 11 C]-Methionine positron emission tomography (PET; [ 11 C]-MET-PET) is principally used for the evaluation of brain tumors in adults. Although amino acid PET tracers are more commonly used in the evaluation of pediatric brain tumors, data on [ 11 C]-MET-PET imaging of pediatric low-grade gliomas (pLGG) is scarce. This study aimed to investigate the roles of [ 11 C]-MET-PET in the evaluation of pLGGs., Methods: Eighteen patients with newly diagnosed pLGG and 26 previously treated pLGG patients underwent [ 11 C]-MET-PET met the inclusion and exclusion criteria. Tumor-to-brain uptake ratio (TBR) and metabolic tumor volumes were assessed for diagnostic performances (newly diagnosed, 15; previously treated 26), change with therapy (newly diagnosed, 9; previously treated 7), and variability among different histology ( n  = 12) and molecular markers ( n  = 7) of pLGGs., Results: The sensitivity of [ 11 C]-MET-PET for diagnosing pLGG, newly diagnosed, and previously treated combined was 93% for both TBR max and TBR peak , 76% for TBR mean , and 95% for qualitative evaluation. TBR max showed a statistically significant reduction after treatment, while other PET parameters showed a tendency to decrease. Median TBR max , TBR peak , and TBR mean values were slightly higher in the BRAFV600E mutated tumors compared to the BRAF fused tumors. Median TBR max , and TBR peak in diffuse astrocytomas were higher compared to pilocytic astrocytomas, but median TBR mean , was slightly higher in pilocytic astrocytomas. However, formal statistical analysis was not done due to the small sample size., Conclusions: Our study shows that [ 11 C]-MET-PET reliably characterizes new and previously treated pLGGs. Our study also shows that quantitative parameters tend to decrease with treatment, and differences may exist between various pLGG types., Competing Interests: Financial disclosure: No potential conflicts of interest relevant to this article exist. Disclaimer: The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health., (© The Author(s) 2024. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)

Published
2024
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44. Trends in pediatric CNS tumors in Armenia: a multicenter retrospective study.

Author

Hoveyan J, Asatryan E, Grigoryan H, Hovsepyan S, Avagyan A, Hakobyan L, Sargsyan L, Iskanyan S, Avagyan M, Hovhannisyan S, Melnichenko I, Minasyan M, Papyan R, Manukyan N, Lazaryan A, Danelyan S, Muradyan A, Arakelyan J, Qaddoumi I, Boop F, Mkhitharyan A, Harutyunyan M, Tamamyan G, and Bardakhchyan S

Subjects
Young Adult, Child, Humans, Male, Infant, Female, Retrospective Studies, Armenia epidemiology, Central Nervous System Neoplasms epidemiology, Central Nervous System Neoplasms therapy, Glioma, Pituitary Neoplasms, Cerebellar Neoplasms
Abstract

Purpose: Central nervous system (CNS) tumors are the most common solid malignancies in children worldwide, including in Armenia. The current study aims to analyze epidemiological data, treatment, and outcomes of children and young adults (≤25 years) with CNS tumors in Armenia during the last 26 years., Methods: We collected data from pediatric and young adult patients treated in selected sites in Armenia from 1 st January 1995 to 31 st December 2020. Incidence by sex, age at diagnosis, time from first complaints to diagnosis, histopathology results, treatment strategies, complications, and overall survival (OS) rates were calculated., Results: The multicenter data analysis revealed 149 patients with diagnosed primary CNS tumors over 26 years. Among them, 84 (56.4%) were male. The median age at diagnosis was 7 years (range, 3 months to 25 years), and the median time from the first complaints to diagnosis was 2 months (range, 1 week to 70 months). Medulloblastomas and other embryonal tumors (47), low-grade gliomas (32), and high-grade gliomas (22) were the most commonly diagnosed malignancies. Ependymomas, craniopharyngiomas, germ cell tumors, and other malignancies were observed in 22 patients. For 26 patients, no histopathological or radiological diagnosis was available. Follow-up information was available for 98 (65.8%) patients. The 5-year OS rate for the whole study group was 67.7%., Conclusion: Consistent with international data, embryonal tumors, and gliomas were the most commonly diagnosed CNS malignancies in Armenia. Multimodal treatment was often not available in Armenia during the study period, especially for early cases., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Published
2024
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45. Smoking-Related Disease Impact in the Eastern Mediterranean Region: A Comprehensive Assessment Using Global Burden of Disease Data.

Author

Sultan Y, Salman Z, Alzaatreh M, Edilbi A, Alani R, Sultan I, Alfaar AS, and Qaddoumi I

Subjects
Humans, Global Burden of Disease, Quality-Adjusted Life Years, Risk Factors, Smoking adverse effects, Smoking epidemiology, Lebanon, Global Health, Cardiovascular Diseases epidemiology, Cardiovascular Diseases etiology, Respiratory Tract Infections
Abstract

Background: Smoking remains a significant risk factor for numerous health issues, including lung cancer, chronic obstructive pulmonary disease, ischemic heart disease, stroke, and respiratory infections. This study investigates the burden of tobacco-related diseases in the Middle East and North Africa (MENA) region., Methods: Utilizing the GBD data, we examined the risk of smoking and second-hand smoke exposure and their related causes of death and disability in the 22 MENA countries. Smoking prevalence and disease burden data were analyzed with estimates reported as age-standardized rates., Results: Tobacco abuse accounted for 14.5% of all deaths and 23.2% of deaths tied to known risk factors, with an age-standardized death rate of 110.8 per 100,000. Cardiovascular diseases were the primary cause of smoking-related deaths and DALYs, representing 53.4% of all deaths and 50.3% of all DALYs. This was followed by neoplasms (24.6% of all deaths and 20.3% of all DALYs), chronic respiratory diseases(12.4% of all deaths and 11.9% of all DALYs), and respiratory infections and tuberculosis(4% of all deaths and 3.4% of all DALYs). Second-hand smoking caused 20.5% of tobacco-related deaths and 21.5% of tobacco-related DALYs, disproportionately affecting younger individuals. An increasing disease burden was observed in Lebanon, Turkey, Syria, Tunisia, UAE, and Libya, and declining rates were most evident in Oman and Qatar., Conclusion: Our study emphasizes the impact of smoking on cardiovascular disease, the primary cause of smoking-related mortality and morbidity in the MENA region. Our findings highlight the urgent need for effective tobacco control policies and interventions.

Published
2024
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46. High-grade glioma in infants and young children is histologically, molecularly, and clinically diverse: Results from the SJYC07 trial and institutional experience.

Author

Chiang J, Bagchi A, Li X, Dhanda SK, Huang J, Pinto SN, Sioson E, Dalton J, Tatevossian RG, Jia S, Partap S, Fisher PG, Bowers DC, Hassall TEG, Lu C, Zaldivar-Peraza A, Wright KD, Broniscer A, Qaddoumi I, Upadhyaya SA, Vinitsky A, Sabin ND, Orr BA, Klimo P Jr, Boop FA, Ashford JM, Conklin HM, Onar-Thomas A, Zhou X, Ellison DW, Gajjar A, and Robinson GW

Subjects
Child, Infant, Humans, Child, Preschool, Retrospective Studies, Prospective Studies, Protein-Tyrosine Kinases, Proto-Oncogene Proteins, Glioma drug therapy, Glioma genetics, Glioma diagnosis, Brain Neoplasms drug therapy, Brain Neoplasms genetics
Abstract

Background: High-grade gliomas (HGG) in young children pose a challenge due to favorable but unpredictable outcomes. While retrospective studies broadened our understanding of tumor biology, prospective data is lacking., Methods: A cohort of children with histologically diagnosed HGG from the SJYC07 trial was augmented with nonprotocol patients with HGG treated at St. Jude Children's Research Hospital from November 2007 to December 2020. DNA methylome profiling and whole genome, whole exome, and RNA sequencing were performed. These data were integrated with histopathology to yield an integrated diagnosis. Clinical characteristics and preoperative imaging were analyzed., Results: Fifty-six children (0.0-4.4 years) were identified. Integrated analysis split the cohort into four categories: infant-type hemispheric glioma (IHG), HGG, low-grade glioma (LGG), and other-central nervous system (CNS) tumors. IHG was the most prevalent (n = 22), occurred in the youngest patients (median age = 0.4 years), and commonly harbored receptor tyrosine kinase gene fusions (7 ALK, 2 ROS1, 3 NTRK1/2/3, 4 MET). The 5-year event-free (EFS) and overall survival (OS) for IHG was 53.13% (95%CI: 35.52-79.47) and 90.91% (95%CI: 79.66-100.00) vs. 0.0% and 16.67% (95%CI: 2.78-99.74%) for HGG (p = 0.0043, p = 0.00013). EFS and OS were not different between IHG and LGG (p = 0.95, p = 0.43). Imaging review showed IHGs are associated with circumscribed margins (p = 0.0047), hemispheric location (p = 0.0010), and intratumoral hemorrhage (p = 0.0149)., Conclusions: HGG in young children is heterogeneous and best defined by integrating histopathological and molecular features. Patients with IHG have relatively good outcomes, yet they endure significant deficits, making them good candidates for therapy de-escalation and trials of molecular targeted therapy., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Published
2024
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47. Outcomes of SARS-CoV-2 infection in 126 children and adolescents with central nervous system tumors.

Author

Moreira DC, Qaddoumi I, Chen Y, Bhakta N, Chantada GL, Santana VM, Caniza MA, Devidas M, Pritchard-Jones K, Rodriguez-Galindo C, Bouffet E, and Mukkada S

Subjects
Humans, Adolescent, Child, SARS-CoV-2, Disease Progression, COVID-19 complications, COVID-19 epidemiology, Central Nervous System Neoplasms epidemiology, Central Nervous System Neoplasms therapy, Leukopenia, Glioma
Abstract

Background: The Global Registry of COVID-19 in Childhood Cancer (GRCCC) seeks to describe the natural history of SARS-CoV-2 in children with cancer across the world. Here, we report the disease course and management of coronavirus disease 2019 (COVID-19) infection in the subset of children and adolescents with central nervous system (CNS) tumors who were included in the GRCCC until February 2021, the first data freeze., Procedure: The GRCCC is a deidentified web-based registry of patients less than 19 years of age with cancer or recipients of a hematopoietic stem cell transplant and laboratory-confirmed SARS-CoV-2 infection. Demographic data, cancer diagnosis, cancer-directed therapy, and clinical characteristics of SARS-CoV-2 infection were collected. Outcomes were collected at 30 and 60 days post infection., Results: The GRCCC included 1500 cases from 45 countries, including 126 children with CNS tumors (8.4%). Sixty percent of the cases were from middle-income countries, while no cases were reported from low-income countries. Low-grade gliomas, high-grade gliomas, and CNS embryonal tumors were the most common CNS cancer diagnoses (67%, 84/126). Follow-up at 30 days was available for 107 (85%) patients. Based on the composite measure of severity, 53.3% (57/107) of reported SARS-CoV-2 infections were asymptomatic, 39.3% (42/107) were mild/moderate, and 6.5% (7/107) were severe or critical. One patient died from SARS-CoV-2 infection. There was a significant association between infection severity and absolute neutrophil count less than 500 (p = .04). Of 107 patients with follow-up available, 40 patients (37.4%) were not receiving cancer-directed therapy. Thirty-four patients (50.7%) had a modification to their treatment due to withholding of chemotherapy or delays in radiotherapy or surgery., Conclusion: In this cohort of patients with CNS tumors and COVID-19, the frequency of severe infection appears to be low, although severe disease and death do occur. We found that greater severity was seen in patients with severe neutropenia, although treatment modifications were not associated with infection severity or cytopenias. Additional analyses are needed to further describe this unique group of patients., (© 2023 Wiley Periodicals LLC.)

Published
2023
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48. Pediatric diencephalic tumors: a constellation of entities and management modalities.

Author

Pinto SN, Chiang J, Qaddoumi I, Livingston D, and Bag A

Abstract

The diencephalon is a complex midline structure consisting of the hypothalamus, neurohypophysis, subthalamus, thalamus, epithalamus, and pineal body. Tumors arising from each of these diencephalic components differ significantly in terms of biology and prognosis. The aim of this comprehensive review is to describe the epidemiology, clinical symptoms, imaging, histology, and molecular markers in the context of the 2021 WHO classification of central nervous system neoplasms . We will also discuss the current management of each of these tumors., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Pinto, Chiang, Qaddoumi, Livingston and Bag.)

Published
2023
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49. The role of neurosurgery in advancing pediatric CNS tumor care worldwide.

Author

Roach JT, Baticulon RE, Campos DA, Andrews JM, Qaddoumi I, Boop FA, and Moreira DC

Abstract

Introduction: There is substantial inequity in survival outcomes for pediatric brain tumor patients residing in high-income countries (HICs) compared to low- and middle-income countries (LMICs). To address disparities in pediatric cancer survival, the World Health Organization (WHO) established the Global Initiative for Childhood Cancer (GICC) to expand quality care for children with cancer., Research Question: To provide an overview of pediatric neurosurgical capacity and detail the burden of neurosurgical diseases impacting children., Material and Methods: A narrative review of the current context of global pediatric neurosurgical capacity as it relates to neurooncology and other diseases relevant to children., Results: In this article, we provide an overview of pediatric neurosurgical capacity and detail the burden of neurosurgical diseases impacting children. We highlight concerted advocacy and legislative efforts aimed at addressing unmet neurosurgical needs in children. Finally, we discuss the potential implications of advocacy efforts on treating pediatric CNS tumors and outline strategies to improve global outcomes for children with brain tumors worldwide in the context of the WHO GICC., Discussion and Conclusion: With both global pediatric oncology and neurosurgical initiatives converging on the treatment of pediatric brain tumors, significant strides toward decreasing the burden of pediatric neurosurgical diseases will hopefully be made., Competing Interests: The authors declare no competing interests., (© 2023 The Authors.)

Published
2023
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50. The clinical and molecular characteristics of progressive hypothalamic/optic pathway pilocytic astrocytoma.

Author

Li X, Moreira DC, Bag AK, Qaddoumi I, Acharya S, and Chiang J

Subjects
Humans, Male, Child, Preschool, Phosphatidylinositol 3-Kinases metabolism, Proto-Oncogene Proteins c-akt metabolism, Tumor Suppressor Protein p53, TOR Serine-Threonine Kinases metabolism, Proto-Oncogene Proteins B-raf genetics, Astrocytoma genetics, Brain Neoplasms genetics
Abstract

Background: Unresectable hypothalamic/optic pathway pilocytic astrocytoma (PA) often progresses despite multiple therapies. Identifying clinical and molecular characteristics of progressive tumors may aid in prognostication and treatment., Methods: We collected 72 unresectable, non-neurofibromatosis type 1-associated hypothalamic/optic pathway PA to identify clinical and biologic factors associated with tumor progression. Tumors that progressed after therapy, metastasized, or resulted in death were categorized into Cohort B; those that did not meet these criteria were categorized into Cohort A. DNA methylation and transcriptome analyses were performed on treatment-naïve tumors, and the findings were validated by immunohistochemistry (IHC)., Results: The median follow-up of the entire cohort was 12.3 years. Cohort B was associated with male sex (M:F = 2.6:1), younger age at diagnosis (median 3.2 years vs 6.7 years, P = .005), and high incidence of KIAA1549-BRAF fusion (81.5% vs 38.5%, P = .0032). Cohort B demonstrated decreased CpG methylation and increased RNA expression in mitochondrial genes and genes downstream of E2F and NKX2.3. Transcriptome analysis identified transcription factor TBX3 and protein kinase PIM1 as common downstream targets of E2F and NKX2.3. IHC confirmed increased expression of TBX3 and PIM1 in Cohort B tumors. Gene enrichment analysis identified enrichment of MYC targets and MAPK, PI3K/AKT/mTOR, and p53 pathways, as well as pathways related to mitochondrial function., Conclusions: We identified risk factors associated with progressive PA. Our results support the model in which the p53-PIM1-MYC axis and TBX3 act alongside MAPK and PI3K/AKT/mTOR pathways to promote tumor progression, highlighting potential new targets for combination therapy and refining disease prognostication., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.)

Published
2023
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