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1.
Pediatr Clin North Am ; 67(6): 1051-1067, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33131534

RESUMO

Advances in multimodality care for patients with pediatric cancer continues to improve long-term survival. The use of surgery, chemotherapy, and radiotherapy may lead to debilitating late effects in childhood cancer survivors. It is critically important to understand, mitigate, and screen for late effects to improve the quality of life in childhood cancer survivors. This review summarizes the use of radiotherapy in children, radiobiology of tissue injury, impact of age on late effects, important organ systems affected by radiotherapy during survivorship, and screening for radiotherapy late effects.

2.
Pediatr Blood Cancer ; : e28619, 2020 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-32790118

RESUMO

To examine the educational background, clinical practice, and preferences regarding continuing medical education (CME) among radiation oncologists who attended the 2019 meeting of the Pediatric Radiation Oncology Society (PROS), a survey consisting of 20 questions was distributed asking for demographic and educational background, clinical practice, and preferences regarding pediatric radiation oncology CME. Of 188 participants, 130 (69.2%) returned the questionnaire. More than 80% reported access to CT simulation, three-dimensional radiotherapy, and general anesthesia while <30% had access to intraoperative radiotherapy, proton, and heavy particle therapy. After residency, 12.1% did further training in pediatric radiation oncology. When asked about further training in pediatrics after residency, 88.8% answered that there should be a formal training program beyond residency in order to treat children. More than 75% acquired knowledge in pediatric radiation oncology through journals, books, live meetings, and tumor boards. The results of this survey may help Pediatric Radiation Oncology Society (PROS) in creating guidelines and recommendations for improvement in pediatric radiation oncology training and practice support as well as the development of CME activities most likely to benefit practitioners.

3.
Pediatr Blood Cancer ; : e28629, 2020 Aug 09.
Artigo em Inglês | MEDLINE | ID: mdl-32776500

RESUMO

This report by the Radiation Oncology Discipline of Children's Oncology Group (COG) describes the practice patterns of pediatric image-guided radiotherapy (IGRT) based on a member survey and provides practice recommendations accordingly. The survey comprised of 11 vignettes asking clinicians about their recommended treatment modalities, IGRT preferences, and frequency of in-room verification. Technical questions asked physicists about imaging protocols, dose reduction, setup correction, and adaptive therapy. In this report, the COG Radiation Oncology Discipline provides an IGRT modality/frequency decision tree and the expert guidelines for the practice of ionizing image guidance in pediatric radiotherapy patients.

4.
Neuro Oncol ; 2020 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-32750703

RESUMO

BACKGROUND: We investigated differences in radiation-induced grade 3+ lymphopenia (G3+L), defined as an absolute lymphocyte count (ALC) nadir of <500 cells/µL, after proton therapy (PT) or X-ray (photon) therapy (XRT) for patients with glioblastoma (GBM). METHODS: Patients enrolled in a randomized phase II trial received PT (n=28) or XRT (n=56), concomitantly with temozolomide. ALC was measured before, weekly during, and within 1 month after radiotherapy. Whole-brain mean dose (WBMD) and brain dose-volume indices were extracted from planned dose distributions. Univariate and multivariate logistic regression analyses were used to identify independent predictive variables. The resulting model was evaluated using receiver operating characteristic (ROC) curve analysis. RESULTS: Rates of G3+L were lower in men (7/47 [15%] vs. women (19/37 [51%], P<0.001)), and for PT (4/28 [14%] vs. XRT (22/56 [39%], P=0.024)). G3+L was significantly associated with baseline ALC, WBMD, and brain volumes receiving 5‒40 Gy(RBE) or higher (i.e., V5 through V40). Stepwise multivariate logistic regression analysis identified being female (odds ratio [OR] 6.2, 05% confidence interval [CI] 1.95‒22.4, P=0.003), baseline ALC (OR 0.18, 95% CI 0.05‒0.51, P=0.003) and whole brain V20 (OR 1.07, 95% CI 1.03‒1.13, P=0.002) as the strongest predictors. ROC analysis yielded an area under the curve of 0.86 (95% CI 0.79-0.94) for the final G3+L prediction model. CONCLUSIONS: Sex, baseline ALC, and whole brain V20 were the strongest predictors of G3+L for patients with GBM treated with radiation and temozolomide. PT reduced brain volumes receiving low and intermediate doses and, consequently, reduced G3+L.

6.
Pancreas ; 49(6): 737-743, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32590617

RESUMO

Pancreatic cancer is the fourth most common cause of cancer-related morality worldwide, and the prognosis remains poor despite aggressive therapy. Carbon ion radiotherapy has favorable radiobiological and physical characteristics in the treatment, including a higher linear energy transfer and higher relative biological effectiveness, which increase the cell kill while potentially reducing toxicities to nearby normal tissues. Although small, early clinical studies have shown promise in both the resectable and unresectable settings to improve local control and overall survival while minimizing toxicities. Currently, there are several trials, including 2 sponsored by institutions in the United States, investigating the role of carbon ion radiotherapy for the treatment of locally advanced pancreatic cancer.

8.
J Neurooncol ; 148(1): 89-95, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32303974

RESUMO

PURPOSE: Stereotactic radiosurgery (SRS) is commonly performed after surgical resection of brain metastases to reduce the chance of local tumor recurrence while maintaining cognitive function. Target delineation in these cases is typically based off T1-weighted post-gadolinium MRI (T1Gd). In this study, we report outcomes for patients having postoperative SRS in which the planning target volume (PTV) was based on T2-weighted MRI (T2W). METHODS: Sixty-two consecutive patients having single-fraction SRS after brain metastases resection were retrospectively reviewed. Excluded were patients with prior whole brain radiation therapy, multiple resection cavities, and small cell pathologies. RESULTS: The median time from surgery to SRS was 11 days; 26 patients (42%) had SRS ≤ 7 days. The median PTV was 8.0 cm3; the median margin dose was 18 Gy. The crude rates of local tumor control (LC), leptomeningeal disease (LMD), distant brain recurrence (DBR), and radiation necrosis (RN) were 85%, 19%, 37%, and 2%, respectively. The 1-year LC, LMD, DBR, and RN rates were 88%, 25%, 36%, and 0%, respectively. No tumor or dosimetric factor was associated with LC. Sub-total tumor resection was a risk factor for LMD (HR 5.11, P = 0.003), whereas patients with multiple brain metastases had a greater risk of DBR (HR 2.88, P = 0.01). The median PTV was smaller compared to the median PTV based off the consensus guidelines utilizing T1Gd MRI (8.0 cm3 vs. 9.1 cm3, P = 0.004). CONCLUSION: T2W MRI provided accurate resection cavity delineation even in the early postoperative period and was associated with decreased PTV compared to T1Gd MRI in the majority of cases.

9.
Breast Cancer Res Treat ; 181(2): 291-296, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32318954

RESUMO

PURPOSE: Breast cancer is the most commonly diagnosed cancer in women, with many efforts aimed at reducing acute and late toxicity given the generally favorable clinical outcomes with the current standard of care. Carbon ion radiation therapy is an emerging technique that may reduce dose to adjacent organs at risk while allowing dose escalation to the target. Given the efficacy of the standard treatments for breast cancer, there have been few prospective studies to date investigating carbon ion radiation therapy in breast cancer. METHODS: PubMed/Medline, Ebsco, Cochrane, and Scopus were systematically reviewed using the search terms "carbon ion" and "breast" in November 2019. Out of the 76 articles screened, 26 articles were included. RESULTS: This comprehensive review describes the physical and biological properties of carbon ion radiation therapy, with an emphasis on how these properties can be applied in the setting of breast cancer. Studies investigating the role of carbon ion radiation therapy in early stage breast cancers are reviewed. Additionally, the use of carbon ion radiation therapy in locally advanced disease, recurrent disease, and radiation-induced angiosarcoma are discussed. CONCLUSION: Although the data is limited, the early clinical results are promising. Further clinical trials are needed, especially in the setting of locally advanced and recurrent disease, to fully define the potential role of carbon ion radiation therapy in the treatment of breast cancer.

10.
J Neurooncol ; 147(3): 503-513, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32206977

RESUMO

BACKGROUND: Carbon ion radiotherapy (CIRT) is an emerging radiation therapy to treat skull base chordomas and chondrosarcomas. To date, its use is limited to a few centers around the world, and there has been no attempt to systematically evaluate survival and toxicity outcomes reported in the literature. Correspondingly, the aim of this study was to qualitatively and quantitatively assess these outcomes. METHODS: A systematic search of seven electronic databases from inception to November 2019 was conducted following PRISMA guidelines. Articles were screened against pre-specified criteria. Outcomes were then pooled by random-effects meta-analyses of proportions. RESULTS: A total of nine studies provided unique metadata for assessment, with six originating from Heidelberg, Germany. The surveyed cohort size was 632 patients, with 389 (62%) chordomas and 243 (38%) chondrosarcomas of the skull base. Across all studies, median cohort age at therapy and female proportion were 46 years and 51% respectively. Estimates of local control incidence at 1-, 5-, and 10-years in chordoma-only studies were 99%, 80%, and 56%, and in chondrosarcoma-only studies were 99%, 89%, and 88%. Estimates of overall survival probability at 1-, 5-, and 10-years in chordoma-only studies were 100%, 94%, and 78%, and in chondrosarcoma-only studies were 99%, 95%, and 79%. The incidence of early and late toxicity (Grade ≥ 3) ranged from 0 to 4% across all study groups. CONCLUSIONS: The emerging use of CIRT to treat skull base chordomas and chondrosarcomas appear promising with regard to tumor control, overall survival, and risk profile of early and late toxicity. The current literature suffers from the fact only a few centers in the world currently employ this technology.

11.
J Clin Oncol ; 38(11): 1175-1185, 2020 Apr 10.
Artigo em Inglês | MEDLINE | ID: mdl-32105509

RESUMO

PURPOSE: Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive, early-childhood brain tumor without standard effective treatment. To our knowledge, we conducted the first AT/RT-specific cooperative group trial, ACNS0333, to examine the efficacy and safety of intensive postoperative chemotherapy and focal radiation to treat AT/RT. PATIENTS AND METHODS: Patients from birth to 22 years of age with AT/RT were eligible. After surgery, they received 2 courses of multiagent chemotherapy, followed by 3 courses of high-dose chemotherapy with peripheral blood stem cell rescue and involved-field radiation therapy. Timing of radiation was based on patient age and disease location and extent. Central testing of tumor and blood for SMARCB1 status was mandated. Tumor molecular subclassification was performed retrospectively. The primary analysis was event-free survival (EFS) for patients < 36 months of age compared with a cooperative groups' historical cohort. Although accrual was based on the therapeutic question, potential prognostic factors, including age, tumor location, M stage, surgical resection, order of therapy, germline status, and molecular subtype, were explored. RESULTS: Of 65 evaluable patients, 54 were < 36 months of age. ACNS0333 therapy significantly reduced the risk of EFS events in patients < 36 months of age compared with the historical cohort (P < .0005; hazard rate, 0.43; 95% CI, 0.28 to 0.66). Four-year EFS and overall survival for the entire cohort were 37% (95% CI, 25% to 49%) and 43% (95% CI, 31% to 55%), respectively. Timing of radiation did not affect survival, and 91% of relapses occurred by 2 years from enrollment. Treatment-related deaths occurred in 4 patients. CONCLUSION: The ACNS0333 regimen dramatically improved survival compared with historical therapies for patients with AT/RT. Clinical characteristics and molecular subgrouping suggest prognostic differences. ACNS0333 results lay a foundation on which to build future studies and incorporate testing of new therapeutic agents.

12.
World Neurosurg ; 136: e328-e333, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31917308

RESUMO

BACKGROUND: Stereotactic radiosurgery (SRS) can be used to achieve local control of metastatic brain lesions. However, the temporal response of these lesions to SRS is incompletely understood and quantified. We aimed to examine the temporal response to SRS of single brain metastases from 4 different primary cancers. METHODS: A retrospective chart review was performed of patients who underwent SRS at our institution from 2009 to 2012. Clinical, treatment, and volumetric data were collected. All patients were treated according to Radiation Therapy Oncology Group 90-05 schema. RESULTS: This study included 204 patients (116 men and 88 women) with a median age of 61 years (range, 27-86 years). Of patients, 20 (10%) had breast cancer, 79 (39%) had non-small cell lung cancer, 69 (34%) had melanoma, and 36 (17%) had renal cell carcinoma. Initial overall median tumor volume was 0.8 cm3 (range, 0.02-16.9 cm3). Median tumor volume was 0.5 cm3 (range, 0.02-20.4 cm3) at 1 month after SRS, 0.3 cm3 (range, 0.02-10.8 cm3) at 3 months after SRS, and 0.4 cm3 (range, 0.02-18.6 cm3) at 6 months after SRS. Based on the joint model constructed from our results, we demonstrate a median 16% (95% confidence interval, 11%-20%) reduction in tumor volume every 30 days. CONCLUSIONS: The results of this study demonstrate a consistent reduction in tumor volume following SRS over a 6-month period. Responses were modest over the first 30 days but accelerated at 3 months and varied by tumor type.


Assuntos
Neoplasias Encefálicas/secundário , Neoplasias Encefálicas/cirurgia , Metástase Neoplásica/patologia , Metástase Neoplásica/terapia , Radiocirurgia/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
13.
J Clin Oncol ; 38(5): 454-461, 2020 Feb 10.
Artigo em Inglês | MEDLINE | ID: mdl-31774710

RESUMO

PURPOSE: Proton radiotherapy (PRT) may lessen the neuropsychological risk traditionally associated with cranial radiotherapy for the treatment of pediatric brain tumors by reducing the dose to normal tissue compared with that of photon radiotherapy (XRT). We examined the change in intellectual scores over time in patients with pediatric medulloblastoma treated with craniospinal PRT versus XRT. METHODS: Intelligence test scores were obtained for a sample of pediatric patients treated between 2007 and 2018 on the same medulloblastoma protocols that differed only in radiotherapy modality (PRT v XRT). Growth curve analyses compared change in scores over time since diagnosis between groups. RESULTS: Longitudinal intelligence data from 79 patients (37 PRT, 42 XRT) were examined. Groups were similar on most demographic/clinical variables, including sex (67.1% male), age at diagnosis (mean, 8.6 years), craniospinal irradiation dose (median, 23.4 Gy), length of follow-up (mean, 4.3 years), and parental education (mean, 14.3 years). Boost dose (P < .001) and boost margin (P = .001) differed between groups. Adjusting for covariates, the PRT group exhibited superior long-term outcomes in global intelligence quotient (IQ), perceptual reasoning, and working memory compared with the XRT group (all P < .05). The XRT group exhibited a significant decline in global IQ, working memory, and processing speed (all P < .05). The PRT group exhibited stable scores over time in all domains with the exception of processing speed (P = .003). CONCLUSION: To our knowledge, this is the first study to compare intellectual trajectories between pediatric patients treated for medulloblastoma with PRT versus those treated with XRT on comparable, contemporary protocols. PRT was associated with more favorable intellectual outcomes in most domains compared with XRT, although processing speed emerged as a vulnerable domain for both groups. This study provides the strongest evidence to date of an intellectual sparing advantage with PRT in the treatment of pediatric medulloblastoma.

14.
Pediatr Blood Cancer ; 67(2): e28064, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31736188

RESUMO

BACKGROUND: Cranial radiotherapy (RT) is associated with risk for cognitive and adaptive dysfunction. Proton RT (PRT) is a technique hypothesized to spare cognition by reducing exposure to nontarget brain tissue. However, little is known regarding functional outcomes in survivors of pediatric brain tumor (BT) treated with PRT. The present study examined the relationship between cognitive and adaptive outcomes in pediatric BT survivors post-PRT. METHODS: Survivors treated with either focal (n = 33) or craniospinal irradiation (CSI; n = 37) PRT completed neurocognitive evaluations approximately 5 years post-treatment. Results of intelligence testing and ratings of adaptive functioning are reported. Mediation models examined the relationship among radiation field, cognition, and adaptive functioning. RESULTS: The PRT CSI group demonstrated worse cognitive outcomes than the PRT Focal group across each cognitive index (Cohen's d = 0.56-0.70). Parent ratings of adaptive functioning were also worse in the PRT CSI group than the PRT Focal group (Global Adaptive Composite, d = 0.53; conceptual skills, d = 0.67). Cognitive performance fully mediated the relationship between radiation field and adaptive outcomes, while controlling for group differences in tumor histology and RT dose. CONCLUSIONS: Focal PRT survivors demonstrated generally positive outcomes with weaknesses in processing speed and aspects of adaptive functioning. CSI exposure was associated with more consistently poor cognitive and adaptive outcomes. The increased risk for adaptive dysfunction in the PRT CSI group appeared due to the effects of CSI on cognition. Efforts to reduce the volume of tissue exposure to RT remain important.


Assuntos
Atividades Cotidianas , Adaptação Psicológica , Neoplasias Encefálicas/radioterapia , Cognição/fisiologia , Radiação Cranioespinal/métodos , Terapia com Prótons/métodos , Sobreviventes/psicologia , Adolescente , Adulto , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/psicologia , Criança , Pré-Escolar , Cognição/efeitos da radiação , Estudos de Coortes , Feminino , Seguimentos , Humanos , Masculino , Prognóstico , Qualidade de Vida , Ajustamento Social , Adulto Jovem
15.
Radiother Oncol ; 142: 36-42, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31431375

RESUMO

BACKGROUND: Pseudoprogression (PsP) following radiation therapy (RT) for low grade glioma (LGG, WHO grade I and II), including both photon-based intensity-modulated RT (IMRT) and proton beam therapy (PBT), has been described. However, its incidence has yet to be consolidated. The aim of this systematic review and meta-analysis was to pool the current literature and establish the incidence of PsP in these groups to better inform surveillance protocols in the future. METHODS: Searches of 4 electronic databases from inception to April 2019 were conducted following PRISMA guidelines. Articles were screened against pre-specified criteria. The incidence of outcomes was then extracted and pooled by random-effects meta-analysis of proportions. RESULTS: A total of 5 pediatric and 4 adult cohort studies describing 517 and 424 LGG subjects respectively satisfied all selection criteria. The estimated incidences of PsP in pediatric subjects following IMRT and PBT were 33% (95% CI, 20-47%) and 34% (95% CI, 23-45%) respectively, with no difference between modalities. The estimated incidences of PsP in adult subjects following IMRT and PBT were 18% (95% CI, 12-25%) and 30% (95% CI, 21-39%) respectively, with PsP significantly less common following IMRT than PBT (P-heterogeneity = 0.04). Median time from radiation initiation to first detection of PsP ranged from 6 to 12 months across all modalities and age groups. CONCLUSIONS: The incidence of PsP following both IMRT and PBT in the management of pediatric and adult LGG is not negligible, and should therefore be recognized as a pertinent sequala within the first year at least following treatment. However, a lack of accountability in the current literature for the differences in PsP interpretation, radiation modality, radiobiology and molecular biology of LGGs precludes any firm surveillance recommendations at this time.

16.
Clin Neurol Neurosurg ; 189: 105632, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31862631

RESUMO

OBJECTIVES: Spinal ependymomas are rare intramedullary neoplasms. The paucity of cases limits the ability to conduct large prospective studies. Current guidelines recommend maximal safe resection followed by adjuvant radiotherapy (RT) in cases of grade II spinal ependymomas with subtotal resections (STR) and all grade III spinal ependymomas. Herein we assess the impact of RT on survival in grades II and III spinal ependymomas. PATIENTS AND METHODS: The National Cancer Database was queried for adult patients with WHO grades II or III spinal ependymomas diagnosed between 2004 and 2014 who underwent resection or biopsy. Kaplan-Meier and multivariable Cox regression models were used to determine the impact of radiotherapy on survival. RESULTS: A total of 1058 patients met inclusion criteria. Most patients (85.9 %) received a biopsy/STR versus gross total resection (GTR, 14.1 %). Radiotherapy was preferentially performed in those with residual tumor (p = 0.001). We found a 10-fold increased hazard of death in grade III versus grade II tumors (HR: 10.33; 95 % CI: 5.01-21.3; p < 0.001). Age positively correlated with worsened survival (HR: 1.04; 95 % CI: 1.02-1.10; p < 0.001). Adjuvant RT did not reduce the hazard of death for the cohort overall (HR: 1.08; 95 % CI: 0.55-2.10; p = 0.810) or among those with grade II tumors (HR: 0.90; 95 % CI: 0.38-2.10; p = 0.810). We found no additional survival benefit of GTR compared to biopsy/STR (HR: 0.52; 95 % CI: 0.19-1.50; p = 0.217). CONCLUSION: While RT may improve progression-free survival, it may not impact overall survival in surgically resected grade II and III spinal ependymomas. Future studies should evaluate the impact of RT on local recurrence and symptomatic improvement.

17.
J Neurooncol ; 145(2): 191-199, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31571113

RESUMO

INTRODUCTION: Gliomas are among the most common primary brain malignancies, with a poor prognosis for high grade gliomas despite aggressive therapy. Carbon ions, which exhibit favorable biological and physical characteristics, have recently been studied in intracranial malignancies as a way to escalate dose to the tumor while minimizing dose to normal tissue. METHODS: Pubmed/Medline, SCOPUS, EMBASE, CINAHL and the Cochrane database were systematically reviewed using the search terms "carbon ion" and "glioma" or "glioblastoma" in August 2019. Out of 332 articles screened, 43 were included in this analysis. RESULTS: This comprehensive review describes the pertinent physics and radiation biology studies relevant to the treatment of gliomas with carbon ions and summarizes the important clinical studies for both high and low grade gliomas. Studies investigating carbon ions as both definitive radiotherapy and as a boost to traditional radiotherapy are reviewed. The use of carbon ion radiotherapy in the setting of recurrent disease is also described. CONCLUSIONS: Carbon ion radiotherapy is both efficacious and safe based on early clinical studies. Current trials, including the CLEOPATRA and CINDERLLA trials, hope to define the role of carbon ion radiotherapy in the treatment of gliomas.


Assuntos
Neoplasias Encefálicas/radioterapia , Glioma/radioterapia , Radioterapia com Íons Pesados , Glioblastoma/radioterapia , Humanos , Resultado do Tratamento
18.
Acta Medica (Hradec Kralove) ; 62(2): 72-76, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31362814

RESUMO

The high incidence of hepato-biliary vascular anatomy variations necessitates its evaluation prior to performing liver transplantation, hepatobiliary, pancreatic, gastric and oesophageal surgeries. We report a unique case of persistence of embryonic arteries of the liver, wherein, the liver was supplied by five vessels. In addition to the usual right and left hepatic arteries from the hepatic artery proper, the liver received two accessory right hepatic arteries, one from the gastroduodenal artery, while another arising from superior mesenteric artery and an accessory left hepatic artery, from the left gastric artery. The origin of gastroduodenal artery was found to be unusually high and its abnormal anterior course over the common bile duct further added complexity to the hepatobiliary anatomy. The presence of these aberrant and accessory arteries predisposes to inadvertent injury leading to patient morbidity and sometimes mortality.


Assuntos
Artéria Hepática/anatomia & histologia , Transplante de Fígado/métodos , Fígado/irrigação sanguínea , Fígado/cirurgia , Artérias Mesentéricas/anatomia & histologia , Idoso de 80 Anos ou mais , Feminino , Humanos , Fígado/anatomia & histologia , Circulação Hepática
19.
Pediatr Blood Cancer ; 66(11): e27952, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31397065

RESUMO

PURPOSE/OBJECTIVE(S): Bladder and prostate are unfavorable sites for rhabdomyosarcoma (B/P-RMS), and represent a challenging location for radiotherapy. MATERIALS/METHODS: Nineteen patients with B/P-RMS were enrolled on a prospective registry protocol (2008-2017) and treated with chemotherapy, proton beam therapy (PBT), and surgical resection (n = 8; 42%). Emphasis was given to treatment technique, disease-related outcomes, and toxicity associated with PBT. RESULTS: The majority of patients had bladder RMS (74%) of embryonal histology (95%), Group III (68%), and intermediate-risk disease by Children's Oncology Group (COG) risk stratification (89%). Seven patients (37%) had primary tumors >5 cm in size. All patients were treated according to COG protocols. With a median follow-up of 66.2 months, 5-year overall survival (OS) and progression-free survival (PFS) were 76%. Four patients (21%) experienced disease relapse, all presenting with local failure. The 5-year local control (LC) rate was 76%. Tumor size predicted LC, with 5-year LC for patients with >5 cm tumors being 43% versus 100% for those with ≤5 cm tumors (P = .006). Univariate analysis demonstrated an effect of tumor size on OS (tumor >5 cm, hazard ratio [HR] 17.7, P = .049) and PFS (HR 17.7, P = .049). Acute grade 2 toxicity was observed in two patients (11%, transient proctitis). Late grade 2+ toxicity was observed in three patients (16%; n = 1 grade 2 skeletal deformity; n = 3 transient grade 2 urinary incontinence; one patient experienced both). CONCLUSIONS: PBT for B/P-RMS affords promising disease-related outcomes with an acceptable toxicity profile. Higher local failure rates were observed for larger tumors, supporting dose-escalation components of ongoing RMS clinical trials.


Assuntos
Neoplasias da Próstata/radioterapia , Terapia com Prótons , Rabdomiossarcoma Embrionário/radioterapia , Neoplasias da Bexiga Urinária/radioterapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Pré-Escolar , Terapia Combinada , Cistectomia , Feminino , Seguimentos , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Estadiamento de Neoplasias , Proctite/etiologia , Intervalo Livre de Progressão , Modelos de Riscos Proporcionais , Estudos Prospectivos , Prostatectomia , Neoplasias da Próstata/tratamento farmacológico , Neoplasias da Próstata/patologia , Neoplasias da Próstata/cirurgia , Terapia com Prótons/efeitos adversos , Terapia com Prótons/métodos , Lesões por Radiação/etiologia , Sistema de Registros , Rabdomiossarcoma Alveolar/tratamento farmacológico , Rabdomiossarcoma Alveolar/patologia , Rabdomiossarcoma Alveolar/radioterapia , Rabdomiossarcoma Alveolar/cirurgia , Rabdomiossarcoma Embrionário/tratamento farmacológico , Rabdomiossarcoma Embrionário/patologia , Rabdomiossarcoma Embrionário/cirurgia , Risco , Carga Tumoral , Neoplasias da Bexiga Urinária/tratamento farmacológico , Neoplasias da Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/cirurgia , Incontinência Urinária/etiologia
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