RESUMO
Due to its rarity, current literature assessing prognostic factors and survival outcomes of hemangioblastoma is limited. Patients with histologically confirmed hemangioblastoma were identified from the US National Cancer Data Base. 1488 patients met inclusion criteria. 644 patients underwent gross total resection (GTR), 220 subtotal resection (STR)/biopsy, 60 stereotactic radiosurgery (SRS), 15 external beam radiotherapy (EBRT), 51 surgery followed by radiotherapy (SR + RT) and 498 no treatment. Independent predictors of shorter OS included age ≥ 40 (HR, 3.897; 95% CI, 2.341-6.487; p < 0.001), Charlson-Deyo score ≥ 1(HR, 1.756; 95% CI, 1.213-2.544; p = 0.003), tumor location in the brainstem (HR, 1.955; 95% CI, 1.129-3. 384; p = 0.017) compared to cerebellum, no treatment (HR, 2530; 95% CI, 1.533-4.177; p < 0.001) and receipt of EBRT (HR, 2.860; 95% CI, 1.073-7.618; p = 0.036) compared to STR/biopsy. GTR was associated with longer OS (HR 0.617; 95% CI, 0.391-0.974; p = 0.038), while SRS had comparable OS to STR/biopsy. The overall trend of OS by treatment modality was consistent after matching to age- and sex-matched US population data. In patients younger than 40 years, treatment was not a significant predictor of OS. In conclusion, GTR remained the optimal treatment for hemangioblastoma. SRS may perform similarly to surgery alone. Treatment was not a significant predictor of survival in younger patients.
Assuntos
Neoplasias Cerebelares/mortalidade , Neoplasias Cerebelares/terapia , Hemangioblastoma/mortalidade , Hemangioblastoma/terapia , Avaliação de Processos e Resultados em Cuidados de Saúde/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Neoplasias Cerebelares/patologia , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Feminino , Seguimentos , Hemangioblastoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Modelos de Riscos Proporcionais , Radiocirurgia , Radioterapia Adjuvante , Taxa de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: The aim of this study is to evaluate the cost-effectiveness of proton beam therapy with cochlear dose reduction compared with conventional X-ray radiotherapy for medulloblastoma in childhood. METHODS: We developed a Markov model to describe health states of 6-year-old children with medulloblastoma after treatment with proton or X-ray radiotherapy. The risks of hearing loss were calculated on cochlear dose for each treatment. Three types of health-related quality of life (HRQOL) of EQ-5D, HUI3 and SF-6D were used for estimation of quality-adjusted life years (QALYs). The incremental cost-effectiveness ratio (ICER) for proton beam therapy compared with X-ray radiotherapy was calculated for each HRQOL. Sensitivity analyses were performed to model uncertainty in these parameters. RESULTS: The ICER for EQ-5D, HUI3 and SF-6D were $21 716/QALY, $11 773/QALY, and $20 150/QALY, respectively. One-way sensitivity analyses found that the results were sensitive to discount rate, the risk of hearing loss after proton therapy, and costs of proton irradiation. Cost-effectiveness acceptability curve analysis revealed a 99% probability of proton therapy being cost effective at a societal willingness-to-pay value. CONCLUSIONS: Proton beam therapy with cochlear dose reduction improves health outcomes at a cost that is within the acceptable cost-effectiveness range from the payer's standpoint.
Assuntos
Neoplasias Cerebelares/economia , Neoplasias Cerebelares/radioterapia , Perda Auditiva/economia , Meduloblastoma/economia , Meduloblastoma/radioterapia , Qualidade de Vida , Lesões por Radiação/economia , Neoplasias Cerebelares/mortalidade , Criança , Cóclea/efeitos da radiação , Análise Custo-Benefício/classificação , Análise Custo-Benefício/economia , Feminino , Custos de Cuidados de Saúde/estatística & dados numéricos , Perda Auditiva/mortalidade , Perda Auditiva/prevenção & controle , Humanos , Japão , Masculino , Meduloblastoma/mortalidade , Modelos Econômicos , Tratamentos com Preservação do Órgão/economia , Tratamentos com Preservação do Órgão/métodos , Órgãos em Risco/efeitos da radiação , Terapia com Prótons , Lesões por Radiação/prevenção & controle , Proteção Radiológica/economia , Dosagem Radioterapêutica , Radioterapia de Alta Energia/economia , Radioterapia de Alta Energia/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
The objective of this study was to quantify improvements in survival due to chemotherapy among children with medulloblastoma treated during the last three decades at a university unit in Greece, compare these gains with figures derived from a specialized unit in the United States and explore the role of extrinsic factors affecting survival. The records of all children with medulloblastoma (n=50) treated at the University Childhood Oncology Unit in Athens, Greece during the period 1973-2003 were reviewed. The role on survival of socio-demographic factors was studied by modeling the data through Cox's proportional-hazards regression, controlling for the mode of treatment (chemotherapy, yes vs. no), whereas survival of children with medulloblastoma treated in Greece was compared with that of 76 children treated in a specialized center in the United States during a respective period. After adjustment for demographic factors, children with medulloblastoma who received adjuvant therapy in Greece had an approximately four times higher instantaneous rate of remaining alive than those who did not (P=0.05). The 5-year survival of children with medulloblastoma treated at specialized medical centers in Greece and the United States was 66 and 63%, respectively. Despite the comparable figure with that of an acceptable standard, however, there was a suggestion (P=0.07) that a rural place of residence in Greece is a poor prognostic indicator. Assuming inherently similar age of occurrence in urban and rural areas, children from rural areas in this study had a more advanced age at diagnosis than those residing in urban Greece (mean age: 7.9 vs. 6.6 years) with a 5-year survival of 57 and 73%, respectively. As expected, incorporation of adjuvant chemotherapy in the treatment of Greek children with medulloblastoma has yielded remarkable improvement in 5-year survival, comparable to that of technologically advanced countries. On the contrary, children residing in rural areas of the country seem to enjoy less favorable prognosis, possibly owing to delays in diagnosis or limited access to optimal treatment facilities.
Assuntos
Neoplasias Cerebelares/mortalidade , Meduloblastoma/mortalidade , Adolescente , Neoplasias Cerebelares/tratamento farmacológico , Criança , Pré-Escolar , Feminino , Grécia/epidemiologia , Humanos , Lactente , Recém-Nascido , Masculino , Meduloblastoma/tratamento farmacológico , Fatores SocioeconômicosRESUMO
BACKGROUND: To investigate the value of surveillance scanning for the detection of recurrences in medulloblastoma. PROCEDURE: The charts of 95 patients with medulloblastoma were retrospectively reviewed. Information regarding the patient characteristics, treatment modalities, dates, types and results of CT and MRI studies, the frequency with which recurrences were identified on surveillance images, changes in patient management, outcome of the patients following recurrences, and survival data were analyzed. RESULTS: Thirty-one patients had a recurrence of tumor in the central nervous system; none experienced extraneural relapses. Of all recurrences, 21 were symptomatic and 10 were discovered by surveillance scans asymptomatically. None of the patients with a recurrence survived. For all 95 patients, 5-year overall and event-free survival rates were 47.1 and 49.8%, respectively. In patients with symptomatic and asymptomatic recurrences, the mean time to recurrence since initial diagnosis, the mean duration of survival post-recurrence, and the mean duration of overall follow-up were 19.2 and 26.1 months, 3.6 and 8.0 months, and 22.8 and 34.1 months, respectively. For 95 patients, 468 surveillance and 38 symptomatic images were reviewed as 313 CTs and 193 MRIs. Rate of diagnosis of recurrence per surveillance image was 2.1% (10/468). CONCLUSIONS: In our study, surveillance scanning brought no survival advantage since it detected a minority of recurrences. Longer survival achieved by early detection of recurrences might be a reflection of lead-time and length biases. Surveillance procedures will gain more importance as new effective therapeutic options are developed for recurrent medulloblastoma.
Assuntos
Neoplasias Cerebelares/diagnóstico , Imageamento por Ressonância Magnética , Meduloblastoma/diagnóstico , Recidiva Local de Neoplasia/diagnóstico , Tomografia Computadorizada por Raios X , Adolescente , Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/mortalidade , Criança , Pré-Escolar , Análise Custo-Benefício , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Masculino , Meduloblastoma/diagnóstico por imagem , Meduloblastoma/mortalidade , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida , Turquia/epidemiologiaRESUMO
Between 1975 and 1989, 98 children with brain tumours under the age of three at time of diagnosis were entered into a retrospective study. Twenty of them are alive and free of tumour more than five years after treatment and were evaluated in this study. Thirteen tumour localizations were infratentorial and 7 were supratentorial. A histological examination was performed in 15 patients: 5 ependymomas, 6 medulloblastomas and 4 astrocytomas were identified. Fifteen patients underwent surgical removal of tumour, all but one received radiotherapy and 8 were given chemotherapy. Only two children have not late effects. Analysis of long-term sequelae in survivors showed central endocrinopathies in 14 (70%), a neurological handicap in 13 (65%) and impaired cognitive functions in 17 (85%). Irradiation was clearly responsible for mental sequelae in 7 patients and endocrinopathies in 6 patients. The other possible causes are tumour injury, hydrocephalus or surgery. The risks incurred with radiotherapy and advances in infant brain tumour therapy are discussed.