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1.
Pediatr Blood Cancer ; 66(10): e27910, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31264356

RESUMO

BACKGROUND: A previous study based on Norwegian Cancer Registry data suggested regional differences in overall survival (OS) after treatment for medulloblastoma (MB) and supratentorial primitive neuroectodermal tumor (CNS-PNET) in Norway. The purpose of the present study was to confirm in an extended cohort whether there were regional differences in outcome or not, and if so try to identify possible explanations. MATERIAL AND METHODS: Data from patients aged 0-20 years diagnosed with and treated for MB/CNS-PNET at all four university hospitals in Norway from 1974 to 2013 were collected and compared. RESULTS: Of 266 identified patients, 251 fulfilled inclusion criteria. MB was diagnosed in 200 and CNS-PNET in 51 patients. Five-year OS and event-free survival (EFS) were 59% and 52%, respectively. There was a significant difference in five-year OS and EFS between MB and CNS-PNET patients; 62% versus 47% (P =  0.007) and 57% versus 35% (P < 0.001). In multivariable analysis, two factors were found to significantly contribute to improved five-year OS and EFS, whereas one factor contributed to improved five-year OS only. Gross total resection (GTR) versus non-GTR (hazard ratio [HR] 0.53, P =  0.003; HR 0.46, P < 0.001) and cerebrospinal irradiation (CSI) versus non-CSI (HR 0.24, P < 0.001; HR 0.28, P < 0.001) for both, and treatment outside Oslo University Hospital for OS only (HR 0.64, P =  0.048). CONCLUSION: Survival was comparable with data from other population-based studies, and the importance of GTR and CSI was confirmed. The cause for regional survival differences could not be identified.


Assuntos
Neoplasias Cerebelares/mortalidade , Meduloblastoma/mortalidade , Tumores Neuroectodérmicos Primitivos/mortalidade , Neoplasias Supratentoriais/mortalidade , Adolescente , Neoplasias Cerebelares/terapia , Criança , Pré-Escolar , Terapia Combinada/métodos , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Meduloblastoma/terapia , Tumores Neuroectodérmicos Primitivos/terapia , Noruega/epidemiologia , Estudos Retrospectivos , Neoplasias Supratentoriais/terapia , Análise de Sobrevida , Resultado do Tratamento , Adulto Jovem
2.
Cancer Radiother ; 23(3): 179-187, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31109839

RESUMO

PURPOSE: Medulloblastoma is the most common primary malignant central nervous system tumour in children. These last decades, treatment modalities have largely evolved resulting in better survival rates. Nevertheless, long-term toxicity is a major concern in this setting. The purpose of this study was to analyse the clinical results and medical outcomes of a cohort of paediatric patients treated for medulloblastoma in Xhinhua Hospital in Shanghai. These results are compared with those from other centres reported in literature. PATIENTS AND METHODS: This was a retrospective study conducted at Xhinhua Hospital in Shanghai, China. It included 121 patients treated for medulloblastoma from 1993 to December 2013. RESULTS: Mean age at diagnosis was 6.7 years (range: 1-14.3 years). Total surgical resection was achieved in 60% of the cases. Classic medulloblastoma was found in 59% of the cases. Adjuvant radiotherapy was delivered in all cases and chemotherapy concerned 70.2% of the studied cohort. The median follow-up time of the study was 84 months (range: 24-120 months). Five- and 10 years progression-free survival rates were 83.2%, and 69.5% and 5 years and 10 years. Overall survival rates were 82.5%, and 72.5%. Patient's age significantly influenced survival: patients under 3 years old had the worse outcomes (P=0.01). T and M stages also significantly impacted survival rates: advanced stages were associated with lower rates (P=0.08 and 0.05 respectively). Finally, patients receiving temezolomide had bad outcomes when compared to the new standard protocol used in the department (P=0.03). The most commonly reported late toxicity was growth suppression in 35 patients (52.2%). Hypothyroidism requiring hormone replacement was recorded in 29% of the cases. Hearing loss, and problems including poor concentration, poor memory and learning difficulties were reported in 19% and 25% of the cases respectively. Second cancers were noted in three cases. CONCLUSION: Overall, our results are comparable to those reported in literature. Nevertheless, efforts should be made to ensure longer follow-ups and correctly assess treatment-related toxicity.


Assuntos
Neoplasias Cerebelares/terapia , Meduloblastoma/terapia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
3.
J Clin Neurosci ; 64: 33-35, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30905661

RESUMO

BACKGROUND: To describe an exceptional case of late recurrence of medulloblastoma after 17 years of complete remission. CASE DESCRIPTION: A 42-year-old male consulted in ER for 10-day occipital headache. He had a previous history of cerebellar medulloblastoma 17 years ago treated with gross total resection, chemotherapy and radiotherapy. During his yearly follow-up he had maintained complete remission. MRi showed a cerebellar mass suggestive of medulloblastoma recurrence vs radio-induced tumor. Craniotomy and complete resection of the tumor was performed. The anatomopathological analysis confirmed the recurrence of medulloblastoma. The patient received high dose of adjuvant chemotherapy and he maintains complete remission after 18 months. CONCLUSION: Recurrence of medulloblastoma may occur despite more than 15 years of complete remission. Because of this fact it is mandatory to continue the follow-up of these patients. Aggressive management of recurrence is recommended in absence of disease dissemination.


Assuntos
Neoplasias Cerebelares/patologia , Meduloblastoma/patologia , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/patologia , Adulto , Neoplasias Cerebelares/terapia , Quimiorradioterapia Adjuvante/métodos , Quimioterapia Adjuvante/métodos , Terapia Combinada/métodos , Humanos , Masculino , Meduloblastoma/terapia , Indução de Remissão
4.
J Clin Oncol ; 37(9): 731-740, 2019 03 20.
Artigo em Inglês | MEDLINE | ID: mdl-30730781

RESUMO

PURPOSE: Treatment of medulloblastoma has evolved from surgery and radiotherapy to contemporary multimodal regimens. However, the impact on long-term health outcomes remains unknown. METHODS: Cumulative incidence of late mortality (5 or more years from diagnosis), subsequent neoplasms (SNs), and chronic health conditions were evaluated in the Childhood Cancer Survivor Study among 5-year survivors of medulloblastoma diagnosed between 1970 and 1999. Outcomes were evaluated by treatment exposure, including historical therapy (craniospinal irradiation [CSI] ≥ 30 Gy, no chemotherapy), high risk (CSI ≥ 30 Gy + chemotherapy), standard risk (CSI < 30 Gy + chemotherapy), and by treatment decade (1970s, 1980s, 1990s). Rate ratios (RRs) and 95% CIs estimated long-term outcomes using multivariable piecewise exponential models. RESULTS: Among 1,311 eligible survivors (median age, 29 years [range, 6 to 60 years]; median time from diagnosis, 21 years [range, 5 to 44 years]), the 15-year cumulative incidence rate of all-cause late mortality was 23.2% (diagnosed 1970s) versus 12.8% (1990s; P = .002), with a recurrence-related mortality rate of 17.7% versus 9.6% ( P = .008). Lower late mortality rates as a result of other health-related causes were not observed. Among 997 survivors who completed a baseline survey, the 15-year cumulative incidence of SNs was higher among survivors with multimodal therapy (standard risk, 9.5%; historical, 2.8%; P = .03). Survivors treated in the 1990s had a higher cumulative incidence of severe, disabling, life-threatening, and fatal chronic health conditions (56.5% in 1990s v 39.9% in 1970s; P < .001) and were more likely to develop multiple conditions (RR, 2.89; 95% CI, 1.31 to 6.38). However, survivors of standard-risk therapy were less likely to use special education services than high-risk therapy survivors (RR, 0.84; 95% CI, 0.75 to 0.93). CONCLUSION: Historical changes in medulloblastoma therapy that improved 5-year survival have increased the risk for SNs and debilitating health conditions for survivors yet reduced the need for special education services.


Assuntos
Sobreviventes de Câncer/estatística & dados numéricos , Neoplasias Cerebelares/terapia , Meduloblastoma/terapia , Inquéritos e Questionários , Adolescente , Adulto , Neoplasias Cerebelares/mortalidade , Quimiorradioterapia/métodos , Criança , Tratamento Farmacológico/métodos , Feminino , Humanos , Masculino , Meduloblastoma/mortalidade , Pessoa de Meia-Idade , Morbidade , Recidiva Local de Neoplasia , Segunda Neoplasia Primária/diagnóstico , Segunda Neoplasia Primária/epidemiologia , /estatística & dados numéricos , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Adulto Jovem
5.
World Neurosurg ; 127: e58-e64, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30797919

RESUMO

OBJECTIVE: To study the prevalence of OTX1 and OTX2 gene expression in 60 medulloblastoma specimen samples and to establish correlations between gene expression and clinical and histopathological aspects. METHODS: We performed a retrospective analysis of 60 patients with a diagnosis of medulloblastoma at the Clinicas Hospital of the School of Medicine, University of São Paulo, and the Cancer Hospital of Barretos. We created a database of the 60 patients containing information on the gene expression of OTX1 and OTX2 (obtained using real-time polymerase chain reaction) and clinical and epidemiological data. Statistical tests were performed to verify potential correlations of clinicopathological data and follow-up aspects with gene expression. RESULTS: The OTX1 gene was expressed in 52% of the study population. Expression varied with age (higher in adults), location (predominantly by hemisphere), and histological type (desmoplastic). The OTX2 gene was expressed in 62% of the study population. Expression varied with age (higher in younger age groups), location (predominantly vermis), and histological type (classic and anaplastic). A statistical correlation between OTX2 gene expression and the development of leptomeningeal metastases was observed. CONCLUSIONS: The relative expression of OTX1 and OTX2 was dependent on patient age, tumor location, and histological variant. In addition, OTX2 expression might be a predictive factor for leptomeningeal metastases of medulloblastoma. The OTX pathway should be consider as an important venue for medulloblastomas development.


Assuntos
Neoplasias Cerebelares/genética , Meduloblastoma/genética , Proteínas de Neoplasias/genética , Fatores de Transcrição Otx/genética , Adolescente , Adulto , Fatores Etários , Brasil , Neoplasias Cerebelares/epidemiologia , Neoplasias Cerebelares/terapia , Criança , Pré-Escolar , Feminino , Regulação Neoplásica da Expressão Gênica , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Meduloblastoma/epidemiologia , Meduloblastoma/secundário , Meduloblastoma/terapia , Neoplasias Meníngeas/secundário , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase em Tempo Real , Estudos Retrospectivos , Adulto Jovem
6.
DNA Repair (Amst) ; 74: 70-79, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30606609

RESUMO

DSBs are harmful lesions produced through endogenous metabolism or by exogenous agents such as ionizing radiation, that can trigger genomic rearrangements. We have recently shown that exposure to 2 Gy of X-rays has opposite effects on the induction of Shh-dependent MB in NHEJ- and HR-deficient Ptch1+/- mice. In the current study we provide a comprehensive link on the role of HR/NHEJ at low doses (0.042 and 0.25 Gy) from the early molecular changes through DNA damage processing, up to the late consequences of their inactivation on tumorigenesis. Our data indicate a prominent role for HR in genome stability, by preventing spontaneous and radiation-induced oncogenic damage in neural precursors of the cerebellum, the cell of origin of MB. Instead, loss of DNA-PKcs function increased DSBs and apoptosis in neural precursors of the developing cerebellum, leading to killing of tumor initiating cells, and suppression of MB tumorigenesis in DNA-PKcs-/-/Ptch1+/- mice. Pathway analysis demonstrates that DNA-PKcs genetic inactivation confers a remarkable radiation hypersensitivity, as even extremely low radiation doses may deregulate many DDR genes, also triggering p53 pathway activation and cell cycle arrest. Finally, by showing that DNA-PKcs inhibition by NU7441 radiosensitizes human MB cells, our in vitro findings suggest the inclusion of MB in the list of tumors beneficiating from the combination of radiotherapy and DNA-PKcs targeting, holding promise for clinical translation.


Assuntos
Neoplasias Cerebelares/genética , Reparo do DNA/efeitos da radiação , Meduloblastoma/genética , Neoplasias Induzidas por Radiação/genética , Receptor Patched-1/deficiência , Receptor Patched-1/metabolismo , Animais , Carcinogênese/genética , Carcinogênese/efeitos da radiação , Linhagem Celular Tumoral , Neoplasias Cerebelares/metabolismo , Neoplasias Cerebelares/patologia , Neoplasias Cerebelares/terapia , Dano ao DNA , Reparo do DNA por Junção de Extremidades/efeitos da radiação , DNA Helicases/genética , Proteína Quinase Ativada por DNA/deficiência , Proteínas de Ligação a DNA/deficiência , Relação Dose-Resposta à Radiação , Recombinação Homóloga/efeitos da radiação , Humanos , Meduloblastoma/metabolismo , Meduloblastoma/patologia , Meduloblastoma/terapia , Camundongos , Terapia de Alvo Molecular , Mutação , Neoplasias Induzidas por Radiação/metabolismo , Neoplasias Induzidas por Radiação/patologia , Neoplasias Induzidas por Radiação/terapia , Proteínas Nucleares/deficiência , Proteínas Nucleares/genética , Risco , Raios X/efeitos adversos
7.
Nat Commun ; 10(1): 332, 2019 01 18.
Artigo em Inglês | MEDLINE | ID: mdl-30659187

RESUMO

Drugs that modify the epigenome are powerful tools for treating cancer, but these drugs often have pleiotropic effects, and identifying patients who will benefit from them remains a major clinical challenge. Here we show that medulloblastomas driven by the transcription factor Gfi1 are exquisitely dependent on the enzyme lysine demethylase 1 (Kdm1a/Lsd1). We demonstrate that Lsd1 physically associates with Gfi1, and that these proteins cooperate to inhibit genes involved in neuronal commitment and differentiation. We also show that Lsd1 is essential for Gfi1-mediated transformation: Gfi1 proteins that cannot recruit Lsd1 are unable to drive tumorigenesis, and genetic ablation of Lsd1 markedly impairs tumor growth in vivo. Finally, pharmacological inhibitors of Lsd1 potently inhibit growth of Gfi1-driven tumors. These studies provide important insight into the mechanisms by which Gfi1 contributes to tumorigenesis, and identify Lsd1 inhibitors as promising therapeutic agents for Gfi1-driven medulloblastoma.


Assuntos
Carcinogênese/efeitos dos fármacos , Neoplasias Cerebelares/patologia , Proteínas de Ligação a DNA/metabolismo , Histona Desmetilases/metabolismo , Meduloblastoma/patologia , Fatores de Transcrição/metabolismo , Animais , Antibióticos Antineoplásicos/uso terapêutico , Proliferação de Células/efeitos dos fármacos , Neoplasias Cerebelares/genética , Neoplasias Cerebelares/terapia , Proteínas de Ligação a DNA/genética , Doxorrubicina/uso terapêutico , Regulação Neoplásica da Expressão Gênica , Células HEK293 , Histona Desmetilases/genética , Humanos , Meduloblastoma/genética , Meduloblastoma/terapia , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Camundongos SCID , Células NIH 3T3 , Transplante de Neoplasias , Vírus Oncogênicos , Retroviridae , Fatores de Transcrição/genética
8.
J Oncol Pharm Pract ; 25(3): 689-693, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30185131

RESUMO

Thiotepa, a highly lipophilic, alkylating agent, and/or its active metabolites may be excreted in part via skin in patients receiving high-dose therapy. We present a case of cutaneous toxicity observed in a 4.5-year-old girl patient with medulloblastoma treated with a high-dose thiotepa conditioning regimen before autologous stem cell transplantation. Skin lesions, as well as their pattern and locations, were evocative of thiotepa toxidermia. After the case herein described, preventive care guidelines were implemented in our unit as from 2014. A retrospective follow-up of 26 pediatric patients receiving thiotepa prior to stem cell transplantation was performed until March 2018. In this series of patients, only one patient experienced cutaneous toxicity as reported herein. Thereafter, only mild cutaneous toxicity was observed, even with double or triple transplantation protocols with high-dose thiotepa. Clear preventive care instructions should be detailed in the Summary of Product Characteristics in order to minimize the cutaneous toxicity of thiotepa.


Assuntos
Neoplasias Cerebelares/terapia , Erupção por Droga/etiologia , Meduloblastoma/terapia , Tiotepa/efeitos adversos , Adolescente , Criança , Pré-Escolar , Terapia Combinada , Feminino , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Lactente , Masculino , Estudos Retrospectivos , Tiotepa/administração & dosagem , Condicionamento Pré-Transplante , Transplante Autólogo
11.
J Med Case Rep ; 12(1): 342, 2018 Nov 18.
Artigo em Inglês | MEDLINE | ID: mdl-30447689

RESUMO

BACKGROUND: Metastasis to the pituitary gland is an infrequent clinical problem, and the symptoms caused by metastases have been reported in only 2.5-18.2% of the cases. However, metastasis to the suprasellar lesion has rarely been reported in the literature. To the best of our knowledge, only nine cases of hypothalamic hypopituitarism due to metastases of solid tumors have been reported in English-language journals. CASE PRESENTATION: A 67-year-old Japanese man presented to our hospital with generalized weakness, lethargy, and weight loss. Laboratory data showed hypoglycemia together with low thyroid-stimulating hormone and free thyroxine. We suspected hypopituitarism and performed imaging of the head, which revealed multiple tumors, one of which was in the suprasellar region. Computed tomography of the chest showed a tumor shadow, and a bronchoscopic biopsy pathologically showed small cell lung cancer. Hormone profiling demonstrated hypothalamic pan-hypopituitarism. We diagnosed hypothalamic hypopituitarism secondary to metastases from the primary lung cancer and initiated radiation, chemotherapy, and hormone replacement, but the patient died 10 months later. CONCLUSIONS: We report a case of a 67-year-old man with hypothalamic hypopituitarism secondary to a suprasellar metastasis from a primary small cell lung cancer, and we review ten cases of hypothalamic hypopituitarism due to metastases, including our patient. Recognizing hypopituitarism can be challenging, especially in the elderly, whose symptoms such as lethargy and visual decline may be mistaken for the natural aging process. In patients with established metastatic conditions, the symptoms may be wrongly attributed to malignancy or to the side effects of therapy. When a patient is suspected of having hypopituitarism, a hormone load test can help to diagnose the type of hypopituitarism. It is important to evaluate the brain and the whole body to confirm whether metastasis and primary cancer exist. Because the mortality rate is very high, aggressive intervention for both diagnosis and therapy is required in cases of hypothalamic hypopituitarism secondary to tumor metastasis.


Assuntos
Neoplasias Cerebelares/secundário , Hipopituitarismo/patologia , Hipotálamo/patologia , Carcinoma de Pequenas Células do Pulmão/complicações , Idoso , Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/terapia , Evolução Fatal , Terapia de Reposição Hormonal , Humanos , Hipopituitarismo/diagnóstico por imagem , Hipopituitarismo/etiologia , Hipopituitarismo/terapia , Masculino , Carcinoma de Pequenas Células do Pulmão/diagnóstico por imagem , Carcinoma de Pequenas Células do Pulmão/patologia , Tomografia Computadorizada por Raios X
12.
J BUON ; 23(4): 1156-1162, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30358225

RESUMO

PURPOSE: The aim of this study was to present the management and treatment of children with medulloblastoma in Serbia, a middle-income country (MIC). METHODS: The data of 87 children diagnosed with medulloblastoma and treated at the Institute for Oncology and Radiology of Serbia from 2000 to 2013 were analyzed. RESULTS: The children's median age was 8.3 years (range 2.5-17.3). Eighty-two (94.2%) were 3 years or older. Sixtytwo (71.3%) patients had stage M0 medulloblastoma, 12 (13.8%) had stage M1 and 13 (14.9%) had stage M2 or M3. As of October 2015, 51 (58.6%) patients were alive and 31 (35.6%) had died. Five patients (5.7%) were lost to followup. Twenty-six patients relapsed. The median follow-up time was 58 months (range 4-187). Mean overall survival (OS) was 76.4% at 3 years, 66.2% at 5 years and 59.2% at 10 years. Mean disease-free survival (DFS) was 75.8% at 3 years, 62.8% at 5 years and 56.6% at 10 years. Mean OS of stage M0 patients was 86.4% at 3 years, 74% at 5 years and 63.1% at 10 years. The OS of stage M1, M2 and M3 patients combined was 48.9% at 3 years, 44.0% at 5 years and 37.7% at 10 years. CONCLUSION: In Serbia, a MIC, it is possible to achieve good treatment results in children with medulloblastoma using international treatment guidelines and recommendations, available resources and an experienced team of professionals dedicated to pediatric neurooncology.


Assuntos
Neoplasias Cerebelares/terapia , Meduloblastoma/terapia , Adolescente , Neoplasias Cerebelares/mortalidade , Neoplasias Cerebelares/patologia , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Meduloblastoma/mortalidade , Meduloblastoma/patologia , Estadiamento de Neoplasias , Prognóstico , Sérvia/epidemiologia , Análise de Sobrevida , Resultado do Tratamento
13.
World Neurosurg ; 120: 131-137, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30172975

RESUMO

BACKGROUND: Cerebellar liponeurocytoma (cLNC) is a rare benign glioneuronal tumor with only ~60 cases reported since its first description in 1978. They have occurred largely as sporadic cases; however, familial cases with possible autosomal dominant inheritance have also been reported. Surgical excision has been considered the main modality of treatment, even for recurrent lesions. Uncertainties exist regarding the natural history, long-term outcomes, and optimal postoperative management and follow-up protocols for cLNC in general and multifocal cLNC in particular. Multifocality is exceedingly rare, with only 4 cases reported previously. CASE DESCRIPTION: We present a case of multifocal cLNC in a young woman who had presented with progressive cerebellar symptoms of 5 months' duration. Her diagnostic neuroimaging evaluations revealed 2 asymmetric mass lesions (1 on each cerebellar hemisphere). We excised the larger lesion to relieve the mass effect and decided to monitor the smaller lesion radiologically. However, she developed a new lesion during the follow-up period and required adjuvant radiotherapy. CONCLUSION: Surgical excision forms the cornerstone of management of cLNCs. However, it is necessary to vigilantly monitor these patients owing to the high recurrence rates of cLNC despite the innocuous histologic features.


Assuntos
Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/terapia , Neurocitoma/diagnóstico por imagem , Neurocitoma/terapia , Adulto , Neoplasias Cerebelares/patologia , Feminino , Humanos , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Neurocitoma/patologia
14.
Eur J Cancer Care (Engl) ; 27(6): e12912, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30204287

RESUMO

Children with medulloblastoma (MB) are predisposed for negative cognitive sequela, which has been widely identified in this population. The purpose of the present study was to explore cognitive deficits and psychopathological symptoms and analyse their relation among MB survivors. The Wechsler Intelligence Scale for Children and the Mini International Neuropsychiatric Interview (MINI-KID) was administered to 34 MB survivors to measure cognitive functioning and psychopathological symptoms. The MB survivors had lower global IQ (86.41 [79.70-93.13]) compared with the control population mean. We found impaired functioning in all IQ subscales in the MB survivors group, of which processing speed (84.15 [77.71-90.58]) was the most affected. Higher radiation dose and high-dose chemotherapy with stem cell rescue were significantly associated factors for lowered global IQ, while age at diagnosis, sex and time period from diagnosis were not significantly associated. Compulsive disorder, generalised anxiety, separation anxiety and posttraumatic stress disorder were significantly more prevalent in the MB survivor group than a group of 46 control participants. No correlation was found between the cognitive deficits and the psychopathological symptoms. Our results identify that MB survivors suffer from cognitive and psychopathological impairments, and these could exist independently from each other.


Assuntos
Antineoplásicos/administração & dosagem , Ansiedade de Separação/psicologia , Sobreviventes de Câncer/psicologia , Neoplasias Cerebelares/terapia , Disfunção Cognitiva/psicologia , Irradiação Craniana , Meduloblastoma/terapia , Transtorno Obsessivo-Compulsivo/psicologia , Transtornos de Estresse Pós-Traumáticos/psicologia , Adolescente , Fatores Etários , Transtornos de Ansiedade/psicologia , Estudos de Casos e Controles , Neoplasias Cerebelares/psicologia , Quimiorradioterapia , Criança , Feminino , Humanos , Masculino , Meduloblastoma/psicologia , Doses de Radiação , Transplante de Células-Tronco , Escalas de Wechsler
15.
Neurol Clin ; 36(3): 533-556, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-30072070

RESUMO

Pediatric central nervous system (CNS) tumors are the most common solid tumors in children and comprise 15% to 20% of all malignancies in children. Presentation, symptoms, and signs depend on tumor location and age of the patient at the time of diagnosis. This article summarizes the common childhood CNS tumors, presentations, classification, and recent updates in treatment approaches due to the increased understanding of the molecular pathogenesis of pediatric brain tumors.


Assuntos
Neoplasias Encefálicas/epidemiologia , Neoplasias Encefálicas/patologia , Adolescente , Neoplasias Encefálicas/terapia , Neoplasias Cerebelares/epidemiologia , Neoplasias Cerebelares/patologia , Neoplasias Cerebelares/terapia , Criança , Pré-Escolar , Feminino , Glioma/epidemiologia , Glioma/patologia , Glioma/terapia , Humanos , Lactente , Recém-Nascido , Masculino , Tumor Rabdoide/epidemiologia , Tumor Rabdoide/patologia , Tumor Rabdoide/terapia , Teratoma/epidemiologia , Teratoma/patologia , Teratoma/terapia
16.
Medicine (Baltimore) ; 97(31): e11329, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30075500

RESUMO

RATIONALE: Pilocytic astrocytoma (PA) is the most common pediatric central nervous system glial tumor. Adult occurrence is rare, especially in elderly adults. How to manage the pilocytic astrocytoma with spontaneous intratumoral hemorrhage in the elderly is still unfamiliar with clinician. PATIENT CONCERNS: Reports of tumors with intratumoral hemorrhage in elderly adults are extremely rare. We report a case of a 62-year-old male diagnosed with cerebellar pilocytic astrocytomas with spontaneous intratumoral hemorrhage. Informed consent was obtained from the patient. DIAGNOSES: Histological examination of the specimens revealed pilocytic astrocytomas, including a hemorrhagic portion. INTERVENTIONS: The patient underwent the radical resections of tumour and was given the regular follow-up. OUTCOMES: 12 months later, he recovered without evidence of disease. LESSONS: Cerebellar pilocytic astrocytomas associated with hemorrhages occur over the age of 60 is extremely rare. Removing the tumor and hematoma completely is the best treatment options. Long term follow-up is very necessary for this tumor.


Assuntos
Astrocitoma/complicações , Astrocitoma/diagnóstico , Neoplasias Cerebelares/complicações , Neoplasias Cerebelares/diagnóstico , Hemorragia Cerebral/etiologia , Idoso , Astrocitoma/terapia , Neoplasias Cerebelares/terapia , Hemorragia Cerebral/diagnóstico , Hemorragia Cerebral/terapia , Humanos , Masculino , Pessoa de Meia-Idade
17.
World Neurosurg ; 117: 344-349, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29966776

RESUMO

BACKGROUND: Lhermitte-Duclos disease is an extremely rare pathologic entity characterized by a cerebellar mass composed of enlarged cerebellar folia containing abnormal ganglion cells. This entity usually presents in young and middle-aged adults and rarely in children. There is no study in the literature analyzing the long-term clinical course of this disease to assess the behavior primarily because of its rarity. CASE DESCRIPTION: We present our experience with a 7-year-old patient of Lhermitte-Duclos disease who was followed up for 5 years and found to have progressed to bilateral World Health Organization grade IV medulloblastoma. This case denotes the malignant potential of this rare disorder. CONCLUSIONS: LDD is seen rarely and demands a high degree of suspicion in patients presenting with cerebellar mass and/or imaging characteristics. It is prudent to keep these patients in close follow-up for early detection of malignant transformation.


Assuntos
Neoplasias Cerebelares/fisiopatologia , Síndrome do Hamartoma Múltiplo/fisiopatologia , Meduloblastoma/fisiopatologia , Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/patologia , Neoplasias Cerebelares/terapia , Criança , Progressão da Doença , Síndrome do Hamartoma Múltiplo/diagnóstico por imagem , Síndrome do Hamartoma Múltiplo/tratamento farmacológico , Humanos , Masculino , Meduloblastoma/diagnóstico por imagem , Meduloblastoma/patologia , Meduloblastoma/terapia
18.
Sci Rep ; 8(1): 11144, 2018 07 24.
Artigo em Inglês | MEDLINE | ID: mdl-30042517

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 , /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 Jovem
19.
Handb Clin Neurol ; 155: 289-299, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29891066

RESUMO

The cerebellum is the most common site of presentation of central nervous system tumors in children but exceedingly rare in adults. Children often present with acute symptoms related to increased intracranial pressure, requiring urgent surgical intervention. The differential diagnosis is broad and includes a variety of benign and malignant entities. Cerebellar low-grade gliomas are the most common and benign, slow-growing tumors, for which surgical resection alone is curative. Embryonal tumors, on the other hand - most commonly medulloblastomas - are highly aggressive and treatment includes intensive postsurgical radiotherapy and chemotherapy. Driven by multiple genomewide profiling studies, the field of neuro-oncology is making great strides towards understanding how different tumors develop and embarking on a new generation of molecularly informed clinical trials.


Assuntos
Neoplasias Cerebelares/terapia , Meduloblastoma/terapia , Antineoplásicos/uso terapêutico , Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/epidemiologia , Humanos , Imagem por Ressonância Magnética , Meduloblastoma/diagnóstico por imagem , Meduloblastoma/epidemiologia , Radioterapia/métodos
20.
J Natl Compr Canc Netw ; 16(6): 683-691, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29891519

RESUMO

Metachronous neoplasms have rarely been reported in patients with neuroblastoma. This report presents the clinical case of a 23-month-old child who was diagnosed with an anaplastic medulloblastoma 5 months after completing treatment for stage IV neuroblastoma. The patient was treated with complete surgical resection and adjuvant chemoradiation followed by maintenance chemotherapy at an outside institution and came to our institution for further management. A pathologic diagnosis and review of both the suprarenal and posterior fossa masses were performed, as well as a genetic analysis of both cerebellar tumor tissue and blood using next-generation gene sequencing. At our institution, the patient was submitted to induction chemotherapy followed by high-dose chemotherapy and autologous stem cell transplantation and remains free of disease 2 years after completion of treatment. Genetic analysis revealed multiple somatic copy number variations with most deleted genes located in 2q37, a region which harbors genes involved in epigenetic regulation and tumor suppression. A homozygous deletion was found in the TSC2 gene, which is a clinically actionable gene, and patients with activating deletions in TSC2 can potentially be eligible for basket clinical trials with mTOR inhibitors. Germline single nucleotide variants were also identified in multiple genes involved in cancer (ALK, FGFR3, FLT3/4, HNF1A, NCOR1, and NOTCH2/3), cancer predisposition (TP53, TSC1, and BRCA1/2), and genes involved in DNA repair (MSH6, PMS2, POLE, and ATM). Metachronous neoplasms are rare and challenging to treat, hence genetic analysis and referral are needed to exclude hereditary cause. DNA sequencing of the tumor and germline can help identify alterations that increase predisposition or can be used to guide treatment decisions on recurrence and when standard options fail.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Cerebelares/diagnóstico , Meduloblastoma/diagnóstico , Segunda Neoplasia Primária/diagnóstico , Neuroblastoma/terapia , Proteína 2 do Complexo Esclerose Tuberosa/genética , Neoplasias Cerebelares/genética , Neoplasias Cerebelares/patologia , Neoplasias Cerebelares/terapia , Quimiorradioterapia/métodos , Feminino , Humanos , Lactente , Imagem por Ressonância Magnética , Meduloblastoma/genética , Meduloblastoma/patologia , Meduloblastoma/terapia , Segunda Neoplasia Primária/genética , Segunda Neoplasia Primária/patologia , Segunda Neoplasia Primária/terapia , Neuroblastoma/genética , Análise de Sequência de DNA
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