RESUMO
PURPOSE: Meningeal solitary fibrous tumour (SFT) and haemangiopericytoma (HPC) are uncommon tumours that have been merged into a single entity in the last 2021 WHO Classification of Tumors of the Central Nervous System. To describe the epidemiology of SFT/HPC operated in France and, to assess their incidence. METHODS: We processed the French Brain Tumour Database (FBTDB) to conduct a nationwide population-based study of all histopathologically confirmed SFT/HPC between 2006 and 2015. RESULTS: Our study included 399 SFT/HPC patients, operated in France between 2006 and 2015, in one of the 46 participating neurosurgical centres. The incidence reached 0.062, 95%CI[0.056-0.068] for 100,000 person-years. SFT accounted for 35.8% and, HPC for 64.2%. The ratio of SFT/HPC over meningioma operated during the same period was 0.013. SFT/HPC are about equally distributed in women and men (55.9% vs. 44.1%). For the whole population, mean age at surgery was 53.9 (SD ± 15.8) years. The incidence of SFT/HPC surgery increases with the age and, is maximal for the 50-55 years category. Benign SFT/HPC accounted for 65.16%, SFT/HPC of uncertain behaviour for 11.53% and malignant ones for 23.31%. The number of resection progresses as the histopathological behaviour became more aggressive. 6.7% of the patients with a benign SFT/HPC had a second surgery vs.16.6% in case of uncertain behaviour and, 28.4% for malignant SFT/HPC patients. CONCLUSION: Meningeal SFT and HPC are rare CNS mesenchymal tumours which both share common epidemiological characteristics, asserting their merging under a common entity. SFT/HPC incidence is less that one case for 1 billion per year and, for around 100 meningiomas-like tumours removed, one SFT/HPC may be diagnosed. SFT/HPC are equally distributed in women and men and, are mainly diagnosed around 50-55 years. The more aggressive the tumour, the higher the probability of recurrence.
Assuntos
Hemangiopericitoma , Neoplasias Meníngeas , Tumores Fibrosos Solitários , Humanos , França/epidemiologia , Hemangiopericitoma/epidemiologia , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Hemangiopericitoma/diagnóstico , Feminino , Masculino , Pessoa de Meia-Idade , Neoplasias Meníngeas/epidemiologia , Neoplasias Meníngeas/patologia , Neoplasias Meníngeas/cirurgia , Neoplasias Meníngeas/diagnóstico , Tumores Fibrosos Solitários/epidemiologia , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia , Tumores Fibrosos Solitários/diagnóstico , Adulto , Idoso , Incidência , Adulto Jovem , Meningioma/epidemiologia , Meningioma/patologia , Meningioma/cirurgia , Meningioma/diagnóstico , Adolescente , Idoso de 80 Anos ou mais , CriançaRESUMO
BACKGROUND: Endolymphatic sac tumors (ELSTs) are, with a prevalence of up to 16%, a component of von Hippel-Lindau (VHL) disease. Data from international registries regarding heritable fraction and characteristics, germline VHL mutation frequency, and prevalence are lacking. METHODS: Systematic registration of ELSTs from international centers of otorhinolaryngology and from multidisciplinary VHL centers' registries was performed. Molecular genetic analyses of the VHL gene were offered to all patients. RESULTS: Our population-based registry comprised 93 patients with ELST and 1789 patients with VHL. The prevalence of VHL germline mutations in apparently sporadic ELSTs was 39%. The prevalence of ELSTs in patients with VHL was 3.6%. ELST was the initial manifestation in 32% of patients with VHL-ELST. CONCLUSION: Prevalence of ELST in VHL disease is much lower compared to the literature. VHL-associated ELSTs can be the first presentation of the syndrome and mimic sporadic tumors, thus emphasizing the need of molecular testing in all presentations of ELST. © 2015 Wiley Periodicals, Inc. Head Neck 38: 673-679, 2016.
Assuntos
Neoplasias da Orelha/patologia , Saco Endolinfático/patologia , Doença de von Hippel-Lindau/complicações , Adolescente , Adulto , Idoso , Criança , Feminino , Mutação em Linhagem Germinativa , Humanos , Masculino , Pessoa de Meia-Idade , Sistema de Registros , Proteína Supressora de Tumor Von Hippel-Lindau/genética , Adulto JovemRESUMO
Forestier's disease, also known as diffuse idiopathic skeletal hyperostosis (DISH), is a pathology of vertebral bodies characterised by exuberant osteophytis formation. Forestier's disease is usually managed conservatively. Surgical resection of the osteophytes is reported to be an effective treatment for severe cases and/ or cases with airway obstruction. We report a 55-year-old man presenting with 6 months' progressive dysphagia and dysphonia. He was managed successfully with an anterior cervical osteophytectomy without fusion. A literature review is included.
Assuntos
Transtornos de Deglutição/complicações , Disfonia/complicações , Hiperostose Esquelética Difusa Idiopática/complicações , Idoso , Transtornos de Deglutição/diagnóstico , Disfonia/diagnóstico , Humanos , Hiperostose Esquelética Difusa Idiopática/diagnóstico , MasculinoRESUMO
PURPOSE: To develop a tumor growth inhibition model for adult diffuse low-grade gliomas (LGG) able to describe tumor size evolution in patients treated with chemotherapy or radiotherapy. EXPERIMENTAL DESIGN: Using longitudinal mean tumor diameter (MTD) data from 21 patients treated with first-line procarbazine, 1-(2-chloroethyl)-3-cyclohexyl-l-nitrosourea, and vincristine (PCV) chemotherapy, we formulated a model consisting of a system of differential equations, incorporating tumor-specific and treatment-related parameters that reflect the response of proliferative and quiescent tumor tissue to treatment. The model was then applied to the analysis of longitudinal tumor size data in 24 patients treated with first-line temozolomide (TMZ) chemotherapy and in 25 patients treated with first-line radiotherapy. RESULTS: The model successfully described the MTD dynamics of LGG before, during, and after PCV chemotherapy. Using the same model structure, we were also able to successfully describe the MTD dynamics in LGG patients treated with TMZ chemotherapy or radiotherapy. Tumor-specific parameters were found to be consistent across the three treatment modalities. The model is robust to sensitivity analysis, and preliminary results suggest that it can predict treatment response on the basis of pretreatment tumor size data. CONCLUSIONS: Using MTD data, we propose a tumor growth inhibition model able to describe LGG tumor size evolution in patients treated with chemotherapy or radiotherapy. In the future, this model might be used to predict treatment efficacy in LGG patients and could constitute a rational tool to conceive more effective chemotherapy schedules.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Glioma/terapia , Modelos Biológicos , Adolescente , Algoritmos , Criança , Pré-Escolar , Feminino , Glioma/tratamento farmacológico , Glioma/radioterapia , Humanos , Masculino , Estadiamento de Neoplasias , Reprodutibilidade dos Testes , Carga Tumoral , Adulto JovemRESUMO
INTRODUCTION: In France, surgical oncology is not recognized as a unique specialty, but as a sub-specialization offered to surgeons in training. To date, their motives and training have not been studied. MATERIALS AND METHODS: We set a dedicated online survey suggested to 102 surgeons applying for the specific national degree in surgical oncology. RESULTS: The answer rate was 60%. Responders were constituted of a majority of male (61%), their median age was 31 years. They were mainly residents (33%) and fellows working in university (25%) or non-university (28%) hospitals. Most responders have chosen their organ specialization at the beginning, and their oncologic sub-specialization at the middle of their residency, after a meeting with a senior surgeon. Regarding practical education, 85% used surgical videos, 62% mechanical training devices, 60% animal surgery, and 38% cadaver dissection. Regarding career expectations, 67% would like to work in a cancer centre, 51% in a university hospital, and 26% in a private institution. To explain these choices, 51% referred to research and 65% to teaching interests. CONCLUSION: This study outlines the role of mentorship and the lack of practical teaching outside the operating room during the training in surgical oncology in France.
Assuntos
Escolha da Profissão , Cirurgia Geral/educação , Oncologia/educação , Adulto , Recursos Audiovisuais/estatística & dados numéricos , Cadáver , Dissecação/educação , Feminino , França , Cirurgia Geral/estatística & dados numéricos , Humanos , Internato e Residência/estatística & dados numéricos , Masculino , Oncologia/estatística & dados numéricos , Modelos Animais , MotivaçãoRESUMO
PURPOSE: The molecular pathogenesis of pediatric ependymoma remains unclear. Our study was designed to identify genetic changes implicated in ependymoma progression. PATIENTS AND METHODS: We characterized 59 ependymoma samples (33 at diagnosis and 26 at relapse) using array-comparative genomic hybridization (aCGH). Specific chromosomal imbalances were confirmed by fluorescent in situ hybridization, and candidate genes were assessed by real-time quantitative polymerase chain reaction (qPCR), immunohistochemistry, sequencing, and in vitro functional studies. RESULTS: aCGH analysis revealed a significant increase in genomic imbalances on relapse compared with diagnosis, such as gain of 9qter and 1q (54% v 21% and 12% v 0%, respectively) and loss of 6q (27% v 6%). Supervised tumor classification showed that gain of 9qter was associated with tumor recurrence, age older than 3 years, and posterior fossa location. Using a candidate-gene strategy, we found an overexpression of two potential oncogenes at the locus 9qter: Tenascin-C and Notch1. Moreover, Notch pathway analysis (qPCR) revealed overexpression of Notch ligands, receptors, and target genes (Hes-1, Hey2, and c-Myc), and downregulation of Notch repressor Fbxw7. We confirmed by immunohistochemistry the overexpression of Tenascin-C and Hes-1. We detected Notch1 missense mutations in 8.3% of the tumors (only in the posterior fossa location and in case of 9q33-34 gain). Furthermore, inhibition of Notch pathway with a gamma-secretase inhibitor impaired the growth of ependymoma stem cell cultures. CONCLUSION: The activation of the Notch pathway and Tenascin-C seem to be important events in ependymoma progression and may represent future targets for therapy. We report, to our knowledge for the first time, recurrent oncogenic mutations in pediatric posterior fossa ependymomas.