RESUMO
OBJECTIVE: Targeted therapy (TT) with BRAF/MEK inhibitors has emerged as a potential treatment in papillary craniopharyngiomas (PCPs). However, standardized data on large cohorts are lacking. Our study aimed to assess real-life efficacy and safety of BRAF/MEK inhibition in patients with PCPs. DESIGN: Retrospective French multicenter study involving BRAF V600E-mutated PCP patients, treated with BRAF/MEK inhibitor combination dabrafenib and trametinib, from April 2019 to July 2023. METHODS: Objective response and clinical and safety outcomes were assessed after 3 months and at the last available follow-up during TT. RESULTS: Sixteen patients (8 females, mean age 50.5 ± 15.75 years), receiving either neoadjuvant therapy (NEO) for non-resectable tumors (n = 6), post-surgical adjuvant therapy (ADJ; n = 8), or palliative therapy (PAL) following failure of multimodal treatment (n = 2), were included.At the last follow-up (mean 7.6 ± 5.3 months), 12 patients showed subtotal response, 3 exhibited partial response, and 1 maintained stable disease. Mean volume reduction was 88.9 ± 4.4%, 73.3 ± 23.4%, and 91.8 ± 4.3% in the NEO, ADJ, and PAL groups, respectively.Targeted therapy resolved headaches in 5/5 patients and visual impairment in 6/9; 2/3 patients had improved neurological symptoms, 1/4 presented weight loss, and 2/14 recovered endocrine function.Targeted therapy was well-tolerated in 62.5% of cases; adverse events led to treatment discontinuation in 5 patients and definitive discontinuation in 3 cases. CONCLUSIONS: In this study, 94% of patients showed partial response or better to TT. Adverse events were acceptable. Further research is needed to establish standardized protocols; however, these results advocate for a NEO approach in invasive PCPs.
Assuntos
Craniofaringioma , Oximas , Neoplasias Hipofisárias , Proteínas Proto-Oncogênicas B-raf , Piridonas , Pirimidinonas , Humanos , Feminino , Masculino , Pessoa de Meia-Idade , Proteínas Proto-Oncogênicas B-raf/antagonistas & inibidores , Proteínas Proto-Oncogênicas B-raf/genética , Adulto , Estudos Retrospectivos , Craniofaringioma/tratamento farmacológico , Piridonas/uso terapêutico , Piridonas/administração & dosagem , Piridonas/efeitos adversos , Idoso , Neoplasias Hipofisárias/tratamento farmacológico , Pirimidinonas/uso terapêutico , Pirimidinonas/administração & dosagem , Pirimidinonas/efeitos adversos , Oximas/uso terapêutico , Oximas/administração & dosagem , Oximas/efeitos adversos , Estudos de Coortes , Inibidores de Proteínas Quinases/uso terapêutico , Inibidores de Proteínas Quinases/administração & dosagem , Inibidores de Proteínas Quinases/efeitos adversos , Terapia de Alvo Molecular/métodos , Imidazóis/uso terapêutico , Imidazóis/administração & dosagem , Imidazóis/efeitos adversos , Resultado do Tratamento , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêuticoRESUMO
(1) Background: The influence of food culture on eating behavior and obesity risk is poorly understood. (2) Methods: In this qualitative study, 25 adults in France with or without overweight/obesity participated in semi-structured interviews (n = 10) or focus groups (n = 15) to examine attitudes to food consumption and external pressures that influence eating behavior and weight management. Results were compared to an equivalent study conducted in the United States, thereby contrasting two countries with markedly different rates of obesity. Emerging key themes in the French data were identified through coding using a reflexive approach. (3) Results: The main themes identified were: (1) influence of commensality, social interactions, and pleasure from eating on eating behavior, (2) having a balanced and holistic approach to nutrition, (3) the role of environmental concerns in food consumption, (4) relationship with "natural" products (idealized) and food processing (demonized), (5) perceptions of weight status and management. Stress and difficulties in hunger cue discernment were viewed as important obstacles to weight management in both countries. External pressures were described as a major factor that explicitly influences food consumption in the U.S., while there was an implicit influence of external pressures through eating-related social interactions in France. In France, products considered "natural" where idealized and juxtaposed against processed and "industrial" products, whereas this was not a salient aspect in the U.S. (4) Conclusions: This first comparative qualitative study assessing aspects of food culture and eating behaviors across countries identifies both common and divergent attitudes to food and eating behavior. Further studies are needed to inform the development of effective behavioral interventions to address obesity in different populations.
Assuntos
Atitude , Ingestão de Energia , Comportamento Alimentar , Alimentos , Obesidade , Adulto , Peso Corporal , Cultura , Ingestão de Alimentos , Feminino , Grupos Focais , Preferências Alimentares , França , Humanos , Fome , Masculino , Pessoa de Meia-Idade , Estado Nutricional , Sobrepeso , Prazer , Pesquisa Qualitativa , Estados UnidosRESUMO
Gangliocytomas are rare and benign neuronal cell tumors, mostly found in the hypothalamic and sellar regions. Their histogenesis is still the subject of discussions. Herein we present a unique case of a pituitary gangliocytoma associated with a prolactinoma and a corticotroph adenoma in a patient affected by MEN1. The histologic study revealed shared features between adenomatous and neuronal cells, supporting the etiological hypothesis of a common origin or a phenomenon of transdifferentiation. Furthermore, gangliocytoma could be a new tumor related to MEN1. The clinical and histologic observations are discussed and the literature on the topic is reviewed.