Innovative solutions? Belzutifan therapy for hemangioblastomas in Von Hippel-Lindau disease: A systematic review and single-arm meta-analysis.

BACKGROUND: Von Hippel-Lindau (VHL) disease is a rare autosomal dominant disorder that predisposes patients to develop multiple cysts and tumors, such as hemangioblastomas (HBs) and clear cell renal cell carcinoma (ccRCC), due to mutations in the VHL tumor suppressor gene. While treatment of HBs varies based on their characteristics and has improved patient survival, it still involves high morbidity and mortality, leading to ongoing debates and studies to refine therapy strategies. Recent developments include the emergence of Belzutifan, a novel inhibitor targeting hypoxia-inducible factor 2α (HIF-2α), which has shown promising results in ongoing trials, particularly for patients not immediately requiring surgery. METHODS: This systematic review and meta-analysis aimed to comprehensively evaluate the efficacy and safety of Belzutifan for treating HBs associated with VHL disease. Search was conducted across Medline, Embase, Cochrane, and Web of Science databases. Statistical Analysis was performed, with proportions and 95 % confidence intervals. Statistical analyses were carried out using R Studio. RESULTS: Ten studies were selected, comprising 553 patients. The population mean age was 40 (24-65), and 50 % of the population was formed by males. In terms of proportion, 6 analyses were performed: Disease Stability of 31 % [95 %CI:14 %-47 %; I2 = 2 %]; Disease Progression of 2 %[95 %CI:0 %-9 %; I2 = 0 %]; Partial Response of 75 % [95 %CI:54 %-96 %; I2 = 58 %]. Complete response of 1 % [95 %CI:0 %-7 %; I2 = 0 %];and Side effects, anemia 81 % rate [95 % CI:54 %-100 %; I2 = 94 %], and fatigue rate of 79 % [95 % CI:54 %-100 %;I2 = 94 %]. CONCLUSION: Results indicate that Belzutifan effectively stabilizes disease, reduces tumor progression, and achieves significant therapeutic responses, although side effects like anemia and fatigue were noted.

Predicting the evolution of pH and total soluble solids during coffee fermentation using near-infrared spectroscopy coupled with chemometrics.

Currently, coffee fermentation is visually operated, which results in incomplete or excessive processes and coffees with undesirable characteristics. In front of it, pH and total soluble solids (TSS) have been shown to be good fermentation indicators, although this requires rapid, accurate, and chemical-free measurement techniques such as NIR spectroscopy. However, the complexity of the NIR spectra requires optimization steps in which variable selection techniques simplify profiles and subsequent models. This work tests a new covering array feature selection (CAFS) approach on NIR spectra to optimize prediction models in coffee samples during fermentation. Spectral profiles in the range 1100-2100 nm were extracted from coffee beans (Typica, Caturra, and Catimor varieties) raw and during fermentation (4, 8, 12, 16, 20, and 24 h). Partial least-squares regressions (PLSR) were performed using full spectra using a five-fold cross-validation strategy for training and validation. The relevant wavelengths were then selected using the ß coefficients, the important projection of variables (VIP), and the CAFS method. Finally, optimized models were performed using the relevant wavelengths and compared among these using their statistical metrics. The models performed using the selected variables (22-47) of CAFS showed the best performance in predicting pH (R 2 = 0.825-0.903, RMSE = 0.096-0.158, RPD = 6.33-10.38) and TSS (R 2 = 0.865-0.922, RMSE = 0.688-1.059, RPD = 0.94-1.45) compared to the other methods. These findings suggest that simple and efficient models could be performed and implemented in routine analysis due to the maximum coverage and minimum cardinality of CAFS.

Prevalencia de patología maligna de seno en mujeres mayores de 14 años que consultaron por masa sólida palpable / Prevalence of breast malignancy in women older than forteen years who consulted for solid palpable mass

Objetivo Establecer la prevalencia de la patología maligna en pacientes con masa sólida palpable, sin diagnóstico previo de cáncer de seno en dos hospitales de Bogotá, Colombia. Materiales y métodos Estudio descriptivo retrospectivo entre marzo de 2010 y febrero de 2013 en los hospitales de San José e Infantil Universitario de San José, Bogotá D. C., Colombia. Se incluyeron mujeres mayores de 14 años que consultaron por masa sólida palpable sin diagnóstico previo de cáncer de seno, corroborada por examen físico; no se consideró ningún criterio de exclusión. Los datos se recolectaron de las historias clínicas y se llevaron a un formato creado por los investigadores. El programa estadístico utilizado fue Stata 13. Resultados Se confirmó la masa en 342 pacientes por examen clínico, en 307 pacientes con resultado de biopsia. La prevalencia de la patología maligna fue 12,2% y benigna 71,66%. Discusión La prevalencia de patología maligna por masa palpable fue menor que los datos reportados a nivel mundial, siendo el tumor más frecuente el carcinoma ductal infiltrante en un 87%, carcinoma lobulillar infiltrante en 6,4%), con estadio clínico IIA y BI-RADS 4 A (ecografía) y BI-RADS 4B (mamografía).

Objective To determine the prevalence rate of malignancy in patients with no prior breast cancer diagnosis who consulted for a solid palpable mass in two hospitals in Bogotá, Colombia. Materials and methods A descriptive retrospective study conducted between March 2010 and February 2013 at San José and Infantil Universitario de San José hospitals in Bogotá D. C., Colombia. Women 14 years or older with no prior breast cancer diagnosis who consulted for a palpable solid mass, confirmed by physical exam, were included. No exclusion criteria were considered. Data was collected from the clinical records and included in a format created by the researchers. Stata 13 was used for data analysis. Results The mass was confirmed by physical exam in 342 patients and by a biopsy in 307 patients. The prevalence rate for malignancy was 12.2% and for benign masses 71.66%. Discussion Our prevalence of breast cancer associated with a palpable mass was less than worldwide reported prevalence. The most frequent malignancy was invasive ductal carcinoma in 87% and invasive lobular carcinoma in 6.4% in stage IIA and BI-RADS 4A ultrasound category and BI-RADS 4B mammogram category.

Glosectomía parcial como tratamiento de macroglosia asociada a neurofibromatosis de Von Recklinghausen / Partial glossectomy as a treatment for macroglossia associated with Von Recklinghausen neurofibromatosis

Dentro de los signos de la neurofibromatosis de Von Recklinghausen se encuentra la macroglosia, la cual va a conllevar problemas de tipo foniátrico y de deglución, entre otros, para el paciente. El tratamiento de elección es la glosectomía parcial. En el presente artículo se presenta un caso referente a esto