Liver transplantation for primary liver tumors in pediatrics. A case series. / Trasplante hepático para tumores hepáticos primarios en pediatría. Serie de casos.

Primary liver tumors are an increasing indication for pediatric liver transplantation. Here we report the cases of 10 patients who underwent liver transplantation for primary liver tumors in our hospital, from 2001 to date. Up to 2011, 1 transplant due to hepatoblastoma was done out of 117 liver transplants (0.8%). Since 2012, there were 9 patients out of 141 (6.4%) (5 due to hepatoblastoma, 2 due to hepatocellular carcinoma, 1 due to hepatic epithelioid hemangioendothelioma, and 1 due to hepatic mesenchymal hamartoma). Follow-up: 13.2 months (median); age at transplantation: living 4.7 years (median); weight: 17.6 kg (median). Eighty percent of patients received grafts from living donors. No tumor recurrence was observed. Survival was 100% in the follow-up period. In our series, patients with primary liver tumors requiring transplantation showed an adequate course, even in the case of hepatocellular carcinoma, Related living donors liver transplantation shortened the time between the indication and the surgery.

Los tumores hepáticos primarios son indicación creciente de trasplante hepático pediátrico. Reportamos los 10 pacientes con trasplantes hepáticos por tumores hepáticos primarios en nuestro centro desde 2001 hasta la actualidad. Hasta el año 2011, se realizó un trasplante por hepatoblastoma de 117 trasplantes hepáticos (0,8 %). Desde 2012, fueron 9 pacientes de 141 (6,4 %) (5 hepatoblastomas, 2 hepatocarcinomas, 1 hemangioendotelioma epitelioide hepático y 1 hamartoma mesenquimático hepático). Seguimiento 13,2 meses (media), edad al trasplante 4,7 años (media), peso 17,6 kg (mediana). El 80 % recibió injertos desde donantes relacionados. No hubo recurrencia tumoral y la sobrevida fue del 100 % en el período de seguimiento. En nuestra serie, los pacientes con tumores hepáticos primarios que requirieron trasplante presentaron buena evolución, aun en hepatocarcinoma. El trasplante hepático con donante relacionado acortó los tiempos entre la indicación y la realización.

Chemotherapy-induced neuropathy and pain in pediatric oncology patients: impact of combination therapies.

Chemotherapy-induced peripheral neuropathy (CIPN) and associated pain are prevalent adverse effects of pediatric cancer treatment, significantly affecting the patient's quality of life. Their impact and risk factors have yet to be assessed in our country. This study aimed to assess the prevalence and clinical characteristics of CIPN, as well as to explore associations with patient- and treatment-related variables, within a cohort of Argentinean pediatric oncology patients. Sixty-six patients diagnosed with malignant hematopoietic tumors and receiving the neurotoxic agent vincristine were included in this observational study. Variables analyzed included age, gender, anthropometric measurements, tumor type, chemotherapy treatment, development of pain and other symptoms, severity, and analgesic treatment. The study population consisted of 39 boys and 27 girls. Most patients received two or three neurotoxic drugs. Symptoms consistent with CIPN were identified in 15 children, reflecting a prevalence of 23%. The main symptom was pain in the lower limbs, with some patients reporting jaw or generalized body pain. Pain was categorized as moderate or severe in 60% and 27% of cases, respectively. NSAIDs, anticonvulsants, and/or opioids were prescribed. Among the patient- and treatment-related variables analyzed as potential risk factors, the use of vincristine in conjunction with cytarabine and the administration of a higher number of neurotoxic drugs demonstrated significant association with the development of CIPN. CONCLUSIONS: Combination therapy stands out as a risk factor for clinical CIPN. The high prevalence of moderate/severe pain underscores the importance of close vigilance given its potential to compromise the patient's overall well-being. WHAT IS KNOWN: • Chemotherapy-induced peripheral neuropathy (CIPN) is a frequent adverse effect and dose-limiting factor in pediatric cancer treatment. • Prevalence varies among regions and risk factors are still under study. WHAT IS NEW: • Prevalence of symptomatic CIPN is 23% among pediatric patients undergoing treatment for hematopoietic tumors in a referral hospital in Argentina. Most patients report moderate or severe pain. • Combining vincristine with cytarabine and using a higher number of neurotoxic drugs in combination therapies exhibit significant association with the development of CIPN-related symptoms.

Association between Hypertriglyceridemia and Disease Severity in Visceral Leishmaniasis.

Visceral leishmaniasis (VL) is a tropical disease endemic to Brazil. The clinical manifestations of the infection range from asymptomatic to severe. In VL, changes in lipid metabolism, such as hypocholesterolemia and hypertriglyceridemia, occur that are believed to be related to its progression and severity. This study investigated the associations between serum levels of cholesterol, triglycerides, and lipoproteins (high-density lipoprotein, low-density lipoprotein, and very low-density lipoprotein) with clinical and hematological parameters that predict severity in a case series of 83 VL patients. Severely ill patients had higher mean serum triglyceride levels than non-severely ill patients. There was a significant positive correlation between disease severity score and serum triglyceride levels, very low-density lipoprotein, international normalized ratio for prothrombin time test, total bilirubin, and age. An inverse correlation was detected between the disease severity score and mean platelet and neutrophil counts. Hypertriglyceridemia can be a prognostic indicator of severity in patients diagnosed with VL.

Treatment and outcome in 12 cases of olfactory neuroblastoma at Mexico´s National Cancer Institute: A retrospective clinical analysis and literature review.

INTRODUCTION: Olfactory neuroblastoma (ONB) is a malignant neoplasm that arises from the upper nasal vault. OBJECTIVE: We present a retrospective case series and clinical analysis of 12 ONB cases. MATERIALS AND METHODS: Patients with ONB treated at Mexico´s National Cancer Institute between 2011 and 2018. RESULTS: The Kadish proportion of B, C, and D stage was 16%, 58%, or 25%, respectively. Hyams Grade 1, 2, or 3 was 25%, 50%, and 25%, respectively. The most common surgical approach was the craniofacial in 5 cases (42%), followed by the transfacial in 4 cases (33%), and the endonasal endoscopic approach in 3 cases (25%). Gross total resection was achieved in 8 patients (67%). Five patients (42%) underwent a second operation due to recurrent/progressive disease. The surgical complication rate was 8.3%. Progression-free survival was 41 months and the mean overall survival was 63.6 months. CONCLUSIONS: Surgical resection followed by radiotherapy, and chemotherapy for metastatic and recurrent disease provides the best outcome in terms of survival and recurrence. To the best of our knowledge, this is the first series of cases reported in Mexico.

ANTECEDENTES: El neuroblastoma olfatorio es una neoplasia maligna que se origina en la bóveda nasal superior. OBJETIVO: Presentar una serie de casos y un análisis clínico retrospectivo. MÉTODO: Pacientes con neuroblastoma olfatorio tratados en el Instituto Nacional de Cancerología, de México, entre 2011 y 2018. RESULTADOS: La proporción de Kadish en las etapas B, C y D fue del 16, el 58 y el 25%, respectivamente. Los grados 1, 2 y 3 de Hyams fueron el 25, el 50 y el 25%, respectivamente. El abordaje quirúrgico más frecuente fue el craneofacial, en cinco casos (42%), seguido del transfacial en cuatro (33%) y del abordaje endoscópico endonasal en tres (25%). La resección total macroscópica se logró en ocho pacientes (67%). Cinco pacientes (42%) se sometieron a una segunda operación debido a enfermedad recurrente o progresiva. La tasa de complicaciones quirúrgicas fue del 8,3%. La sobrevida libre de progresión fue de 41 meses y la supervivencia media global fue de 63,6 meses. CONCLUSIONES: La resección quirúrgica seguida de radioterapia y quimioterapia para la enfermedad metastásica y recurrente proporciona el mejor resultado en términos de supervivencia y recurrencia. Hasta donde sabemos, esta es la primera serie de casos reportados en México.

Molecular characterization of Capim and Enseada orthobunyaviruses.

Capim and Enseada viruses are members of the genus Orthobunyavirus isolated from mosquitoes and mammals in Brazil. Despite seroprevalence studies indicating human infections in Latin America, these viruses remain relatively unknown and unstudied. In order to better understand the genetic and evolutionary relationships among orthobunyaviruses, we sequenced the three genomic segments of Capim and Enseada orthobunyaviruses. Based on phylogenetic analysis, we demonstrated that these viruses depicted two new distinct clades, one represented by Enseada and another composed of Capim virus. In general, the genome organization and genetic traits of these viruses are similar to other orthobunyaviruses however, the open reading frame (ORF) of the putative nonstructural NSs protein of Enseada orthobunyavirus precedes the nucleocapsid ORF. Overall, our study provides details on the molecular characteristics of the prototype species of two groups within the Orthobunyavirus genus, revealing novel features into the genetic diversity and evolution of this genus.

Interrupción de la vena cava inferior / Inferior vena cava interruption: US, CT and MR features

La interrupción de la vena cava inferior (VCI), con continuación con el sistema ácigos-hemiácigos, es una entidad poco común, con una incidencia de presentación del 0,6 por ciento. La más frecuente es la ausencia del segmento suprarrenal, que constituye el 90 por ciento de estas anomalías y sólo un 6 por ciento compromete el segmento renal e infrarrenal. Esta entidad puede asociarse a poliesplenia o asplenia y a situs inversus, aunque también puede presentarse aisladamente. Presentamos un paciente de sexo masculino, de 51 años, que presenta interrupción del segmento intrahepático de la VCI, con continuación de las venas ácigos y hemiácigos asociada a poliesplenia

Interrupción de la vena cava inferior / Inferior vena cava interruption: US, CT and MR features

La interrupción de la vena cava inferior (VCI), con continuación con el sistema ácigos-hemiácigos, es una entidad poco común, con una incidencia de presentación del 0,6 por ciento. La más frecuente es la ausencia del segmento suprarrenal, que constituye el 90 por ciento de estas anomalías y sólo un 6 por ciento compromete el segmento renal e infrarrenal. Esta entidad puede asociarse a poliesplenia o asplenia y a situs inversus, aunque también puede presentarse aisladamente. Presentamos un paciente de sexo masculino, de 51 años, que presenta interrupción del segmento intrahepático de la VCI, con continuación de las venas ácigos y hemiácigos asociada a poliesplenia (AU)

Fibromatosis extraabdominal agresiva múltiple: utilidad de la RM / Agressive extraabdominal fibromatosis: MR features

La fibromatosis agresiva es un tumor de tejidos blandos que asienta en las estructuras musculoaponeuróticas. A pesar de su apariencia histológica benigna, tiene un comportamiento invasivo. Para demostrar la utilidad de los distintos métodos de imágenes, especialmente la RM en su evaluación, diagnóstico y tratamiento de la fibromatosis agresiva, estudiamos una mujer de 26 años mediante ecografía de miembros inferiores, TC, Rx simple, RM, arteriografía, linfografía centellográfica y biopsia quirúrgica. A pesar de que siempre se requiere la biopsia quirúrgica para arribar al diagnóstico definitivo, la RM demostró ser el método que mejor acota los posibles diagnósticos diferenciales

Fibromatosis extraabdominal agresiva múltiple: utilidad de la RM / Agressive extraabdominal fibromatosis: MR features

La fibromatosis agresiva es un tumor de tejidos blandos que asienta en las estructuras musculoaponeuróticas. A pesar de su apariencia histológica benigna, tiene un comportamiento invasivo. Para demostrar la utilidad de los distintos métodos de imágenes, especialmente la RM en su evaluación, diagnóstico y tratamiento de la fibromatosis agresiva, estudiamos una mujer de 26 años mediante ecografía de miembros inferiores, TC, Rx simple, RM, arteriografía, linfografía centellográfica y biopsia quirúrgica. A pesar de que siempre se requiere la biopsia quirúrgica para arribar al diagnóstico definitivo, la RM demostró ser el método que mejor acota los posibles diagnósticos diferenciales (AU)