Diagnosis and treatment of benign adipocytic tumors in children.

PURPOSE: To describe demographic, clinical, diagnostic and therapeutic aspects of pediatric patients with benign adipocytic tumors admitted to a high complexity teaching hospital from 2007 to 2021. METHODS: Retrospective observational descriptive study. Patient information was retrieved from clinical records. A descriptive analysis was carried out for qualitative data and frequencies were calculated for quantitative data. RESULTS: 76 patients were included with a mean age of 7.5 years old where 60.5% were boys. The main symptom was a mass (73.7%) mostly found in the lower limbs (23.6%). Congenital birth defects were identified in 48.6% of the cases. Preoperative imaging was available in 78.9% of the patients allowing characterization of lesions or differential diagnosis. The therapeutic goal was resection with negative margins, which was feasible in all cases except for one case. The histopathological diagnosis was lipoma in 68.4% of the cases followed by lipoblastoma in 13.1%. The mean follow-up period was 17.9 months. 79.7% of the patients were asymptomatic at their last out-patient visit. CONCLUSION: Benign adipocytic tumors constitute a wide spectrum of lesions, which involve diverse anatomic segments from the neural axis to the inguinoscrotal region. The present work contributes to the general understanding of the clinical presentation and differential diagnosis for these infrequent neoplasms.

Melanotic neuroectodermal tumor of infancy: a case report / Tumor neuroectodérmico melanocítico de la infancia: reporte de caso

Abstract Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a rare neoplasm originating from neural crest cells, which generally affects pediatric patients, most frequently during the first year of life. The behavior of MNTIs is benign, locally aggressive, with a recurrence of 10-15% and eventually malignant in 6.97%. This study describes the clinical, imaging, histopathological, immunohistochemical characteristics and the management of MNTI in a 5-month-old girl, whose lesion was resected and monitored. The present case illustrates the benefits of multidisciplinary integration for a correct diagnosis to ensure adequate therapeutic management, in addition to providing a report on this rare and understudied pathology.

Resumen El Tumor Neuroectodérmico Melanocítico de la Infancia (TNEMI) es una neoplasia infrecuente derivada de las células de la cresta neural, que afecta generalmente pacientes pediátricos y se presenta con mayor frecuencia durante el primer año de vida. Su comportamiento es benigno, localmente agresivo, con una recurrencia de 10% a 15% y eventualmente maligno en un 6.97%. En este estudio se describen las características clínicas, imagenológicas, histopatológicas, inmunohistoquímicas y el manejo del TNEMI en una niña de 5 meses de edad, a la cual se le realizó resección de la lesión y seguimiento. El presente caso ilustra el beneficio de la integralidad multidisciplinaria que permite establecer un diagnóstico correcto para asegurar un manejo terapéutico adecuado, además de aportar un reporte sobre esta patología poco frecuente y estudiada.