RESUMEN
BACKGROUND: The value of gross total resection (GTR) for children with high-risk neuroblastoma (NB) is controversial. We hypothesized that patients undergoing GTR would demonstrate improved overall survival (OS) compared those having
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía , Neuroblastoma/cirugía , Neoplasias de las Glándulas Suprarrenales/tratamiento farmacológico , Neoplasias de las Glándulas Suprarrenales/epidemiología , Neoplasias de las Glándulas Suprarrenales/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Preescolar , Terapia Combinada , Femenino , Trasplante de Células Madre Hematopoyéticas , Humanos , Lactante , Estimación de Kaplan-Meier , Masculino , Neoplasias del Mediastino/tratamiento farmacológico , Neoplasias del Mediastino/epidemiología , Neoplasias del Mediastino/cirugía , Neoplasias del Mediastino/terapia , Terapia Neoadyuvante , Neuroblastoma/tratamiento farmacológico , Neuroblastoma/epidemiología , Neuroblastoma/secundario , Neuroblastoma/terapia , Trasplante de Células Madre de Sangre Periférica , Modelos de Riesgos Proporcionales , Radioterapia Adyuvante , Neoplasias Retroperitoneales/tratamiento farmacológico , Neoplasias Retroperitoneales/epidemiología , Neoplasias Retroperitoneales/cirugía , Neoplasias Retroperitoneales/terapia , Estudios Retrospectivos , Riesgo , Sesgo de Selección , Acondicionamiento Pretrasplante , Trasplante Autólogo , Resultado del TratamientoRESUMEN
Single cases of B- and T-lymphoblastic leukemia/lymphoma occurring after remission of JMML, and JMML occurring after remission of B-lymphoblastic leukemia have been reported in the literature. We present a unique case of a child with concurrent JMML and T-lymphoblastic lymphoma in which an identical missense mutation in NRAS was found in both the neoplastic JMML and T-LBL cells. JMML has been considered a stem cell disorder, and our case provides additional molecular evidence for a stem cell lesion underlying the two different disease phenotypes.