RESUMO
Juvenile xanthogranuloma is a benign and self-limited disease which usually appears in the skin of children. Visceral involvement has been rarely reported, as has fatal outcome in some affected individuals. We report a case of systemic juvenile xanthogranuloma in a female newborn with mainly skin, bone marrow, and liver involvement, leading to death at the age of 2 months.
Assuntos
Falência Hepática/patologia , Pancitopenia/patologia , Pele/patologia , Xantogranuloma Juvenil/patologia , Biópsia , Evolução Fatal , Feminino , Histiócitos/patologia , Humanos , Recém-Nascido , Fígado/patologiaRESUMO
Myelodysplastic syndromes (MDS) in childhood are rare hematologic diseases. MDS with t(3;5) (NPM/MLF1) is an unusual subtype without a well-defined clinical and prognostic pattern. A poor outcome has been reported, suggesting that hematopoietic transplantation is the only treatment option. Here in we described a 2-year-old child diagnosed with the disease, without a suitable hematopoietic donor, treated early in the disease with chemotherapy. He is alive and well 4 years after the end of treatment. This unusual MDS needs further studies to better understand the disease.
Assuntos
Cromossomos Humanos Par 3 , Cromossomos Humanos Par 5 , Leucemia Mieloide Aguda/genética , Proteínas de Fusão Oncogênica/genética , Translocação Genética , Antígenos CD/sangue , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Pré-Escolar , Ciclofosfamida/uso terapêutico , Citarabina/uso terapêutico , Doxorrubicina/uso terapêutico , Humanos , Leucemia Mieloide Aguda/tratamento farmacológico , Leucemia Mieloide Aguda/imunologia , Masculino , Metotrexato/uso terapêutico , Prednisona/uso terapêutico , Tioguanina/uso terapêutico , Resultado do Tratamento , Vincristina/uso terapêuticoRESUMO
El rabdomiosarcoma es el tumor maligno de partes blandas más frecuente en la edad pediátrica; sin embargo, la localización vulvar y la aparición congénita son excepcionales. Presentamos el caso de una niña recién nacida con un rabdomiosarcoma botrioides de vulva tratada con quimioterapia, cirugía conservadora y trasplante autólogo. El rabdomiosarcoma botrioides es una variante del tipo embrionario que crece típicamente en órganos huecos con revestimiento mucoso desde donde puede extenderse hacia la superficie corporal. El tratamiento del rabdomiosarcoma botriodes del área genitourinaria se basa en la poliquimioterapia y, si es necesario, se puede complementar con radioterapia y cirugía conservadora, consiguiéndose de este modo un pronóstico excelente y escasas secuelas funcionales a largo plaz
Rhabdomyosarcoma is the most frequent malignant soft tissue tumor in pediatric patients; however, the vulvar location and congenital appearance are exceptional. We present the case of a newborn girl with botryoid rhabdomyosarcoma of the vulva, treated with chemotherapy, conservative surgery and autologous transplant. Botryoid rhabdomyosarcoma is a variation of embryonal rhabdomyosarcoma that typically grows in mucosa-lined hollow organs, from where it can spread to the body surface. The treatment of botryoid rhabdomyosarcoma in the genito-urinary area is based on polychemotherapy, and it can be complemented with radiotherapy and conservative surgery if necessary, thus resulting in an excellent prognosis and few long-term functional sequelae
Assuntos
Recém-Nascido , Feminino , Humanos , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/tratamento farmacológico , Quimioterapia Combinada , Imuno-Histoquímica/métodos , Imuno-Histoquímica , Rabdomiossarcoma , Rabdomiossarcoma/radioterapia , Vulva/anormalidades , Vulva/patologia , Tomografia Computadorizada de Emissão , Imageamento por Ressonância Magnética , Neoplasias Vulvares/diagnóstico , Neoplasias Vulvares/tratamento farmacológicoRESUMO
Rhabdomyosarcoma is the most frequent malignant soft tissue tumor in pediatric patients; however, the vulvar location and congenital appearance are exceptional. We present the case of a newborn girl with botryoid rhabdomyosarcoma of the vulva, treated with chemotherapy, conservative surgery and autologous transplant. Botryoid rhabdomyosarcoma is a variation of embryonal rhabdomyosarcoma that typically grows in mucosa-lined hollow organs, from where it can spread to the body surface. The treatment of botryoid rhabdomyosarcoma in the genito-urinary area is based on polychemotherapy, and it can be complemented with radiotherapy and conservative surgery if necessary, thus resulting in an excellent prognosis and few long-term functional sequelae.
