RESUMO
Duplication cysts of the pylorus are the rarest of alimentary tract duplications with very few reported cases. We present such a cyst in a neonate presenting with gastric outlet obstruction. We have also reviewed the literature and outlined the theories of origin, modes of presentation, diagnosis and the surgical procedures. Even though several modes of surgery have been described, it is best to individualize the surgical option in each case. The results depend on the nature and site of duplication, complications and the associated anomalies.
Assuntos
Cistos/congênito , Diagnóstico Diferencial , Obstrução da Saída Gástrica/congênito , Humanos , Recém-Nascido , Masculino , Piloro/anormalidadesRESUMO
Extra renal Wilm's tumour (EWT) is an exceedingly rare entity and needs to fulfill certain criteria. Of the 48 cases reported, only about 36 have been found to be convincingly proved. We present one more well proven case of EWT and have extensively reviewed the literature. The diagnostic, staging and management guidelines are discussed. Amongst the reported cases, the behaviour of the tumour appears to parallel that of similarly staged "intra renal" Wilm's tumour. Hence the staging and the management protocols of "intra renal" Wilm's tumour can well be applied to tumour of extra renal location albeit with some modifications.
Assuntos
Neoplasias Abdominais/diagnóstico , Pré-Escolar , Humanos , Masculino , Tumor de Wilms/diagnósticoAssuntos
Bleomicina/administração & dosagem , Criança , Cisplatino/administração & dosagem , Terapia Combinada , Dexametasona/administração & dosagem , Tratamento Farmacológico , Tumor do Seio Endodérmico/tratamento farmacológico , Feminino , Germinoma/tratamento farmacológico , Humanos , Imageamento por Ressonância Magnética , Metástase Neoplásica , Medula Espinal/patologia , Neoplasias da Medula Espinal/patologia , Vimblastina/administração & dosagemRESUMO
Twelve cases of endodermal sinus tumor were reviewed. There were 10 females and 2 males with a median age at presentation of 3 years. The primary site was sacrococcygeal in 4 patients, vaginal in 3, retroperitoneal in 2, and testicular, ovarian and left chest wall in one each. The diagnosis rested on histopathological examination and elevation of serum alfa feto protein levels (median 46,200 ng/ml). Two patients had Stage I disease, 9 had Stage III and one had Stage IV disease. Patients were managed by surgery and chemotherapy (BVP regime). All patients on BVP (even those lost at later stages), had achieved clinical remission with the first cycle of treatment.