RESUMO
Primary Biliary Cholangitis (PBC) is an autoimmune liver disease that is sometimes associated with CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. If left untreated, PBC eventually progresses to liver cirrhosis. We describe an adult patient with CREST-PBC who presented with recurrent variceal bleeding and ultimately required transjugular intrahepatic portosystemic shunt (TIPS) insertion. Liver biopsy excluded cirrhosis, resulting in a diagnosis of noncirrhotic portal hypertension. This case report describes the pathophysiology of presinusoidal portal hypertension as a rare complication of PBC and its association with coexisiting CREST.
Assuntos
Síndrome CREST , Varizes Esofágicas e Gástricas , Hipertensão Portal , Cirrose Hepática Biliar , Derivação Portossistêmica Transjugular Intra-Hepática , Adulto , Humanos , Síndrome CREST/complicações , Cirrose Hepática Biliar/complicações , Varizes Esofágicas e Gástricas/complicações , Hemorragia Gastrointestinal/etiologia , Hipertensão Portal/complicações , Cirrose Hepática/complicações , Resultado do TratamentoAssuntos
Doenças Biliares/induzido quimicamente , Doenças Biliares/diagnóstico , Colangite Esclerosante/diagnóstico , Ketamina/efeitos adversos , Transtornos Relacionados ao Uso de Substâncias/complicações , Administração por Inalação , Anestésicos Dissociativos/administração & dosagem , Anestésicos Dissociativos/efeitos adversos , Doenças Biliares/complicações , Biópsia , Colangite Esclerosante/etiologia , Humanos , Ketamina/administração & dosagem , Fígado/patologia , Masculino , Centros de Tratamento de Abuso de Substâncias , Transtornos Relacionados ao Uso de Substâncias/terapia , Resultado do Tratamento , Adulto JovemRESUMO
Myoepitheliomas have been described most commonly in salivary glands and have been reported elsewhere but are rare in the lung, with only six previously reported cases. To our knowledge, this represents the first endotracheal myoepithelioma. These tumors have characteristic features that distinguish them from other tumors, and the diagnosis is a pathologic one, based on the morphology and supported by immunohistochemistry. Myoepitheliomas should be considered in the diagnosis of any pulmonary nodule.
Assuntos
Actinas/análise , Brônquios/patologia , Neoplasias Pulmonares/diagnóstico , Mioepitelioma/diagnóstico , Idoso , Biópsia , Broncoscopia , Diagnóstico Diferencial , Humanos , Imuno-Histoquímica , Neoplasias Pulmonares/metabolismo , Masculino , Mioepitelioma/metabolismo , Tomografia Computadorizada por Raios XRESUMO
Mucinous cystic neoplasms (MCNs) make up a morphologic family of similar appearing tumors arising in the ovary and various extraovarian sites, including the pancreas, hepatobiliary tract, paratesticular soft tissues, and mesentery. Other than the uncommon mucinous cystadenoma of the ovary presenting in adolescence, MCNs are rarely seen by the pediatric pathologist. The present case is a 5-year-old boy with an abdominal mass appearing to arise in the mesentery of the small intestine. Because of its unresectability, a generous biopsy was performed and disclosed a MCN with focal complex papillary architecture in the absence of appreciable cytologic atypia or invasion into the wall. Like other MCNs, this tumor had an inhibin-positive, ovarian-like stroma that was nonreactive for estrogen and progesterone receptors. Only 1 other case of a mesenteric MCN has been reported to date in a child and none in a male. The MCN of the mesentery joins other, somewhat more common cystic lesions of the omentum and mesentery presenting in childhood.