ABSTRACT
ABSTRACT: Type 1 autoimmune pancreatitis is a cause of chronic pancreatitis related to the systemic disease known as IgG4-related Sclerosing Disease. Case report: We report the case of a 64-year-old male patient who presented recurrentepigastric pain radiating to the back, associated with jaundice, xerostomia, nausea, and vomiting, since 2014, diagnosed two years later with an unresectable pancreatic adenocarcinoma. The diagnosis was questioned after a few follow-up months without clinical deterioration when it was suggested the possibility of type 1 autoimmune pancreatitis in its pseudotumoral form. The patient was then treated with glucocorticoids, obtaining significantclinical improvement. After two years of follow-up, he returned asymptomatic with images suggestive of sclerosingcholangitis and a large liver abscess. Importance of the issue: The present case denotes the difficulty found in this diagnosis due to clinical and radiological resemblances with pancreatic adenocarcinoma. Besides that, it presents a seldom described disease complication, the liver abscess. (AU)
RESUMO: A pancreatite autoimune tipo 1 é uma causa de pancreatite crônica relacionada à doença sistêmica conhecida como Doença Esclerosante relacionada à IgG4. Relato do caso: Relatamos o caso de um paciente do sexo masculino,64 anos, que apresentou quadros recorrentes de dor epigástrica com irradiação para as costas, associada com icterícia, xerostomia, náuseas e vômitos desde 2014, diagnosticado após 2 anos com adenocarcinoma pancreático irressecável. O diagnóstico foi questionado após alguns meses de acompanhamento sem deterioração clínica, quando aventaram a possibilidade de forma pseudotumoral da pancreatite autoimune tipo 1. Realizou tratamento com glicocorticoides, obtendo melhora clínica importante. Após dois anos de acompanhamento, retorna assintomático com imagens sugestivas de colangite esclerosante e volumoso abscesso hepático. Importância do problema: O presente caso denota uma dificuldade encontrada no diagnóstico dessa entidade devido a semelhanças clínico-radiológicas com o adenocarcinoma pancreático. Além disso, apresenta uma complicação pouco descrita da doença, o abscesso hepático. (AU)
Subject(s)
Humans , Male , Middle Aged , Pancreatitis , Autoimmune Diseases , Immunoglobulin G , Cholangitis, Sclerosing , Clinical Deterioration , Immunoglobulin G4-Related Disease , Autoimmune Pancreatitis , Liver AbscessABSTRACT
Hepatocellular adenomas (HCAs) are rare benign monoclonal hepatic tumors that commonly occur in females (3-4 per 100,000 women) due to the use of oral contraceptives, its primary risk factor. Recently, HCAs have been classified into 4 distinct subtypes according to genotypic and phenotypic characteristics and clinical features: inflammatory HCA (40-50%), which are hypervascular with marked peliosis and a tendency to bleed; hepatocyte nuclear factor 1α (HNF1A)-mutated HCA (H-HCA, 30-40%) that are diffusely steatotic and rarely undergo malignant transformation; ß-catenin activated HCA (10-15%), which frequently undergo malignant transformation and may seem hepatocellular carcinoma on imaging; and unclassified HCA (10-25%). In this study, we report the case of a 23-year-old female oral contraceptive user with H-HCA. Usually, H-HCA is considered to be nonsevere in most cases and often requires outpatient follow-up. However, in this case, the injury had substantially increased in volume and evolved with a major bleeding frame, which was an unusual finding for this subtype of adenoma. The therapeutic used for this patient was a laparoscopic left hepatic segmentectomy. Thus, the choice of treatment to be performed in a patient with H-HCA can depend on the tumor size (>5 cm), the outcome of previous bleeding, and the risk of bleeding recurrence.
ABSTRACT
OBJECTIVES: The present article provides an overview of the spectrum of abdominal findings of histiocytic disorders that may be observed in multimodality imaging illustrated by clinical cases from our Imaging Center. METHODS: We will review abdominal findings of Langerhans cell histiocytosis, Rosai-Dorfman disease, Erdheim-Chester disease, and hemophagocytic syndrome illustrated by clinical cases from our imaging department with histologic correlation. RESULTS: Abdominal involvement of histiocytic disorders is rare and may occur in the liver, biliary tract, kidney, retroperitoneum, kidney, gastrointestinal tract, and lymph nodes. CONCLUSION: Histiocytic disorders encompass a group of rare diseases with a wide range of manifestations in which the abdominal involvement is quite infrequent. The role of the radiologist is to report the major imaging findings and the differential diagnosis; however, the imaging features are unspecific and biopsy usually is necessary to establish the definitive diagnosis.
