RESUMO
INTRODUCTION: Signet ring cell carcinoma of the ampulla of Vater is a rare entity and less than 20 cases have been described in the literature. We report the cases of two patients with this disease and provide a literature review of previous studies. CASE REPORT: We describe two patients with obstructive jaundice. Abdominal ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic and common bile duct. Duodenoscopy indicated a protruding mass on the ampulla of Vater. Histopathological examination showed round cells and their nuclei were located on one side with prominent signet-ring features. One patient underwent a cephalic pancreatoduodenectomy with lymphadenectomy and the other a total pancreatectomy. DISCUSSION: Signet ring cell carcinoma of the ampulla of Vater has only been described in isolated cases in the literature. Therefore, the clinicopathological features and prognosis of this disease have not yet been well defined.
Assuntos
Ampola Hepatopancreática/patologia , Carcinoma de Células em Anel de Sinete/patologia , Neoplasias do Ducto Colédoco/patologia , Idoso , Ampola Hepatopancreática/cirurgia , Antimetabólitos Antineoplásicos/uso terapêutico , Carcinoma de Células em Anel de Sinete/complicações , Carcinoma de Células em Anel de Sinete/diagnóstico , Carcinoma de Células em Anel de Sinete/tratamento farmacológico , Carcinoma de Células em Anel de Sinete/cirurgia , Quimioterapia Adjuvante , Colangiopancreatografia Retrógrada Endoscópica , Terapia Combinada , Neoplasias do Ducto Colédoco/complicações , Neoplasias do Ducto Colédoco/diagnóstico , Neoplasias do Ducto Colédoco/tratamento farmacológico , Neoplasias do Ducto Colédoco/cirurgia , Desoxicitidina/análogos & derivados , Desoxicitidina/uso terapêutico , Humanos , Icterícia Obstrutiva/etiologia , Jejuno/cirurgia , Fígado/cirurgia , Linfoma Folicular , Masculino , Invasividade Neoplásica , Segunda Neoplasia Primária , Pancreatectomia , Pancreaticoduodenectomia , Esplenectomia , Neoplasias da Bexiga Urinária , GencitabinaRESUMO
INTRODUCTION: To report a case of idiopathic orbital sclerosing inflammation (ISOI) with intranasal extension. MATERIAL AND METHODS: The patient presented with a 6-month history of epiphora, upper eyelid swelling, ptosis and mild orbital pain. Ophthalmologic examination, CT, MRI and biopsy with surgical debulking were performed. RESULTS: MRI revealed a homogeneously enhancing diffuse right orbital mass in the inferonasal quadrant of the orbit, which extended to the nasal cavity up to inferior nasal concha, maxillary and ethmoid sinuses. Histological analysis showed dense collagenous tissue with sparse infiltration of mixed inflammatory cells. Inmunohistochemical analysis confirmed polyclonality. The diagnosis of idiopathic sclerosing orbital inflammation was made and 80 mg/day of oral prednisolone was prescribed. At last follow up, one year later, there was no clinical evidence of recurrent orbital disease. CONCLUSION: ISOI can present with extraorbital extension. Corticosteroids are a reasonable first-line treatment, until the pathogenesis is better understood.
