RESUMEN
Rare autosomal dominant disorders associated with various tumors and cysts, and several kinds of pancreatic lesions have been described in patients with von Hippel- Lindau (VHL) disease, but there have been few studies concerned with the variations of the pancreatic duct. We experienced a case of pancreatic dual Wirsung ducts in a patient with VHL disease. Computed tomography scans showed that multiple cysts had nearly completely replaced the pancreas, there was a solid tumor on the uncinate process of the pancreas and a left renal mass suggested renal cell carcinoma. ERCP shows that the Santorini duct and Wirsung duct were fused in a normal fashion, and another Wirsung duct had shrub-like branches that are commonly seen in pancreatic divisum.
Asunto(s)
Humanos , Carcinoma de Células Renales , Colangiopancreatografia Retrógrada Endoscópica , Páncreas , Conductos Pancreáticos , Enfermedad de von Hippel-LindauRESUMEN
'Num-chin syndrome', isolated mental neuropathy, is a rare manifestaion of malignant hernatologic diseases, but important sign for early diagnosis and prediction of clinical course and prognosis. Here we report 7 cases of numb-chin syndrome; one in leukemic transformation of malignant lymphoma, one in blastic crisis of chronic myelogenous leukemia and 5 in acute leukemia Two cases of acute leukemia revealed the 'numb-chin sign' in early course of disease before the diagnosis of leukemia and other three in aggravating state of acute leukemia. The therapeutic response and prognosis was poor and 5 cases expired in a few months.