Author/Authors :
Hari Kumar, K. V. S. Medwin Hospitals - Department of Endocrinology, India , Jha, Ratan Medwin Hospitals - Department of Nephrology, India , Shekhar, S. Medwin Hospitals - Department of Nephrology, India , Sunil, K. Medwin Hospitals - Department of Endocrinology, India , Modi, K. D. Medwin Hospitals - Department of Endocrinology, India
Abstract :
Von Hippel-Lindau (VHL) disease,which is an autosomal dominant inherited disease,is characterized by highly vascularized tumors in different organs. We report a 42-year-old male who presented to our hospital with diarrhea and weight loss of six months duration. Ultrasonography of the abdomen revealed bilateral polycystic kidneys with multiple cystic and solid components as well as polycystic pancreas. A computerized tomography scan of the abdomen revealed bilateral multiple simple and complex renal cysts,cystic lesions in the head and body of the pancreas and a non-enhancing lesion in the left adrenal gland. The features raised the possibility of VHL syndrome and a biopsy of the kidney revealed atypical cells with a suspicion of malignancy. He underwent bilateral nephrectomy and is on maintenance dialysis since then.
