Contributed by John A Ozolek, MD, Eizaburo Sasatomi, MD, PhD, Patricia A Swalsky, BS, Sydney D Finkelstein, MD and Rajiv Dhir, MD
Published on line in May 2003
This elderly man had a medical and oncologic history significant for a myeloproliferative disorder in the remote past, chronic lymphocytic leukemia, recurrent osteochondroma, prostatic adenocarcinoma, renal insufficiency and coronary artery disease. Past surgical history is notable for a left nephrectomy for "benign" renal disease 28 years earlier and partial colectomy for severe polyposis. He presented for total hip replacement due to worsening hip pain unresponsive to analgesic treatments leaving him virtually bedridden. Several days before his admission, he developed nausea and vomiting with loss of appetite and indeterminate weight loss. Physical examination showed only a slightly enlarged prostate and an abdominal hernia. No abdominal masses or lymphadenopathy were appreciated. Laboratory evaluation then included a complete blood count showing a white blood cell count of 50,700/cu mm with a differential of 6% polymorphonuclear leucocytes, 89% lymphocytes, 3% monocytes, 1% eosinophils, and 1% basophils. Hemoglobin was 11.6 g/dl and platelet count 168,000/cu mm. Electrolytes were within normal limits. Blood urea nitrogen was 32 mg/dl and creatinine 1.7 mg/dl. Liver enzymes, alkaline phosphatase, and total bilirubin were within normal limits. Albumin was marginally low at 3.1 g/dl. Prostatic specific antigen level was 0.1 ng/ml. An abdominal and retroperitoneal ultrasound was performed revealing a 12 cm by 9.5 cm lobulated, hypoechoic soft tissue mass with central necrosis in the right suprarenal region. Subsequent computed tomography scan demonstrated a 10.3 cm by 9.3 cm lobulated heterogeneous right adrenal mass. The mass displaced the right kidney posteriorly and inferiorly with focal compression of the inferior vena cava and extending to the renal hilum (Figures 1A, 1B, and 1C). CT guided biopsy of the mass was diagnosed as cytologically and histologically bland adrenal cortical tissue. The patient subsequently underwent exploratory laparotomy with resection of the adrenal mass. Intraoperatively, a large necrotic appearing tumor encompassed the right adrenal gland, but extended to and was fixed to the inferior vena cava. The tumor was also seen to extend under the right renal vein and involve the right renal hilum. The tumor was incompletely resected secondary to fixation to adjacent structures and intraoperative hemorrhage. This final pathologic diagnosis in this case was based on a compilation of pathologic data including immunohistochemistry, electron microscopy, and mutational profiling.