Clinical Genitourinary Cancer August 2009 E45 Adenocarcinoma of Unknown Primary in a 20-Year-Old African American Male Derek Power, Diane Reidy Lagunes Memorial Sloan-Kettering Cancer Center, New York, NY Clinical Genitourinary Cancer, Vol. 7, No. 2, E45-E48, 2009 Keywords: Hematuria, Renal medullary carcinoma, Sickle cell disease, Video- assisted thoracoscopic surgery DOI: 10.3816/CGC.2009.n.023 Submitted: Aug 18, 2008; Revised: Nov 5, 2008 Accepted: Nov 24, 2008 Address for correspondence: Derek Power, MD, Memorial Sloan-Kettering Cancer Center, Medicine, 1275 York Avenue, New York, NY 10021 Fax: 1-212-794-7186; e-mail: powerd@mskcc.org Case Report A 20-year-old African American college student presented to the emergency room with a 1-month history of intermittent left flank pain and painless pass- ing of light pink colored urine. He had a personal and family history of sickle cell trait, no history of sickle crisis and no other relevant medical or surgical history. He was a life-long never-smoker and was not taking any medications. One month previously, a renal biopsy of his left kidney was performed because of similar symptoms and revealed a “minor glomerular membrane abnor- mality,” mild arteriosclerosis, and unremarkable immunologic and electron microscopy studies. Urine analysis was positive (2+) for protein and blood. A full blood count and comprehensive metabolic profile was normal with a serum creatinine of 1 mg/dL and a lactate dehydrogenase of 200 U/L. A small population of partially sickled cells was seen on peripheral blood smear but the overall morphology was normal. Hemoglobin electrophoresis revealed 41.8% HbS diagnostic of sickle cell trait. Autoantibody screen was negative and complement levels were normal. Urine collection over 24 hours revealed 300 mg of protein and a creatinine clearance of 90 mL/min. A noncontrast computed tomography (CT) scan of the abdomen showed mild left hydro- nephrosis and proximal hydroureter, no nephrolithiasis, and shotty left-sided A 20-year-old African American male presented with a history of left flank pain and passing of light pink urine. Medical history included sickle cell trait. Urine analysis was positive for protein and blood. Metabolic profile, autoantibody screen, and complement levels were normal. Hemoglobin electrophoresis revealed an 41.8% HbS diag- nostic of sickle cell trait. Creatinine clearance was normal and pro- teinuria was nonnephrotic. A noncontrast computed tomography (CT) scan showed left proximal hydronephrosis. Urology follow-up was ar- ranged and the differential included renal papillary necrosis, or renal cyst rupture. He presented 3 months later with sudden onset left flank pain and gross hematuria. Serum creatinine was 1.8 mg/dL. Computed tomography scan with contrast revealed innumerable lung lesions, an enlarged heterogenously enhancing left kidney, and retroperitoneal adenopathy. Ultrasound revealed an obstructed left collecting system and a 14-cm enlarged left kidney with no discrete mass. Testicular markers/ultrasound, upper/lower endoscopies were normal. Lung biopsy revealed poorly differentiated adenocarcinoma positive for cytokeratin 7. Renal, sarcoma, and gastrointestinal mark- ers were negative. By exclusion, it appeared that the patient had a carcinoma of unknown primary. However, with the clinical and per- sonal history, a diagnosis of renal medullary carcinoma (RMC) was made. RMC is a rare and highly malignant tumor that should always be included in the differential of a patient with sickle cell disorder and hematuria. Renal biopsy typically fails to sample the renal medulla and radiologic findings might not raise the suspicion of a renal tumor. Thus, clinical suspicion must always be high in order to preserve the patient’s only chance of prolonged survival. Abstract Case Report This article might include the discus- sion of investigational and/or unla- beled uses of drugs and/or devices that might not be approved by the FDA. Electronic forwarding or copying is a violation of US and international copyright laws. Authorization to photocopy items for internal or personal use, or the internal or personal use of specific clients, is grant- ed by CIG Media Group, LP, ISSN #1558-7673, provided the appropriate fee is paid directly to Copyright Clearance Center, 222 Rosewood Drive, Danvers, MA 01923 USA. www.copyright.com 978-750-8400.