MGMT. OF COMPLEX CASES IN GI ONCOLOGY A Man with Klinefelter’ s Syndrome and New Abdominal Distension: A Discussion of Evaluation and Management Michael Postow & Manish A. Shah & Maeve Lowery & Ali Shamseddine & Aghiad El-Kutoubi & Ashwaq Al Olayan & Mohamed Naghy & Celina Ang & Sally Tamraz & Abdul-Rahman Jazieh & Eileen M. O’Reilly & David P. Kelsen & Ghassan K. Abou-Alfa Published online: 10 June 2011 # Springer Science+Business Media, LLC 2011 Case History A 50-year-old gentleman with Klinefelter ’ s syndrome (XXY karyotype) presented to his primary care physician with generalized abdominal discomfort. His pain was described as diffuse, constant, and associated with progressive abdominal distension. There was no clear exacerbation after eating, and he had no nausea, vomit- ing, constipation, diarrhea, or weight loss. Laboratory evaluation was remarkable for an elevated creatinine of 2.1 mg/dL. Liver function testing was notable for a mild transaminitis with AST 39 Units/L and ALT 73 Units/L (normal range, AST 10–37 Units/L and ALT 5–37 Units/ L), but total bilirubin and alkaline phosphatase were within normal limits. The coagulation profile was also within normal limits. The patient underwent an esoph- agogastroduodenoscopy which demonstrated chronic gas- tritis and a colonoscopy was performed and revealed a single tubulovillous adenoma 20 cm from the anal verge. To evaluate the elevated creatinine, a renal ultrasound was performed and showed unilateral, right-sided hydro- nephrosis, requiring a ureteral stent. Of note, the patient had undergone a cholecystectomy for symptomatic cholelithiasis in the remote past. Dr. Shah (New York): What is the appropriate work- up for new, unilateral hydronephrosis? Dr. Lowery (New York): Hydronephrosis refers to dilation of the renal pelvis as a result of distal urinary tract obstruction. If left uncorrected, hydro- nephrosis can result in impairment of renal func- tion, particularly when both kidneys are affected as is often seen in men with urinary retention due to prostatic hypertrophy. Unilateral hydronephrosis, present in this patient, raises the possibility of a focal abdominal or retroperitoneal lesion obstruct- ing the course of a single ureter. The obstruction may be due to enlarged retroperitoneal lymphade- nopathy, omental thickening, or bladder abnormal- ities involving the ureterovesicular junction. When renal insufficiency is believed to be due to urinary obstruction, relieving the obstruction is important, but this should be combined with efforts to understand the etiology of the obstruction. A non- contrast enhanced abdominal CT scan is an appro- priate next diagnostic test. Case History (Continued) The patient’ s abdominal pain continued, and a CT scan of his abdomen was performed (Fig. 1). Due to renal insufficiency, no intravenous contrast was administered. Dr. Abou-Alfa (New York): Dr. El-Kutoubi, can you please comment on the CT scan? M. Postow : M. A. Shah : M. Lowery : C. Ang : E. M. O’Reilly : D. P. Kelsen : G. K. Abou-Alfa (*) Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA e-mail: abou-alg@mskcc.org A. Shamseddine : A. El-Kutoubi : S. Tamraz American University of Beirut, Beirut, Lebanon A. Al Olayan : M. Naghy : A.-R. Jazieh Narional Guard Hospital, Riyadh, Saudi Arabia J Gastrointest Canc (2012) 43:314–318 DOI 10.1007/s12029-011-9294-9