Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com Case Report Med Princ Pract 2009;18:486–489 DOI: 10.1159/000235900 Concurrent Renal Cell Carcinoma and Central Nervous System Lymphoma in a Patient with Autosomal Dominant Polycystic Kidney Disease Ming-Yang Chang Yu-Ming Chen Yung-Cheng Chen Ya-Chung Tian Ji-Tseng Fang Chih-Wei Yang Department of Nephrology, Kidney Research Center, Chang Gung Memorial Hospital, Chang Gung University, Kueishan, Taiwan Introduction Autosomal dominant polycystic kidney disease (AD- PKD) is caused by mutations of PKD1 or PKD2, and has an incidence of 1/1,000 individuals [1]. Common compli- cations of ADPKD include renal insufficiency, hyperten- sion, liver cysts, and cerebral aneurysms [1]. Renal cell carcinoma is infrequent in ADPKD and may be difficult to identify due to its non-specific symptoms such as he- maturia, flank pain and palpable large kidneys [2, 3]. Pri- mary CNS lymphoma is a rare cancer that accounts for less than 3% of all brain tumors and is more commonly seen in immunocompromised patients [4] . Here, we pres- ent an unusual case of ADPKD with synchronous CNS lymphoma and renal cell carcinoma. Case Report A 58-year-old previously healthy woman had experienced progressive right hemiparesis for 2 months. She became confused and was brought to the hospital for further treatment. There was no fever, flank pain, or gross hematuria. Her parents had died of unknown causes, and her 3 children gave no history of systemic disease. On admission, her Glasgow Coma Score was E4V4M6 (E: eye opening, V: verbal response, M: motor response). She was afe- brile and normotensive. The muscle power of the right arm was 1, and that of the right leg was 3. She had left ptosis and pupil size was asymmetric (left: 5 mm, right: 3 mm). The abdomen was soft on examination without palpable mass. The patient had no skin Key Words Polycystic kidney disease  Lymphoma  Renal cell carcinoma Abstract Objective: To report an unusual case of synchronous renal cell carcinoma and CNS lymphoma in a patient with autoso- mal dominant polycystic kidney disease (ADPKD). Case Pre- sentation and Intervention: A 58-year-old woman present- ed with progressive right hemiparesis of 2 months’ duration. A brain CT scan revealed multiple enhanced lesions in the basal ganglia and the right occipital lobe. CNS lymphoma was confirmed by a stereotactic biopsy. Polycystic kidneys and a right renal mass were found incidentally. It was decid- ed to treat the patient with cranial radiotherapy and chemo- therapy first. The patient achieved complete remission of CNS lymphoma after 3 months, but the renal mass remained unchanged. A needle biopsy of the renal mass revealed renal cell carcinoma and unilateral nephrectomy was performed successfully. The patient remained in complete remission at 6-year follow-up. Conclusion: The patient was treated suc- cessfully with a combination of chemotherapy, radiothera- py, and unilateral nephrectomy. This report highlights the need for clinicians to remain alert to the possibility of double malignancies while caring for ADPKD patients, especially when multiple unexplained manifestations exist. Copyright © 2009 S. Karger AG, Basel Received: December 18, 2008 Revised: March 22, 2009 Dr. Ming-Yang Chang, MD, PhD Department of Nephrology, Kidney Research Center Chang Gung Memorial Hospital, 5 Fu-Shing St. Kueishan, Taoyuan 333 (Taiwan) Tel. +886 3 328 1200 ext. 8181, Fax +886 3 328 2173, E-Mail mingyc@adm.cgmh.org.tw © 2009 S. Karger AG, Basel 1011–7571/09/0186–0486$26.00/0 Accessible online at: www.karger.com/mpp Downloaded by: Chang Gung Memorial Hospital - LinKou Branch 61.220.52.4 - 4/22/2015 8:16:35 AM