CASE REPORT Littoral Cell Angioma of the Spleen Mimicking Posttransplantation Lymphoma in a 63-Year-Old Renal Transplant Patient Anja Susanne Mühlfeld, MD, 1 Frank Eitner, MD, 1 Alberto Perez-Bouza, MD, 2 Ruth Knuechel, MD, 2 Bernhard Heintz, MD, 1 and Jürgen Floege, MD 1 In addition to lymphomas, vascular tumors represent the most common neoplasms of the spleen. Littoral cell angiomas are benign vascular tumors originating from the littoral cells lining the splenic sinuses. In this report, we describe the case of a 63-year-old patient who developed night sweats 16 months after renal transplantation. Diagnostic workup showed multiple splenic masses believed to represent lymphoma infiltration to the spleen. Lymph nodes and bone marrow were unaffected, and diagnostic splenectomy was performed. Histological examination of the pathological specimen from the splenectomy specimen showed multiple littoral cell angiomas of the spleen. We recommend that physicians involved in the area of organ transplantation, especially kidneys, remain alert for other rarer splenic lesions in transplant recipients than posttransplantation lymphoma. More specific tools need to be developed to aid in the differential diagnosis of splenic masses to avoid splenectomy in patients with littoral cell angiomas. Am J Kidney Dis 52:e11-e14. © 2008 by the National Kidney Foundation, Inc. INDEX WORDS: Littoral cell angioma; lymphoma; splenic tumor; vascular tumor; night sweats. L ittoral cell angiomas are recently recognized vascular tumors of the spleen. They were described as a distinct entity by Falk et al 1 in 1991 after reviewing 200 cases of benign vascu- lar tumors of the spleen. Littoral cell angiomas arise from the littoral cells lining the sinuses of the splenic red pulp. In most patients, the diagno- sis is made while evaluating for asymptomatic splenomegaly. However, some patients present with symptoms of hypersplenism such as anemia and thrombocytopenia or other nonspecific symp- toms. Although computed tomographic (CT) mor- phological and ultrasound features of the tumor have been well described, 2,3 they are nonspecific and the diagnosis often is made by means of histological examination of the splenectomy specimen. Pathological features consist of one or multiple splenic nodules of variable size. Histo- logically, the tumors consist of anastomosing vascular channels, often with papillary projec- tions and cyst-like spaces. These are lined by tall cells expressing both endothelial and histocytic cell markers. 1 In this case report, we describe a renal trans- plant recipient with multiple littoral cell angio- mas of the spleen. CASE REPORT A 63-year-old patient presented to our transplant clinic reporting night sweats and nasal congestion 16 months after renal transplantation. His general practitioner had prescribed a week-long course of cefuroxime for sinusitis, but symp- toms had not ceased. The patient’s medical history was remarkable for kidney transplantation 16 months before presentation. End-stage renal disease was caused by presumed chronic glomerulone- phritis. Immunosuppression consisted of prednisolone, my- cophenolate mofetil, and cyclosporine A. At the time of presentation, laboratory examination re- sults were unremarkable. There was no sign of systemic inflammation, with C-reactive protein level less than 5 mg/L and a normal blood cell count. Lactate dehydrogenase level was also normal at 219 U/L, as were liver enzyme levels. Kidney function was excellent, with a creatinine level of 0.9 mg/dL (80 mmol/L) and a measured creatinine clearance of 144 mL/min (2.4 mL/s). Physical examination showed no abnormal findings. An ultrasound of the abdomen and neck had been performed 4 months before without pathological changes. A new examination showed no pathological changes in the liver, pancreas, and native kidneys, whereas the spleen now showed multiple echo-dense masses. CT scan of the neck, thorax, and abdomen was obtained, but showed no From the 1 Division of Nephrology and Immunology and 2 Institute of Pathology, University Hospital of the RWTH Aachen, Aachen, Germany. Received September 17, 2007. Accepted in revised form January 2, 2008. Originally published online as doi: 10.1053/j.ajkd.2008.01.033 on May 14, 2008. Address correspondence to Anja Susanne Mühlfeld, MD, University Hospital of the RWTH Aachen, Division of Nephrology and Immunology, 52074 Aachen, Germany. E-mail: amuehlfeld@ukaachen.de © 2008 by the National Kidney Foundation, Inc. 0272-6386/08/5203-0040$34.00/0 doi:10.1053/j.ajkd.2008.01.033 American Journal of Kidney Diseases, Vol 52, No 3 (September), 2008: e11-e14 e11