Pediatric Hematology and Oncology, 27:490–495, 2010 Copyright C  Informa Healthcare USA, Inc. ISSN: 0888-0018 print / 1521-0669 online DOI: 10.3109/08880018.2010.493576 Case Report SARCOIDOSIS PRESENTING WITH MASSIVE SPLENOMEGALY IN A CHILD WITH A HISTORY OF IRIDOCYCLITIS AND SENSORINEURAL DEAFNESS Abdulrahman Alsultan, MD ✷ Department of Pediatrics, College of Medicine, King Saud University and King Khalid University Hospital, Riyadh, Saudi Arabia Emad Raddaoui, MD ✷ Department of Pathology, College of Medicine, King Saud University and King Khalid University Hospital, Riyadh, Saudi Arabia Mohamed Elfaki Osman, MD ✷ Department of Pediatrics, College of Medicine, King Saud University and King Khalid University Hospital, Riyadh, Saudi Arabia Saleh Othman, MD ✷ Department of Nuclear Medicine, College of Medicine, King Saud University and King Khalid University Hospital, Riyadh, Saudi Arabia Mohamed Khalifah, MD and Sarah Alsubaie, MD ✷ Department of Pediatrics, College of Medicine, King Saud University and King Khalid University Hospital, Riyadh, Saudi Arabia ✷ Childhood sarcoidosis is a rare multisystemic disorder that can have variable clinical presen- tations. A triad of skin, eye, and joint involvement is common in children younger than 5 years; however, pulmonary disease is more common in older children, similar to adults. The authors report the case of a 10-year-old girl who presented solely with massive splenomegaly. Her history was signif- icant for iridocyclitis and unilateral sensorineural deafness at 6 and 7 years of age, respectively. A gallium scan showed diffuse splenic uptake, and the pathology of the spleen was consistent with a noncaseating granuloma, with no evidence of malignancy. A work-up for infectious etiology was un- remarkable. This case demonstrates that the challenge in diagnosing sarcoidosis in young children stems from its ability to present in several unique clinical scenarios. It also reinforces the importance of tissue evaluation and the exclusion of other differential diagnoses, such as lymphoma, to confirm the diagnosis of sarcoidosis. Received 5 November 2010; accepted 4 March 2010. Address correspondence to Abdulrahman Alsultan, MD, Assistant Professor and Attending Physi- cian, Hematology & Oncology Section, Department of Pediatrics, College of Medicine, King Saud University and King Khalid University Hospital, P O Box 261691, Riyadh, Saudi Arabia 11342. E-mail: aalsultan@gmail.com 490