TY - JOUR ID - TI - Management of a patient with Rosai–Dorfman syndrome and large adrenal pheochromocytoma AU - Taha O. Mahwi AU - Najmaddin Khoshnaw PY - 2018 VL - 7 IS - 1 SP - 45 EP - 47 JO - Iraq Joural of Hematology المجلة العراقية لامراض الدم SN - 20728069 25432702 AB - Sinus histiocytosis with massive lymphadenopathy that known as Rosai Dorfman syndrome (RDS)is a rare histiocytic disease which includes benign lymph nodes (LN) enlargement with extralymphaticsoft tissue involvement. The concomitant presentation of RDS with an adrenal tumor israrely encountered among our patients. Here in we describe a middle-aged man, presented with achronic history of repeated LN enlargement. The nodes had mild local discomfort and tendernesswith associated fever. LN biopsy showed sinus histiocytosis with lymphophagocytosis characterizedas emperipolesis and diagnosis of RDS was decided. After one year, the patient presented with highblood pressure, raised blood sugar, obesity, and abdominal pain. Computerized tomography showeda large adrenal mass, and the patient was treated urgently for his malignant blood pressure followedby surgical excision of the left adrenal mass. The biopsy subsequently revealed non-malignantadrenal tumor (pheochromocytoma). We are reporting successful treatment of rare case with RDSconcomitantly presented with large adrenal pheochromocytoma. He was treated medically andsurgically, the patient got recovery. RDS is a rare disease but we should consider it in patients withrepeated benign lymphadenopathy. we are emphasizing that knowledge about RDS and its diseaseassociations may be informative to the general medical community.

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