Management of a patient with Rosai–Dorfman syndrome and large adrenal pheochromocytoma

• Main Authors: Taha O Mahwi, Najmaddin Khoshnaw
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2018-01-01
• Series: Iraqi Journal of Hematology
• Subjects: Adrenal pheochromocytoma; Rosai–Dorfman syndrome; sinus histiocytosis
• Online Access: http://www.ijhonline.org/article.asp?issn=2072-8069;year=2018;volume=7;issue=1;spage=45;epage=47;aulast=Mahwi

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 extra-lymphatic soft tissue involvement. The concomitant presentation of RDS with an adrenal tumor is rarely encountered among our patients. Here in we describe a middle-aged man, presented with a chronic history of repeated LN enlargement. The nodes had mild local discomfort and tenderness with associated fever. LN biopsy showed sinus histiocytosis with lymphophagocytosis characterized as emperipolesis and diagnosis of RDS was decided. After one year, the patient presented with high blood pressure, raised blood sugar, obesity, and abdominal pain. Computerized tomography showed a large adrenal mass, and the patient was treated urgently for his malignant blood pressure followed by surgical excision of the left adrenal mass. The biopsy subsequently revealed non-malignant adrenal tumor (pheochromocytoma). We are reporting successful treatment of rare case with RDS concomitantly presented with large adrenal pheochromocytoma. He was treated medically and surgically, the patient got recovery. RDS is a rare disease but we should consider it in patients with repeated benign lymphadenopathy. we are emphasizing that knowledge about RDS and its disease associations may be informative to the general medical community.