[Rosai-Dorfman disease presented with involvement of the adrenal gland. A clinical case reported]

Adrenal gland involvement is a very rare extranodal presentation of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Its pathogenesis still remains unknown with many theories about its probable cause. Symptoms and signs of adrenal involvement presentation are uncertain. Although, it is a disease with a good prognosis and a favorable clinical course in most cases, the low index of suspicion and difficulty in radiological differential diagnosis lead to radical treatment.

We present a case of Rosai-Dorfman disease with right adrenal plus left infrahiliar nodal involvement. It was treated with adrenalectomy and node exeresis. The infrahilial involvement evolution was appropriate.

Rosai-Dorfman disease is a rare disease and the extra-nodal presentation is even more rare. The incidence and evolution of this disease in the adrenal gland is unknown. The presence of this pathology should be considered if the patient shows adrenal gland affected and lymph node growth, in order to consider the individualized treatment, which may vary (treatment with corticosteroids, chemotherapy and/or radiotherapy).