Primary bilateral adrenal lymphoma associated with idiopathic thrombocytopenic purpura

Autoimmune disorders are occasionally associated with malignant lymphoma. To date only one case of primary adrenal lymphoma associated with idiopathic thrombocytopenic purpura (ITP) has ever been reported. This paper reports the case of a 63-year-old man with bilateral adrenal masses whose laboratory data showed decreased platelet count. Despite normal blood pressure, the adrenal tumors endocrinologically appeared to be pheochromocytoma. Core needle biopsy was not done due to thrombocytopenia attributed to concurrent ITP. After intravenous immunoglobulin treatment, splenectomy and bilateral adrenalectomy were performed since the first pathological findings of the frozen specimen suggested the possibility of a poorly-differentiated carcinoma. Immunohistochemical study finally showed the tumors to be diffuse large B-cell lymphoma. The patient underwent a subsequent course of combination chemotherapy and survived 6 years recurrence-free without any need for further treatment other than steroid replacement. The coincidence of adrenal lymphoma and ITP should be considered even if another kind of tumor is suspected, and core needle biopsy should be performed prior to operation, since the specific kind of tumor found alters the therapeutical strategy adopted.