Adrenal Lymphoma

Lymphoma can afflict the adrenal glands in two forms: primary disease, arising from the adrenal gland itself, and secondary disease, due to metastasis from another site. Secondary adrenal lymphoma is more common, having been identified in up to 25% of all non-Hodgkin’s lymphoma patients at autopsy [8]. The disease usually originates from either the retroperitoneum or the ipsilateral kidney [1]. Primary adrenal lymphoma is very rare with only 100 cases having been reported worldwide [6].

The most common histologic type of primary adrenal lymphoma is diffuse large B-cell. T cell primary adrenal lymphomas are uncommon and only two have been reported in the literature worldwide [2]. Although the exact pathologic cause of the disease is unknown, primary adrenal lymphoma likely arises from hematopoietic tissue inherent to the adrenal gland, with Epstein-Barr virus as a possible causative agent [2].

Wang’s survey of international case reports concluded that patients with primary adrenal lymphoma have a median age at presentation of 68 with a male to female ratio of 2.2 to 1 [2]. The majority of primary adrenal lymphomas are bilateral at presentation and half of these have symptoms of adrenocortical insufficiency. There was no relationship between tumor size and adrenal insufficiency [2]. Symptoms of primary adrenal lymphoma are non-specific and include fever of unknown origin, weight loss, fatigue, and hyperpigmentation [6].

Primary adrenal lymphoma commonly appears as a complex mass on both CT and MRI, although a few cases have shown a homogenous density on CT [2,4]. Lesions with cystic necrosis have also been documented [2]. Radiographic imaging with MRI shows the tumors with low signal intensity on T1-weighted MR images and high signal intensity on T2-weighted images [5]. However, the appearance of lymphoma in the adrenal gland is nonspecific and cannot differentiate primary from secondary disease.

Diagnosis of primary adrenal lymphoma can be suspected with an adrenal mass on imaging, no evidence of nodal involvement at first admission, and absence of a leukemic blood picture and other organ involvement [2]. The diagnosis must then be confirmed with a percutaneous aspiration or core needle biopsy of the mass to initiate treatment [3]. The current therapies include corticosteroid replacement and surgical excision with or without radiation and chemotherapy. Prognosis is poor with over half of the patients reported in the literature dying within one year from their diagnosis [6].