Primary adrenal lymphoma more commonly affects older men with a median age of 68 and has a male-to-female ratio of 2.2 to 1
2). Affected patients may manifest with adrenocortical insufficiency (36% of cases), even when the neoplasm is small in size, and some as Addisonian crisis
5–7). Other presenting symptoms include fever (46%), weight loss (24%) and abdominal pain (26%)
2). Bilateral primary adrenal lymphoma (70%) appears to predominate over the unilateral counterpart
2). Most cases are discovered incidentally on abdominal ultrasound and CT
8). There is no pathognomonic appearance on CT or US to indicate lymphomatous involvement of the adrenal gland
9). Although the sonographic appearance may vary from anechoic or hyperechoic to mixed hypoechoic and hyperechoic lesions, the most common is the hypoechoic variety
10). On CT, primary adrenal lymphoma tends to appear as complex masses with variable fluid contents
4). However, a few cases also showed homogeneous density
11). Frederick et al.
4) reported a case of primary adrenal lymphoma with CT appearance of a cystic mass containing mural calcification. In contrast, a secondary adrenal lymphoma most commonly appears as a solid homogeneous mass of soft tissue density distorting the adrenal shape and shows contrast enhancement
11). The diagnosis can be confirmed by image-guided percutaneous biopsy of the adrenal gland. This procedure should not be performed until endocrine evaluation has been completed
12). Adrenal aspiration is a sensitive and safe method for diagnosing metastasis to the adrenal gland, adrenal lymphoma, and an infectious process
13). Over 90% of primary adrenal lymphoma are of B-cell lineage and over 80% are of diffuse large-cell type
8). The prognosis of primary adrenal lymphoma is very poor, with a duration of survival ranging from 3 days to 26 months
2). There was one case report of long-term survival for 8 years following surgery and adjuvant radiation therapy
8). Indicators of a poor prognosis are an advanced age, a large tumor, adrenal insufficiency at the time of presentation which indicates extensive adrenal destruction, and a high level of serum lactate dehydrogenase
12). In conclusion, primary adrenal lymphoma should be included in the differential diagnosis of a retroperitoneal mass. Early diagnosis and intervention may dramatically affect the clinical outcome.