Adrenal Lymphoma: Case Reports and Mini-review

Adrenal lymphoma is a rare condition which may occur in one of two forms; either as primary adrenal lymphoma (PAL), or secondary to a systemic lymphoma. Primary adrenal lymphoma is a very rare diagnosis and the most common histological pattern is diffuse large B-cell non‑Hodgkin lymphoma.

In this study, we represent two examples of adrenal lymphoma, primary and secondary. In addition, we have included a mini-review of the literature regarding this rare presentation.

We retrospectively reviewed all patients who were diagnosed with adrenal lymphoma in our hospital. We represent mainly the most two challenging cases where adrenal surgery was required to confirm the diagnosis. We have included a mini-review of the literature (PubMed data base: 1990 - 2020) on the clinical presentation and management of adrenal lymphoma cases.

Seventeen patients had adrenal lymphoma in our hospital; 16 of them had secondary involvement of the adrenal gland, while the last one had primary adrenal lymphoma. Patients with adrenal lymphoma mainly present with fever, lumbar pain, and/or symptoms of adrenal insufficiency. Primary adrenal lymphoma usually appears as heterogeneous complex large masses with low density on computerized tomography (CT) scan or magnetic resonance imaging (MRI); however, there is no pathognomonic features to diagnose PAL. The diagnosis is confirmed only with tissue biopsy. Chemotherapy is generally the standard treatment for lymphoma, while the role of surgery is limited.

The prognosis of these rare cases is generally poor with only about a third of patients achieving partial or complete remission following treatment.