Primary Adrenal Lymphoma: Clinical Experience and Outcomes in a Single Tertiary Referral Centre in India
Shubhashis Saha, Felix K. Jebasingh, Anu Korula, Elanthenral Sigamani, Shweta Singh, Krupa George, Ramya Iyyadurai, Santhosh A. Raj, Kripa E. Cherian, Nitin Kapoor, Hesarghatta S. Asha, Nihal ThomasAbstract
Introduction:
Primary adrenal lymphoma (PAL) is an extranodal lymphoma with common bilateral involvement and poor prognosis. Our case series describes the clinical presentation, biochemistry, imaging, histopathology, and treatment outcomes of patients with PAL at a tertiary care centre.
Methods:
We analysed case records and conducted telephonic follow-up of PAL patients at our institution from January 2011 to January 2022.
Results:
Sixteen patients were included, with a mean age of 52.5 ± 12.3 years (range: 26–69) and a 75% male predominance. Nine (53.8%) patients presented with abdominal pain, and 81.25% had weight loss or fever. Bilateral adrenal involvement occurred in nine (56.25%) patients, with four having adrenal insufficiency. Serum lactate dehydrogenase (1657.5 ± 1049.3 U/L) and C-reactive protein (67.35 ± 61.00 mg/L) were elevated. Lymph nodal disease was present in 68.75% of patients, and secondary extra-nodal spread in 56.25%. Diffuse large B-cell lymphoma (DLBCL) was diagnosed in 87.5% (12/14) of patients, with six showing a double-expressor phenotype. All patients received R-CHOP chemotherapy or variations; two underwent surgeries, and one received radiation. The median survival was 4.0 months (range, 0–142 months), with a 2-year survival rate of 18.18%.
Conclusion:
PAL is a rare non-Hodgkin lymphoma with a poor prognosis, considered in diagnosing adrenal masses. DLBCL is its most common subtype, and chemotherapeutic agents are the treatment of choice. Patients can present with adrenal crisis or features of Addison’s disease (25%) and hence should be considered as one of the differential diagnoses in patients with a short history of Addison’s disease with fever, weight loss, and less hyperpigmentation.