Endocrine Abstracts

JOINT1812

Introduction: Primary adrenal lymphoma (PAL) is a rare malignancy, accounting for <1% of extra-nodal lymphomas yet highly aggressive and invasive disease. Timely diagnosis is crucial for improved prognosis. We describe ten cases of PAL seen in our department who presented with diverse clinical presentations, posing challenges in both diagnosis and treatment.

Patients and Methods: Ten cases of PAL were confirmed by histopathology and immunohistochemistry from a retrospective cohort of 125 cases of primary adrenal insufficiency admitted in the Endocrinology ward over 15 years duration from 2010-2025. Data-analysis was performed using SPSS 27.

Results: The median age at presentation was 53 years (range: 22–70 years), 7 were males and had symptom duration of 1–4 months. Features of adrenal insufficiency like nausea, vomiting, hyperpigmentation weight loss, and loss of appetite were reported in 7 out of 10 cases and were confirmed in all 7. Fever and B symptoms were present in 8 out of 10 cases. Anemia was present in 9 out of 10 cases, while pancytopenia was observed in 1. Hyponatremia was noted in 8 out of 10 cases, and severe hypercalcemia occurred in 1 case. The mean LDH level was 1796 U/l (range: 703–3584 U/l) normal range (85-450). All patients had bilateral adrenal enlargement with lymphadenopathy in 8 out of 10 cases, hepatomegaly in 3, and splenomegaly in 2 out of 10 cases. The adrenal gland contour was oval in 5 out of 10 cases, irregular in 4 cases, and round in 1. All 10 cases were identified as B-cell non-Hodgkin lymphoma (NHL). Five patients received the R-CHOP regimen, and one received the R-CVP regimen. Chemotherapy was not initiated in four patients due to poor performance status. The median survival was 8 months (range: 4–30 months) in the chemotherapy group compared to 1 month (range: 1–4 months) in the non-chemotherapy group. Survival was 1–3 months in 3 cases, 3–6 months in 3 cases, 6–12 months in 1 case, and over 12 months in 1 case.

Conclusion: In India, tuberculosis and histoplasmosis are the leading causes of primary adrenal insufficiency with bilateral adrenal masses. PAL should be considered if infectious causes are ruled out, as its treatment differs and carries a poor prognosis. PAL prognosis is generally unfavorable, with predictors of poor outcomes including older age, large tumor size, bilateral adrenal involvement, adrenal insufficiency at presentation, elevated LDH levels, and non-germinal B-cell lymphoma.