A Case of Primary Adrenal Lymphoma: How Do We Detect Early Progression to Adrenal Insufficiency?

Introduction: Diffused large B cell lymphoma (DLBCL) is a type of aggressive non-Hodgkin’s lymphoma. Non-Hodgkins lymphoma arising in endocrine organs account for 3% of extra-nodal malignant lymphoma, and Primary adrenal lymphoma account for only 0.2% of them. Primary adrenal lymphoma involves the bilateral adrenals in 70% cases, and can cause adrenal insufficiency even if the lesion is unilateral.
Case Description: We present a 60-year-old female, who presented with constitutional symptoms and dyspnoea. On examination she was underweight, had marginally low blood pressure, and was desaturated. She had hyponatremia and high lactate dehydrogenase levels. 2D echocardiogram showed two echogenic
masses: one in the anterior pericardial space, and another in the right atrium obstructing the tricuspid origin. Ultrasound scan showed a left suprarenal mass, and bilateral pleural effusions. CECT showed the same enhancing mass involving tricuspid valve, which was extending into the right atrium, and right ventricle causing luminal narrowing and an enhancing solid lesion in the left adrenal gland (HU 30.8). 9am cortisol was 422 nmol/L, with high serum ACTH. She developed hypotension which responded to IV hydrocortisone. Adrenal biopsy revealed high grade DLBCL with Ki 67 index of 80%. She suddenly succumbed before definitive treatment.
Conclusion: Adrenal insufficiency in the setting of a unilateral non secretory adrenal lesion should rise the suspicion of Primary adrenal lymphoma rather than adrenal metastasis. Similarly, when biopsy of an adrenal lesion reveals Primary adrenal lymphoma rather than metastasis, there should be a higher index of suspicion to detect the presence of or progression to early adrenal insufficiency, even if the lesion is unilateral.