Adrenal Insufficiency Due to Primary Bilateral Adrenal Non-Hodgkin's Lymphoma

Abstract

A case of primary adrenal insufficiency, secondary to primary bilateral adrenal lymphoma is reported. A 50-year-old woman presented with features of primary adrenal insufficiency (darkening of skin, asthenia, anorexia, constipation) for at least 8 months. Clinical examination was unremarkable except for low body mass index and generalized skin and buccal mucosal pigmentation. Routine investigations including complete hemogram, serum chemistry, urine analysis, chest radiograph and electrocardiogram were normal; serum lactate dehydrogenase was moderately elevated. Primary adrenal insufficiency was confirmed on cortisol dynamics (very low basal and peak cortisol) after insulin-induced hypoglycemia. Routinely detected adrenal masses on ultrasonography were confirmed by contrast enhanced CT abdomen. A diagnosis of primary adrenal non- Hodgkin's lymphoma (B-cell) was made after exploratory laprotomy and further staging. The patient was put on combination chemotherapy (CHOP) protocol, but was lost to follow-up after receiving two cycles of treatment. Primary adrenal lymphoma, although a rare entity, needs to be suspected in patients with features of primary adrenal insufficiency who have evidence of bilateral adrenal masses on imaging.

Primary adrenal lymphoma: Differential involvement with varying adrenal function

“…[8] Thus, patient may present with fever, lumbar pain, and or symptoms of adrenal insufficiency. [610] Our cases represent two ends of the spectrum, viz, abdominal pain and marked weight loss as predominant manifestations in one with unilateral lesion, while as adrenal failure was the presentation in the second one with bilateral involvement. Primary adrenal lymphomas should be considered in patients with elevated serum lactate dehydrogenase, characteristic CT findings of appearance of enlargement of adrenal gland with maintenance of adreniform shape with variable density of the lesion, and primary adrenal insufficiency.…”

“…Primary adrenal lymphomas should be considered in patients with elevated serum lactate dehydrogenase, characteristic CT findings of appearance of enlargement of adrenal gland with maintenance of adreniform shape with variable density of the lesion, and primary adrenal insufficiency. [910] Magnetic resonance imaging enables differentiation of adrenal adenomas from other adrenal malignancies, but specific diagnosis might not be achieved during the study. The initial diagnosis can usually be established on the basis of image-guided FNA/ biopsy; however, it is still considered inadequate for diagnosis by many clinicians.…”

Histopathological Experience of Primary Adrenal Lymphoma From Two Tertiary Hospitals

“…Suggested theories on the pathogenesis of PAL include the presence of autoimmune adrenalitis, immune dysfunction, viral infection such as human immunodeficiency virus (HIV) and Epstein virus (EBV), and genetic mutation of p53 and the c-kit gene [30][31][32]. The homing theory of hematopoietic tissue may explain the bilateral adrenal lymphomas with a lack of bone marrow and nodal involvement [33]. Rarely, lymphoproliferative disorders can develop in the adrenal gland as a result of immunosuppressive therapy for organ transplants [34][35][36][37][38] or other diseases [39].…”