Primary adrenal lymphoma: Differential involvement with varying adrenal function

Abstract

Primary adrenal Non-Hodgkin's lymphoma is rare. The symptoms of the disease and response to treatment are variable depending on the type of lymphoma, tumor size, and presence of adrenal insufficiency. We report two cases of primary adrenal lymphoma who had varied presentations. One presenting with abdominal pain and weight loss was documented to have unilateral disease without any adrenal insufficiency and showed a good response to combination chemotherapy, while the second one had bilateral adrenal involvement with adrenal insufficiency and died after second chemotherapy. Functional adrenal involvement in lymphoma depends on the extent of involvement; patients with bilateral involvement almost always have adrenal insufficiency.

Origin of primary adrenal lymphoma and predisposing factors for primary adrenal insufficiency in primary adrenal lymphoma

“…[1] The authors have presented a good and exhaustive literature review on the topic, but have not discussed the origin of lymphoma primarily from the adrenals.…”

“…[1] Adrenal insufficiency in patients with bilateral adrenal metastasis is rare (1%)[4] and develops only when >90% of the adrenal tissue is infiltrated. [5] In contrast, a very high frequency of primary adrenal insufficiency (60%-70%) has been reported in patients with primary adrenal lymphomas, including patients with mildly enlarged adrenals also.…”

Bilateral primary adrenal non-Hodgkin′s lymphoma without adrenal insufficiency

“…[ 8 ] Due to the rich blood supply to the adrenal glands, hematopoietic metastasis to these glands is much more common than lymphatic metastasis. Cancer arising in the adrenal tissue is most commonly due to metastatic infiltration of carcinomas originating in the lungs, breast, skin, and colon;[ 3 ] all of which our patient lacked. Lymphatic metastasis to the adrenal glands most commonly originates from the retroperitoneal lymph nodes or the ipsilateral kidney,[ 5 ] none of which were enlarged on CT.…”

“…Although the majority of primary adrenal lymphomas have been shown to cause adrenal insufficiency, metastatic NHL to the adrenals has only been shown to do so on few occasions. [ 3 5 ] In their study Gamelin et al . they found that of 127 patients with widespread NHL, only 4 had adrenal insufficiency.…”

“…[ 2 ] As of the year 2008, only 116 of cases of primary lymphoma had been reported; 70% of these cases have presented bilaterally. [ 3 4 ] Of the primary adrenal non-Hodgkin's lymphoma (NHL) cases reported to date, 60-70% present with adrenal insufficiency at the time of diagnosis. [ 5 6 ] In the case reported below, the bilateral primary adrenal NHLs were an incidental finding on CT and lacked the associated adrenal insufficiency seen in the majority of cases.…”