Primary Adrenal Lymphoma Presenting with Adrenal Failure: A Case Report and Review of the Literature

Abstract

IntroductionPrimary adrenal lymphoma is rare, with a few cases reported in the literature. Most often it manifests as bilateral adrenal lesions and adrenal insufficiency is a common complication.Case PresentationA 53-year-old male was referred with abdominal discomfort and darkening of the skin since 1 month prior to admission. His workups detected large bilateral adrenal masses. The patient was admitted due to hypotension, and was diagnosed with adrenal insufficiency. Laboratory studies showed high erythrocyte sedimentation rate (ESR), lactate dehydrogenase (LDH), and adrenocorticotropin levels. There was no other organ involvement and computed tomography (CT)-guided percutaneous biopsy of the adrenal gland revealed B-cell type of non-Hodgkin lymphoma. Replacement therapy with glucocorticoid and mineralocorticoid was initiated with remission of symptoms. After 3 months of chemotherapy his condition improved, but the patient worsened thereafter and died 2 months later.ConclusionsHowever, this case reminded the importance of considering primary adrenal lymphoma in the differential diagnosis of bilateral adrenal masses, especially if the patient presents with adrenal insufficiency.

Asymptomatic Unilateral Primary Adrenal Lymphoma Mimicking a NonFunctioning Adenoma in Adult Male with a Good Outcome

“…PAL is extremely uncommon; accounting for only 3% of primary extranodal lymphomas, and less than 1% of all non-Hodgkin's lymphomas (NHL) [1][2][3][4]. The aetiology of PAL is unclear; however, possible but inconclusive theories include pre-existing autoimmune adrenalitis with lymphocytic infiltration or hematopoietic rest tissue in the adrenal glands [3,4]. Furthermore, Epstein-Barr viral infection or mutations in p53 and other genes may also play a role in the tumor pathogenesis [4,5].…”

“…PAL is primarily bilateral; however, unilateral involvement of the adrenal gland is scarcely reported and is usually secondary to malignancies elsewhere that disseminated [4,6]. Studies suggest a predominance in the male population [4,6].…”

“…PAL is primarily bilateral; however, unilateral involvement of the adrenal gland is scarcely reported and is usually secondary to malignancies elsewhere that disseminated [4,6]. Studies suggest a predominance in the male population [4,6]. Though, most cases seen are usually asymptomatic and are found incidentally on radiology scans or on post-mortem examination [4,5,7].…”

“…Studies suggest a predominance in the male population [4,6]. Though, most cases seen are usually asymptomatic and are found incidentally on radiology scans or on post-mortem examination [4,5,7]. It has been noticed that elderly male patients are mostly affected by PAL, presenting with fatigue, abdominal pain and frequent association with symptoms of adrenal insufficiency or failure [2].…”

“…Rituximab-CHOP chemotherapy regimen is the commonly recommended first line treatment, with laparoscopic adrenalectomy as adjuvant to chemotherapy for the larger adrenal masses [4,7]. Despite various treatment modalities, PAL is generally known for its poor prognosis, which could be attributed to advanced age with symptoms of adrenal insufficiency at presentation, larger tumors and bilateral adrenal gland involvement [7].…”