Endocrinol Metab.  2011 Mar;26(1):101-105. 10.3803/EnM.2011.26.1.101.

Primary Bilateral Adrenal Non-Hodgkin's Lymphoma Presented with Adrenal Insufficiency: A Case Report

• Lee EY
• Kim KM
• Kim KJ
• Noh S
• Kim JS
• Yang WI
• Lim SK

• KMID: 2169134
• DOI: http://doi.org/10.3803/EnM.2011.26.1.101

Abstract

Primary adrenal lymphoma is a very rare disease and it is known to have a poor prognosis. We report here on a case of primary adrenal insufficiency that was secondary to primary bilateral adrenal lymphoma. A 54-year old man was hospitalized because of easy fatigability, weight loss and consistent malaise for 6 months. The physical examination revealed hyperpigmentation on the anterior chest and hypotension. According these findings and symptoms, we did a rapid ACTH stimulation test with a clinical suspicion of adrenal insufficiency. He showed an inadequate adrenal response and so he was diagnosed with adrenal insufficiency. The abdominal CT images showed bilateral huge adrenal masses and increased uptake of the adrenal glands on PET. The pathologic diagnosis by ultrasound-guided gun biopsy of the right adrenal gland was diffuse large B cell lymphoma. The patient was administered combination chemotherapy with the R-CHOP regimen, and after 8-cycles of chemotherapy, he achieved complete remission of tumor according to the image studies and he recovered his adrenal function. Primary adrenal lymphoma, although a rare disease, should be considered in patients with bilateral enlargement of the adrenal glands and when the adrenal glands show increased uptake on a PET scan, and especially there is adrenal insufficiency.

Keyword

Adrenal insufficiency; Non-Hodgkin's lymphoma

MeSH Terms

Addison Disease
Adrenal Glands
Adrenal Insufficiency
Adrenocorticotropic Hormone
Biopsy
Drug Therapy, Combination
Humans
Hyperpigmentation
Hypotension
Lymphoma
Lymphoma, B-Cell
Lymphoma, Non-Hodgkin
Physical Examination
Positron-Emission Tomography
Prognosis
Rare Diseases
Thorax
Weight Loss
Adrenocorticotropic Hormone

Figure

• 

  Fig. 1 Appearance of the patient: Hyperpigmentation on skin (A) face and the upper chest (B) back. Arrows: hyperpigmented spots.

• 

  Fig. 2 Abdomen CT scan at diagnosis showed about 6.4 cm sized adrenal gland masses.

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  Fig. 3 FDG-PET scan at diagnosis showed huge bilateral adrenal masses with intense FDG uptake. Right adrenal mass invaded into the right hepatic lobe.

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  Fig. 4 High-power view of the adrenal gland tumor (both, × 200). A. The gun biopsied specimen showed diffuse proliferation of large atypical cells, which had folded nuclei with one or multiple prominent nucleoli (H&E staining). B. The tumor cells were positive to CD20 immunohistochemical staining.

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  Fig. 5 Imaging studies after 8 cycle R-CHOP. A. Abdominal CT scan. B. FDG-PET scan.

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