Massive bilateral adrenal mass with adrenal insufficiency: a case report of primary adrenal lymphoma

Abstract

Primary Adrenal Lymphoma (PAL) is extremely rare and usually occurs in men in the 6th-7th decade as bilateral, diffuse large B-cell lymphoma (DLBCL). Here, an 80-year-old patient admitted to the urology outpatient clinic with flank pain and bilateral adrenal mass detected on ultrasound is presented. Positron Emission Tomography-Computed Tomography (PET-CT) was planned for the patient who was referred to the endocrine outpatient clinic. The PET-CT scan revealed lobulated-contoured masses containing necrotic areas with a size of 7.4×5.5×9.8 cm, 19 Hounsfield Unit (HU), and SUVmax value of 23.9 the right adrenal, and with the size of 8.4×8.7×10.8 cm, 28 HU, SUVmax value of 27.3 in the left adrenal. These masses were reported to be not compatible with metastasis and suggested a tumor of the adrenal origin or bilateral adrenocortical carcinoma. In laboratory tests, since Adrenocorticotropic hormone level 291-592 pg/mL (high) and Cortisol level was 7.5-9.5 mcg/dL (low), bilateral adrenalectomy was performed considering adrenocortical cancer primarily. Diffuse large B cell lymphoma was determined as the result of the pathology. Postoperative hydrocortisone and fludrocortisone treatment was initiated for the patient immediately. The patient was transferred to the hematology inpatient clinic to receive Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone chemotherapy. In this case report, we aimed to emphasize that the differential diagnosis should be made well in massive bilateral adrenal masses. Although the diagnosis is challenging, clinicians should be alert in diagnosing lymphoma in bilateral, massive adrenal masses with adrenal insufficiency. Since this disease's prognosis is poor and aggressive, a histopathological diagnosis should be obtained, and treatment should be initiated as soon as possible.

Keywords

• Adrenal insufficiency
• Primer Adrenal Lymphoma
• Non-Hodkgin's Lymphoma

References

1. 1. Wang J, Sun NC, Renslo R, Chuang CC, Tabbarah HJ, Barajas L, et al. Clinically silent primary adrenal lymphoma: a case report and review of the literature. Am J Hematol 1998;58:130-6.
2. 2. Rashidi A, Fisher SI. Primary adrenal lymphoma: a systematic review. Ann Hematol 2013;92:1583-93.
3. 3. Bourdeau I, El Ghorayeb N, Gagnon N, Lacroix A. Management of endocrine disease: differential diagnosis, investigation, andtherapy of bilateral adrenal incidentalomas. Eur J Endocrinol 2018;179:R57-R67.
4. 4. Levy NT, Young WF, Habermann TM, Stricker JG, Carney JA, Stanson AW. Adrenal insufficiency as a manifestation of disseminated non-Hodgkin's lymphoma. Mayo Clin Proc 1997;72:818-22.
5. 5. Rosenthal FD, Davies MK, Burden AC. Malignant disease presenting as Addison’s disease. Br Med J 1978;1:1591-2.
6. 6. Turkey Endocrinology and Metabolism Diseases Society of Adrenal and Gonadal Guide 2019:p.67.
7. 7. Anis M, Irshad A. Imaging of abdominal lymphoma. Radiol Clin North Am 2008;46:265-85.
8. 8. Allolio B, Fassnacht M. Clinical review: an adrenocortical carcinoma-clinical update. J Clin Endocrinol Metab 2006;91:2027-37.

9. 9. Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol 2016;175: G1-G34.
10. 10. Grigg AP, Connors JM. Primary adrenal lymphoma. Clin Lymphoma 2003;4:154-60.