| خلاصه مقاله | Background: Primary adrenal lymphoma (PAL) usually presents as a bilateral heterogeneous adrenal mass on computed tomography (CT). PAL is a rare type of lymphoma originating from adrenal tissue and causes adrenal insufficiency (AI). We present a case of PAL with the complaint of loss of appetite and signs of AI.
Case presentation: A 75-year-old male with symptoms of weight loss (approximately 10 kg ), nausea and vomiting over 4 weeks was referred to the endocrin clinic with the possibility of AI. On physical exam, the patient had slightly dark skin and had hypotension (BP: 90/55). The lab tests revealed hypekalemia (K+: 5.1 mEq/L), near-normal sodium levels (Na+: 135 mEq/L), and high ferritin levels (ferritin: 791 ng/dL, normal range: 17-327) high LDH levels( 420 U/L ,NL: 135-225 U/L)
The hormonal evaluation of patients confirmed AI due to low cortisol (Cortisol: 3µg/dL, normal level: 5-25 µg/dL) and high ACTH (ACTH: 2135 pg/dL, normal range: 4.7-48.8 pg/dL). In compound tomography (Figure 1), the patient had bilateral adrenal masses of 58*54mm and 58*48mm on the right and left adrenal , with no lymphadenopathy. To evaluate the nature of the mass, an adrenal biopsy was performed, which reported a malignant neoplasm. The IHC study was positive for CD45 with a Ki-65 level of approximately 80%. The IHC study was in favor of high-grade lymphoma. The patient was given 0.1 mg of fludrocortisone and 20 mg of hydrocortisone daily for AI and then was referred to the oncology ward for chemotherapy and, unfortunately, died before the start of chemotherapy.
Conclusion: Adrenal lymphoma can be divided into two categories: primary and secondary adrenal lymphoma. Secondary adrenal lymphoma is the most prevalent type of adrenal lymphoma. PAL is usually seen in the elderly male population and causes a bilateral adrenal mass. PAL causes AI due to the cytokines released from immune cells, rather than the mass effect. The exact pathophysiology of PAL is unknown, and the most common reported subtype of PAL is DLBCL lymphoma, but other subtypes, such as peripheral T-cell lymphoma and Burkitt`s lymphoma, are also reported. The prognosis of PAL is poor and is associated with high mortality rates. Currently, there are no specific biomarkers for PAL, but studies suggest high LDH and ferritin levels in PAL. Adrenal biopsy is needed to confirm the diagnosis of PAL and determine the exact subtype of PAL. Some of the important markers for PAL are CD45, Ki-67 level, CD10, CD20, MUM1, BCL2, and BCL6. The preferred treatment for PAL is the R-CHOP chemotherapy regimen with a 2-year overall survival rate of 57%. |