Endocrine Abstracts

Most differential diagnoses of unilateral adrenal lesions include non-functional adenoma, adrenocortical carcinoma or pheochromocytoma. Primary adrenal lymphoma(PAL) is an extremely uncommon type of primary extranodal non-Hodgkin’s lymphoma(<1%). Most cases are bilateral (~75%), being unilateral PAL scarcely reported. The apparent unilateral involvement of this entity at presentation, in the CT scan/MRI may difficult the diagnosis, delaying the start of chemotherapy. We report a case of a bilateral PAL interpreted primarily as a unilateral suspicious adrenal lesion. A 68-year-old(yo) male patient presented to the emergency department with persistent fatigue for 3 months getting worse in the last 3 weeks. His past medical history included type 2 diabetes with macrovascular disease, prostate cancer at 61 yo and active smoking. Physical examination was unremarkable, except for a mild edema in the lower limbs. Laboratory investigations revealed: hemoglobin 13.1g/dL, white blood cell count 4×109/L, D-dimer 2232 ng/ml(<500), LDH 488U/L(85-227), NT-proBNP 636pg/mL(<125). A computed tomography(CT) angiogram of the chest was performed to exclude pulmonary embolism(PE)/COVID-19 infection. A lesion of 139 mm on the left adrenal gland, suggestive of a mass or hemorrhage was observed. No signs of PE were evident. The patient was hospitalized for further investigation. Hormonal evaluation revealed: ACTH 37.8 pg/mL(<46), serum cortisol 20 mg/dL, 24-hour urine cortisol levels were slightly increased with 437 mg/24h(28-213), aldosterone and total urinary metanephrines were normal, except for a slightly increased normetanephrine of 545 mg/24h(<444). An abdominal MRI confirmed a large neoformative lesion on the left adrenal gland of 140 mm. The patient underwent left adrenalectomy and nephrectomy. Histology revealed a diffuse large B-cell lymphoma, non-germinal centre B-cell. Staging 18F-FDG PET/CT scan showed intense 18F-FDG 14 mm uptake in the right adrenal gland(SUVmax 5.7). He received 6 cycles of R-CHOP(rituximab-cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy. The follow-up 18F-FDG PET/CT scan performed at the end of the treatment revealed no evidence of tumor. Thus, this case illustrates the difficulties found in the diagnosis of PAL and draws attention to consider it as a differential diagnosis during evaluation of adrenal masses. In this case, an adrenalectomy was performed given the presence of unilateral large adrenal mass and the possibility of an adrenal carcinoma, leading to a delay in treatment initiation. Furthermore the absence of adrenal insufficiency commonly associated with bilateral adrenal lymphomas was absent. It is noteworthy that the staging 18F-FDG PET/CT scan revealed uptake in the other non-operated adrenal gland that was not evident after chemotherapy, making obvious the diagnosis of a bilateral PAL.