Endocrine Abstracts

Introduction

Primary adrenal lymphoma (PAL) are rare extranodal lymphomas and account for<1% of all non-Hodgkin lymphomas, with characteristic clinical features including high incidence of bilateral involvement, predominantly diffuse large B-cell histology, and low incidence of extra-adrenal disease at diagnosis. We report a case of 50-year-old patient affected by bilateral B-cell adrenal lymphoma.

Case presentation

A 50-year-old woman was admitted for exploration of suspicious bilateral adrenal incidentalomas. History of the disease dates back to February 2020, marked by the onset of recurrent urinary tract infection. Abdominal ultrasound showed two right and left adrenal masses measuring respectively 9 and 10 cm. There were no hyperpigmentation, no touchable lymph nodes, no abdominal mass, untouchable liver and spleen and no breast nodule at physical examination. Computed Tomography (CT) showed oval hypodense right and left adrenal masses, with limited contours measuring respectively 66 * 90 mm and 100 * 81 mm, with average spontaneous density of 43UH and 42UH, and absolute washout at 33% and 27%, slightly enhanced after injection of contrast product, heterogeneous with necrosis and without calcifications. Biology revealed microcytic hypochromic anemia with normal ferritinemia, elevated levels of erythrocyte sedimentation rate at 80 mm (reference: 25 mm/h), C-reactive-protein (CRP) at 51 mg/l (reference<6) and lactate dehydrogenase (LDH) at 400 UI/l (0–247). Morning plasma cortisol was low at 133 nmol/l (185–624 nmol/l), Adrenocorticotropin (ACTH) was increased at 470 pg/ml (reference<46 pg/ml). The patient was treated with hydrocortisone. Serum catecholamines, plasma renin activity, aldosterone, testosterone and dehydroepiandrosteone sulfate (DHEA-S) were normal. β₂-microglobulin was slightly elevated at 3.51 mg/l (1.42–3.21 mg/l). Serum tumor markers were normal. Thoraco-abdomino-pelvic CT showed right thyroid nodule with no other lesions especially no lymphadenopathy. Biopsy of left adrenal mass revealed a diffuse lymphomatous proliferation partially necrotic. Cells are large with a rounded or ovoid nucleus strongly nucleolated. The patient presented an acute adrenal decompensation related to macrophage activation syndrome. Broad-spectrum antibiotics and dexamethasone were administrated with a good clinical course. After chemotherapy, the patient presented a tumor lysis syndrome with septic shock and acute respiratory distress syndrome and she died.

Conclusion

PAL is rare and prognosis is generally poor. More studies are needed in the future to identify the predictors of good clinical and radiological response to therapy.