Endocrine Abstracts

Introduction: Primary adrenal lymphoma is a rare cause of adrenal insufficiency, with most cases being of B-cell lymphoma.

Case report: We present the case of a 51 yo man with a 1.5-month history of recurrent fever, nausea, vomiting, anorexia, weight loss of 12 kg, fatigue, polyarthalgia, hypotensive episodes (70/40 mmHg) and singultus. Before admission in our clinic he had a thoracic CT scan, which showed a 5.5 cm right adrenal mass and hyperplasia of the left adrenal, followed by plasmatic measurement of cortisol and ACTH, consistent with primary adrenal insufficiency: plasmatic cortisol=22.6 nmol/l (172-497), ACTH=729.5 pg/ml (7.2-63.3). Consequently, 3 days prior to admission in our clinic, his GP recommended daily 4 mg Dexamethasone iv treatment, with significant improvement of symptoms. On physical examination he had hyperpigmentation of the palmar creases, buccal mucosa and gums. Further work-up revealed mild leukocytosis [11.14 *103/μl (4.9-10.2)] with lymphocytosis [4.12 *103/μl (0.6-3.4)], elevated inflammatory markers [ESr=71 mm/h (0-15), CRP=19.9 mg/dl (<10)], mild hypercalcemia [10.6 mg/dl (8.4-10.2)] with low-normal PTH [15.8 pg/ml (15-65)]. An infectious cause of the fever was excluded by performing cardiac ultrasound, urine culture, nasal, rectal, inguinal swabs, respiratory pathogens panel test, blood cultures. Abdomen CT scan revealed an inhomogenous right adrenal gland of 7/3.3/4.7 cm and a left adrenal adenoma of 1.5/1 cm. Screening for primary hyperaldosteronism and pheochromocytoma was negative. We started glucocorticoid and mineralocorticoid replacement therapy and referred the patient to the Surgery department where tumor debulking was performed. Histopathological report described undifferentiated malignant tumor and immunohistochemistry analysis was consistent with anaplastic large cell lymphoma, ALK-negative, an aggressive neoplasm of T-cell lineage.

Conclusions: Primary adrenal T-cell lymphoma is an extremely rare cause of adrenal insufficiency. Our patient is currently receiving glucocorticoid and mineralocorticoid replacement therapy and combination chemotherapy (epirubicin + etoposide).