Endocrine Abstracts

Background: Primary adrenal lymphoma (PAL) is a rare cause of primary adrenal insufficiency (PAI). Most often patients present with unspecific symptoms. Bilateral adrenal enlargement with signs and symptoms of PAI are clues, percutaneous biopsy after having excluded pheochromocytoma in a situation of high suspicion is diagnostic. Most of PAL are highly malignant B-cell lymphomas with a bad prognosis.

Case: A 71-year-old patient was sent for endocrine workup because of a 2-month history of intermittent dyspnea, thoracic discomfort, weight loss and abdominal pain. An ambulant CT scan to exclude pulmonary embolism showed bilateral adrenal enlargement (right 77×31×55 mm, left 63×38×41 mm, native 25-29HE), which were new compared to a CT scan done one year earlier. On clinical examination, the patient was orthostatic and in an im-paired general condition. He had hyperpigmented hand lines. Laboratory evaluation showed a slight hyponatremia (131 mmol/), potassium in the upper normal range (4.2 mmol/l), pathologic ACTH-stimulation test (peak cortisol level: 153 nmol/l) and elevated ACTH levels (231 ng/l, normal range <46 ng/l). Antibodies for 21-hydroxylase were negative, as well as free metanephrines in plasma, aldosterone-renin ratio was decreased (aldosterone 122 pmol/l, renin 39 mU/l). 17-hydroxyprogesterone was low (3.3 nmol/l, normal range 1.9–6.5 nmol/l). Quantiferon test was negative, and the CT scan did not raise suspicion for tuberculosis. We diagnosed PAI and started substitution with hydrocortisone (initial dose 50 mg/d) and fludrocortisone (0.1 mg/d). Histology of a CT-guided needle biopsy revealed infiltration of a highly malignant B-cell lymphoma. The patient was sent for oncological evaluation and start of chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP).

Discussion: PAL is a rare manifestation of primary extranodal lymphomas (1/3 of all lymph node neoplasms, PAL<1% of all extranodal lymphomas). Only about 100 cases are published in the literature worldwide. Conversely, secondary spreading of a lymphoma to the adrenals is quite common (in autopsy studies up to 25%). Other reasons for bilateral adrenal enlargement are adrenal hyperplasia (any cause), metastasis of lung, breast and stomach (>50% of metastasis), bilateral pheochromocytoma, adrenal hemorrhage, adrenal involvement with granulomatous diseases, histicytosis and primary pigmented nodular adrenal dysplasia (PPNAD). Symptoms of PAL are unspecific (asthenia, weight loss, vague abdominal pain, fever). Diagnosis is made by percutaneous biopsy. In nearly 70% of cases PAL are bilateral, causing primary adrenal insufficiency. Average age of affected patients is around 70 years. Most of PAL are diffuse large B-cell lymphomas with BCL6 gene rearrangement and poor prognosis, as in our case. Therapy consists of R-CHOP.

Conclusion: Patients with bilateral adrenal enlargement and PAI need immediate replacement of glucocorticoid (including instructions about dosing in stressful situations) and mineralocorticoid hormones. While PAL is rare, it has a poor prognosis, thus rapid induction of treatment is necessary.