Endocrine Abstracts

Large adrenal masses are strongly suggestive of malignancy. Nevertheless, bilateral adrenal masses are less common (around 20% of incidental adrenal lesions). Metastases and lymphoma are more frequent (especially when aging), and not so easy to differentiate on CT or MRI. Simple biological markers, such as plasma LDH or beta2microglobulin, could help.

Case report: A 69-year-old patient was admitted for abdominal pain with progressive lethargy. Abdominal CT revealed heterogeneous necrotic bilateral adrenal lesions, measuring over 70 mm, invading the left diaphragmatic pillar and the gastric wall. Spontaneous density was above 10HU and wash out negative. Adrenal insufficiency was confirmed by low 0800 h cortisol and increased ACTH. After ruling out pheochromocytoma, oncologists decided of adrenal biopsy before receiving increased levels of β2microglobulin and LDH. Adrenal biopsy diagnosed non centrogerminative diffuse large B cell lymphoma. In situ hybridization analyses showed a BCL6 rearrangement without BCL2 or MYC rearrangements. An 18FDG PET-CT revealed splenic invasion and multiple lymphadenopathies, which would have been easily accessible to biopsy. Further evaluation revealed marrow infiltration on bone marrow biopsy and a high risk of central nervous system invasion. Overall the age adjusted IPI score was 3 and CNS-IPI score was 6 (high risk). These conclusions led to 8 cycles of R-CHOP21 immuno-chemotherapy plus two cycles of high dose methotrexate regimen in conjunction with intrathecal methotrexate. Treatment was well tolerated and lead to complete remission on PET-CT, CT scan and bone marrow biopsy, still lasting 2 years after the end of treatment.

Discussion and conclusion: When facing bilateral adrenal incidentaloma, lymphoma must be evoked more commonly than with unilateral masses. Adrenal imaging (either CT, PET-CT, or MRI) or existence of adrenal insufficiency, have a limited role in guiding to etiology. Biopsy of adrenal mass is an exposed action that may lead to complications (hemorrhage, sepsis, tumor dissemination) or unhelpful conclusion due to necrosis. Therefore, simple markers such as LDH (and possibly other markers including beta2microglobulin) should be measured to suggest lymphoma (increased in over 70% of cases), and further lead to a screen (by CT and PET-CT if not obvious clinically) for associated lymphadenopathies which are much easier to biopsy than the adrenal gland or for lesions with a higher standardized uptake value which are more commonly conclusive for the pathological diagnosis of lymphoma.