Endocrine Abstracts

A 68 year old man presented with shortness of breath and was found to have a pulmonary embolus. During work up of the underlying aetiology he was noted to have bilateral adrenal masses. He was subsequently readmitted with pyrexia and feeling generally unwell. Imaging revealed the progression of adrenal masses and there was biochemical evidence of adrenal insufficiency with a random cortisol at the low end of normal. CT guided biopsy was consistent with the diagnosis of a high grade B cell lymphoma and after completing staging with bone marrow biopsy and PET scan R CHOP chemotherapy was instituted.

Primary adrenal non Hodgkin lymphoma is rare with fewer than 200 reported cases. It is more prevalent in men and has a peak incidence in the mid-1960s. Diffuse large B cell is the most common accounting for approximately 78% of reported cases. Up to 70% are bilateral at presentation. There is some association with EBV and mutations in p53 and c-kit oncogenes.

Symptoms of isolated adrenal lymphoma are variable and often non-specific. Factors associated with poor prognosis include presentation with adrenal insufficiency, large tumour size and an elevated LDH. The addition of Rituximab to the CHOP regimen has been associated with better outcomes. Adrenal lymphoma is also an indication for prophylactic intrathecal chemotherapy which has been used in this case for CNS prophylaxis. There has been a good response to therapy. The end of treatment PET scan showed residual uptake which is not thought to be clinically significant however this will continue to be monitored.