Case Presentation:
A 68 year-old female known to have hypertension, nephrolithiasis, and chronic urinary incontinence presented to a tertiary-care hospital with three days of left sided flank pain, unilateral headache, diaphoresis, and a recent history of unexpected weight loss. On exam, she was found to have a blood pressure of 203/88 mmHg, with benign cardiopulmonary and neurological findings. A CT scan on admission revealed large bilateral retroperitoneal masses replacing the adrenal glands. Given her constellation of symptoms, a broad workup ensued, including evaluations for pheochromocytoma and other functional malignancies. Initial serologies and tumor markers for endocrine, gynecological, and gastrointestinal malignancies returned negative. CT scan of the chest revealed no primary malignancy. Pheochromocytoma was ruled out with normal metanephrines. At this point, the decision was made to pursue an adrenal biopsy, which returned positive for diffuse large B-cell lymphoma. Immunochemistry revealed CD20, CD45, BCL-2, BCL-6, Vimentin and MUM1 positivity, with a Ki-67 (proliferation index) of greater than 90%. Bone marrow biopsy showed no evidence of lymphoma involvement. Due to the severity of her symptoms, largely the persistent flank pain, chemotherapy was started. The patient completed a five-day cytoreductive induction with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) and intrathecal methotrexate. After three cycles of R-CHOP, repeat CT of the chest, abdomen, and pelvis revealed marked improvement in disease, with near complete resolution of adrenal masses. After six cycles, a staging PET scan revealed no evidence of active disease. Repeat scans at six months and one year resulted similarly.
Discussion:
Primary adrenal lymphomas (PAL) are among the rarest of oncological entities, with fewer than 200 cases reported in the English literature. Limited published cases, and increased heterogeneity within those cases, has led to difficulty in the identification and treatment of this disease. Review of the literature suggests that patients with PAL most often present with weight loss, lumbar pain, and signs or symptoms related to adrenal insufficiency. Generally a B-cell lymphoma, as seen in this case, PAL is an aggressive disease with a poor five-year disease free prognosis. There is no defined treatment protocol for PAL, and patients have largely been treated with common B-cell lymphoma regimens such as R-CHOP. In contrast to published reports of poor response to conventional-dose chemotherapy alone, and high mortality rates, our patient demonstrated that standard treatment protocols may achieve remission in cases of localized PAL.
Conclusions:
It is important for clinicians to consider PAL in patients presenting with bilateral adrenal masses. Our case represents yet another unique presentation of PAL, but with an unusually positive outcome.