Case Presentation: 54-year old man with a history of hypertension and hyperlipidemia presented with a month of periumbilical pain that progressed to involve his bilateral flanks, as well as cough productive of yellow sputum and a 20-pound weight loss. He immigrated from Honduras 18 years prior to admission.On admission, he was afebrile and hemodynamically stable. Physical exam was remarkable for diffuse abdominal tenderness without rebound tenderness or guarding. The rest of the exam was non-contributory. Initial labs were within normal limits. CT scan of the abdomen was suspicious for bilateral adrenal hemorrhages. MRI identified bilateral adrenal masses with the right adrenal mass measuring 8 x 18 x 17 cm without any signs of hemorrhage. Interferon gamma release assay was positive and CT scan of the chest showed bilateral pleural effusions. Active pulmonary tuberculosis (TB) was ruled out via bronchoalveolar lavage. Biopsy was postponed until pheochromocytoma was ruled out with normal plasma metanephrines. His course was further complicated by lower extremity edema due to extensive bilateral DVTs requiring anticoagulation. Biopsy of the right adrenal mass suggested myeloid sarcoma, however the sample was insufficient. He was discharged to follow up in clinic, but returned to the hospital with dyspnea due to pleural effusions and was ultimately found to have plasmablastic lymphoma (PBL) via pleural biopsy.
Discussion: The differential diagnosis of bilateral adrenal lesions is vast and includes hemorrhage, pheochromocytoma, lymphoma, metastatic disease, TB, fungal infections and others. These have the potential to precipitate adrenal insufficiency leading to a shock state; therefore they must be handled with urgency.
PBL refers to a subtype of Non-Hodgkins Lymphoma (NHL) that is thought to arise from a plasmablast: an activated B-cell that has undergone somatic hypermutation and class switching. These cell lines commonly lack specific B-cell markers, such as CD20. The pathogenesis of PBL has yet to be fully elucidated; most of our information has been obtained from studying AIDS patients. Nevertheless, transplant and immunocompetent patients are also at risk. Experts suspect a relationship with MYC rearrangements, EBV and CD45.
PBL is known to be aggressive and relapsing. It is typically diagnosed at Ann Arbor Stage IV, with the oral/nasal cavity and GI tract as the most commonly affected extranodal sites. It has a median survival of eight months, as compared to diffuse large B-cell lymphoma, which carries a median survival of 70 months. An intense treatment regimen, such as EPOCH, is necessary as standard CHOP therapy is considered insufficient.
Conclusions: PBL is an aggressive and rare form of NHL and it is even rarer for it to present with bilateral adrenal masses. Little is known about the pathogenesis of PBL and scarce case reports exist of this poorly understood disease. However, when suspected, diagnosis should be pursued and treatment started promptly given its grave prognosis.