Spontaneous tumour lysis syndrome in a primary adrenal lymphoma

A 56-year-old man presented with dyspnoea, abdominal pain and 10 kg weight loss. He had a history of diabetes mellitus (treated with metformin), obesity, sleep apnoea and bilateral femoral stenting. Physical examination showed hepatomegaly. His chest X-ray showed a right pleural effusion, and a computed tomography (CT) scan revealed bilateral adrenal gland tumours and mediastinal lymphadenopathy (left). An 18F-fluorodeoxyglucose positron emission tomography (PET)/CT scan demonstrated the hypermetabolic nature of both the adrenal masses and the mediastinal lymph nodes (right). Laboratory investigations indicated an inflammatory syndrome with a white blood cell count of 11 × 109/l, C-reactive protein (CRP) 66 mg/l, lactate dehydrogenase (LDH) 754 iu/l (normal <350), alkaline phosphatase 154 iu/l (normal <116) and gamma-glutamyltransferase 157 iu/l (normal <73). A pulmonary neoplasm was suspected and he underwent an endobronchial ultrasound examination and a thoracentesis, but the cytology of these was negative for malignant cells. He was then transferred to critical care for acute adrenal insufficiency. A second pleural tap was performed, finding 4·5 × 109 lymphocytes/l, with 70% lymphomatous T cells (CD3+, CD7+, CD8+, and CD2−, CD4−, CD5−). Urinary metanephrines and 123I-meta-iodobenzylguanidine (MIBG) scintigraphy ruled out suspected phaeochromocytoma. An ultrasound-guided biopsy of the right hypoechogenic and heterogeneous adrenal mass led to the diagnosis of a peripheral T-cell lymphoma, not otherwise specified. The initial laboratory abnormalities were, in fact, related to a spontaneous tumour lysis syndrome (TLS). Our patient received corticosteroid therapy, worsening the TLS. Despite intensive medication and renal replacement therapy, he suffered refractory hyperkalaemia and died from multiple organ failure after cardiac arrest and attempted resuscitation. Primary adrenal lymphomas are very rare, with fewer than 200 cases in the literature. Various subtypes have been described, involving B or T cells. Patients are usually symptomatic with B signs and non-specific abdominal symptoms (Laurent et al, 2017). β2 microglobulin, LDH and CRP can be elevated. CT scan shows tumours of various sizes, which are bilateral in 71%. They are hypermetabolic on PET scanning with extra-adrenal disease also present in 70% of patients. Magnetic resonance imaging is hypointense on T1 and hyperintense on T2 scanning. As with other high-grade non-Hodgkin lymphomas, TLS is typically associated with bulky and proliferating tumours. They can respond well to combination chemotherapy, but often relapse and are associated with a relatively poor prognosis (survival of 61·9% at 2 years).