Endocrine Abstracts

Case History: A 73-year-old gentleman with no prior comorbidities, presented with 2-month history of decreased appetite, weight loss and lethargy. He denied experiencing paroxysmal symptoms of headache/sweating/palpitations, abdominal pain, fever, night sweats or chronic cough. Clinically, he was cachexic, dehydrated (blood pressure 98/82 mmHg; heart rate 125 beats per minute) with a vague mass palpable over the right lumbar. There were no palpable lymphadenopathy or features suggestive of Cushing’s syndrome.

Investigations: Biochemical analysis revealed non-parathyroid hormone (PTH) dependent severe hypercalcemia [corrected calcium 3.67 mmol/l (N 2.2-2.6); phosphate 1.13 mmol/l (N 0.78-1.65); PTH 0.1 pmol/l (N 1.4-6.8)] with acute kidney injury [creatinine 157 umol/l (N 54-97); urea 16.3 mmol/l (N 3.2-8.2)], elevated lactate dehydrogenase 941 U/l (N 120-246) and anaemia [haemoglobin 100 g/l (N 130-170); white blood cell 10 × 10⁹/l (N 4-10); platelet 417 × 10⁹/l (N 150-400); iron 3.5 umol/l (N 11.6-31.3); transferrin saturation 10% (N 20-45)]. A random serum cortisol was appropriately elevated for his ill condition, at 1169 nmol/l (N 145-619). 24hour urine metanephrines and 24hour urine cortisol were within normal limits. CT imaging showed bilateral adrenal lesions with heterogenous enhancement. The right lesion (12.4 × 9.0 × 12.6 cm) encased the right renal artery and vein with infiltration of the right kidney while the left lesion measured 4.0 × 4.2 × 4.3 cm.

Results/Treatment: In addition to saline diuresis, he received subcutaneous (S/C) calcitonin 200 IU TDS for 3 days and a dose of S/C Denosumab 60 mg. He showed marked improvement clinically and biochemically (Table 1). CT-guided adrenal biopsy was performed and histopathology of the biopsy established the diagnosis of diffuse large B-cell lymphoma (DLBCL) with high Ki-67 (>90%). Staging done via FDG PET-CT showed extensive disease involving both supra and infra-diaphragmatic lymph nodes, spleen, lung and brain. Treatment with intravenous dexamethasone and pre-phase cyclophosphamide was initiated. Unfortunately, he succumbed after developing sepsis post-treatment.

Conclusion/Discussion Points: This case highlights primary adrenal lymphoma (PAL) as a rare but lethal cause of bilateral adrenal lesions. PAL accounts for <1% of non-Hodgkin lymphomas and CT-guided adrenal biopsy is the gold standard to establish the diagnosis. R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone) chemotherapy regime remains the mainstay of treatment, though the long-term prognosis remains guarded with reported median survival time of 14 months (based on a study of 136 patients with PAL). Besides hypercalcemia, a more prevalent endocrine complication in these patients is adrenal insufficiency, which requires prompt intervention if present.