Xanthogranulomatous adrenalitis masquerading as a functioning adrenocortical malignancy: a case report

Abstract

Introduction: Xanthogranulomatous adrenalitis (XA) is a rare chronic inflammatory disease of the adrenal glands and resembles adrenal neoplasm in clinical and radiologic characteristics. There is no report on XA presenting as a functioning adrenal mass in the literature. We present a case of XA mimicking a functioning adrenocortical carcinoma. Case report: A 52-year-old man presented with right flank pain, fever, vomiting, and loss of appetite for 2 weeks. He had signs of dehydration and elevated blood glucose level. Ultrasonography revealed a right adrenal mass. Contrast-enhanced computed tomography showed lobulated and necrotic mass replacing the right suprarenal gland and encasing the right renal vein, adjacent inferior vena cava, psoas, and diaphragm. There was loss of fat planes with liver and upper pole of the right kidney. Biochemical evaluation indicated increased serum and urine cortisol levels. As a sequel to hypercortisolism-induced hyperglycemia, he developed spontaneous chest wall abscess and bilateral sudden vision loss due to vitreous hemorrhages. Pus and blood culture grew methicillin-sensitive Staphylococcus aureus. Adrenal suppressant ketoconazole was administered for better glycemic control. With a diagnosis of locally advanced adrenocortical malignancy, right radical adreno-nephrectomy was performed, and cut section revealed a pus collection of around 100 mL. Histopathology examination showed xanthogranulomatous inflammation involving adrenal gland, Gerota’s fascia, psoas, and lymph nodes. Postoperatively, the patient recovered satisfactorily with favorable glycemic control. Conclusion: XA can mimic adrenal neoplasms both clinically and radiologically and is associated with staphylococcal infection. It warrants surgical excision and culture-based antibiotics and is mostly diagnosed on postoperative histopathology.

Xanthogranulomatous Adrenalitis: A Case Report and Literature Review

“…Whether the parenchymal portion of XGA has a secretory function or it induces abnormal secretion in normal adrenal tissue, is unknown. A recent case reported that the preoperative basal serum cortisol and 24-hour urinary cortisol of a male patient were elevated with a positive result of dexamethasone suppression test and uncontrollable hyperglycemia (1). Hormone levels dropped to the normal range after adrenalectomy.…”

“…It has been reported that XGA often occurs in patients with an underlying condition, such as infection or hemorrhage. Some cases have evidence of bacterial infection, namely, methicillin-resistant Staphylococcus aureus (MRSA) and methicillin-sensitive S. aureus (MSSA) (1,2), which are rare in xanthogranulomatous pyelonephritis. Uniquely, Jayanth et al reported a case masquerading as a functioning adrenocortical malignancy with elevated cortisol levels (1).…”

“…Some cases have evidence of bacterial infection, namely, methicillin-resistant Staphylococcus aureus (MRSA) and methicillin-sensitive S. aureus (MSSA) (1,2), which are rare in xanthogranulomatous pyelonephritis. Uniquely, Jayanth et al reported a case masquerading as a functioning adrenocortical malignancy with elevated cortisol levels (1). To the best of our knowledge, this is the first reported case of XGA associated with superficial infection and low renin levels which could resemble a functional adrenal neoplasm.…”