Ectopic ACTH syndrome complicated by multiple opportunistic infections treated with percutaneous ablation of the adrenal glands

Abstract

Ectopic adrenocorticotropic hormone (ACTH)-related Cushing's syndrome can lead to multiple complications including severe immunosuppression. If the ACTH-secreting tumour cannot be found, definitive treatment is surgical adrenalectomy, typically followed by glucocorticoid replacement. Here, we present a case of fulminant respiratory failure secondary to coinfection with and cytomegalovirus in a patient with ectopic ACTH-dependent Cushing's syndrome with occult primary. Due to significant deconditioning, she was unable to undergo definitive adrenalectomy and instead underwent percutaneous microwave ablation of the adrenal glands.

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

“…These are particularly frequent during intense hypercortisolim of any etiology (13,96,97,113,116,117,118,119). The definition of intense hypercortisolism is debated.…”

“…Cushing's syndrome associated infections have four main characteristics: (1.) These infections include all kinds of opportunistic infections due to bacteria and related organisms as viruses and fungi, including Pneumocystis carinii or jirovecii (115,116,117,118,119). All organs may be affected (skin, hollow organs and brain), but the lungs are the site of the most frequent and severe infections (116); (2.)…”

“…All organs may be affected (skin, hollow organs and brain), but the lungs are the site of the most frequent and severe infections (116); (2.) the classical symptoms of infection such as fever and increased levels of C-Reactive Protein may be lacking (115,116,117,118,119); (3.) infections will often result in delay of invasive diagnostic procedures such as inferior petrosal sinus sampling (IPSS) and any surgery such as excision of the ACTH-secreting tumor or bilateral adrenalectomy (BLA); (4.)…”

“…their occurrence may be favored by diabetes mellitus and also by antineoplastic chemotherapy that should be ideally deferred until hypercortisolism is controlled and (5.) Pneumocystis jirovecii pneumonia can emerge rapidly once hypercortisolism is brought under control (115,116,117,118,119). Prophylaxis of Pneumocystis jirovecii pneumonia with sulfamethoxazole-trimethoprim is recommended for all patients with intense hypercortisolism (120).…”

“…Although poorly studied in EAS, osteoporosis resulting from altered bone quality appears to be more frequent than in florid Cushing's Syndrome (95,96). Fractures can affect the entire skeleton, with a high risk of vertebral fractures that will result in pain and the patient being bedridden (118,119,120,121). Aseptic necrosis of the femoral head is a painful complication that reduces mobility (127) and again confines the patient to bed.…”