Surgical treatment of ectopic adrenocorticotropic hormone syndrome with intra-thoracic tumor

Abstract

The thoracic cavity should be a focus in routine examinations of patients with symptoms of Cushing's syndrome (CS), because ectopic ACTH-producing tumors are commonly found in bronchus/lung and mediastinum. Despite the incidence of the pulmonary nodule secondary to opportunistic infection in some cases, surgery is still the first choice if the tumor is localized. The surgical procedure should be performed according to the principles in resection of lung cancer and mediastinal tumor. The surgical efficacy is significant for short-term periods; however, the recurrence of the disease in long-term periods is in great part related to distal metastasis or relapse of the tumor.

Cushing�s syndrome, main characteristics, different treatments, and outcomes: A single-center study

“…With reference to ectopic tumors, our study found 69% remission rate, comparable to the findings described in the literature, where cure rates vary greatly, ranging from 12 to 71%, in view of the various tumor types that can be the culprits thereof 24 .…”

Cushing’s syndrome caused by ACTH-producing thymic typical carcinoid with local invasion and regional lymph node metastasis: a case report

“…Ectopic ACTH-producing thymic carcinoid tumor is an extremely rare clinical condition, comprising 29% of all thymic carcinoids and 5–42% of all ectopic ACTH-producing syndrome [ 1 , 2 ]. It has been reported that radical surgical resection of the ACTH source is the only effective treatment [ 3 ]. Prior to surgery, medication therapy should be done to prevent perioperative complications and perform surgery when hormone values and symptoms are controlled.…”

A Case of Pulmonary Carcinoid Tumor with a Superimposed Aspergilloma Presenting As a Covert Ectopic Adrenocorticotropic Hormone Syndrome

“…Concerning the management of EAS, surgical excision is the first treatment of choice once a single source of the ectopic tumor is successfully localized. Approximately 83% of EAS patients with bronchial carcinoid tumors recover after radical excision ( 4 , 11 ). If curative resection is not successful, medications including adrenal enzyme inhibitors and glucocorticoid antagonists should be considered to control the deleterious consequences of hypercortisolemia ( 11 ).…”

“…If curative resection is not successful, medications including adrenal enzyme inhibitors and glucocorticoid antagonists should be considered to control the deleterious consequences of hypercortisolemia ( 11 ). Furthermore, a bilateral adrenalectomy with steroid replacement is an alternative treatment when the primary tumor cannot be found and medicinal controls fail ( 4 ). Fortunately, our patient endured hypercortisolism with the only necessary treatment being successful surgical resection of the source.…”

“…Most cases of ACTH-dependent Cushing syndrome are caused by corticotroph pituitary adenomas (90%), while ectopic ACTH production occurs in approximately 10% of cases ( 2 ). Ectopic ACTH syndrome (EAS) is defined as endogenous hypercortisolism caused by ACTH-secreting non-pituitary solid tumors, most of which originate in the chest cavity ( 3 , 4 ). Accurately localizing the source of the ACTH-secreting lesion is crucial for resolving the condition through the removal of the primary ACTH-producing tumor ( 5 , 6 ).…”