Successful resolution of ectopic Cushing syndrome by minimally invasive thoracoscopic resection of the neuroendocrine tumor of the thymus: a rare case report

BMC Surg. 2022 Jun 11;22(1):226. doi: 10.1186/s12893-022-01674-0.

Abstract

Background: Ectopic Cushing syndrome (ECS) is a sporadic condition. Even uncommon is an ECS that derives from a carcinoid tumor of the thymus. These tumors may pose several diagnostic and therapeutic conundrums. This report discusses the differential diagnosis, clinicopathological findings, and effective treatment of a rare case of ECS using a minimally invasive approach.

Case presentation: A 29-year-old woman with Cushing syndrome presented with facial flushing. Physical examination revealed hypertension (blood pressure: 141/100 mmHg). A mediastinal tumor was discovered to be the cause of the patient's chronic hypokalemia and hypercortisolemia. Cortisol levels increased in the morning, reaching 47.7 ug/dL. The levels of the hormones ACTH, aldosterone, and renin were determined to be 281 pg/mL, 3.0 ng/dL, and 2.1 pg/mL, respectively. The presence of hypertension, hypokalemia, and alkalinity suggested Cushing's syndrome, which was proven to be ACTH-dependent ECS by a dexamethasone suppression test. A chest CT scan revealed inflammation in the posterior basal region of the right lower lobe. The superior anterior mediastinum was characterized by round-shaped isodensity lesions with distinct borders. She underwent thoracoscopic anterior mediastinal tumor excision via the subxiphoid technique (R0 resection); following surgery, her blood pressure returned to normal, and the hypernatremia/hypopotassemia resolved. The tumor was determined to be a thymic carcinoid. Most notably, cortisol levels fell to half of their presurgical levels after one hour of surgery, and other abnormalities corrected substantially postoperatively.

Conclusion: Thoracoscopic excision of thymic tumors by subxiphoid incision may be a useful treatment option for ECS caused by neuroendocrine tumors of the thymus.

Keywords: Case report; Cushing syndrome; Hyperaldosteronism; Neuroendocrine tumors; Thoracoscopic surgery.

Publication types

  • Case Reports

MeSH terms

  • ACTH Syndrome, Ectopic* / complications
  • ACTH Syndrome, Ectopic* / diagnosis
  • Adrenocorticotropic Hormone
  • Adult
  • Carcinoid Tumor* / complications
  • Carcinoid Tumor* / surgery
  • Cushing Syndrome* / diagnosis
  • Cushing Syndrome* / etiology
  • Cushing Syndrome* / surgery
  • Female
  • Humans
  • Hydrocortisone
  • Hypertension* / complications
  • Hypokalemia* / complications
  • Mediastinal Neoplasms* / complications
  • Mediastinal Neoplasms* / diagnosis
  • Mediastinal Neoplasms* / surgery
  • Neuroendocrine Tumors* / complications
  • Neuroendocrine Tumors* / pathology
  • Neuroendocrine Tumors* / surgery
  • Thymus Neoplasms* / complications
  • Thymus Neoplasms* / diagnosis
  • Thymus Neoplasms* / surgery

Substances

  • Adrenocorticotropic Hormone
  • Hydrocortisone