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

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

Zizi Zhou  1 , Wenxiang Chai  1 , Longhai Yang  1 , Yi Liu  1 , Yao Liu  1 , Huiyu Pan  1 , Qiang Wu  1 , Xiaoming Zhang  2 , Eric Dominic Roessner  3
Affiliations
Case Reports

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

Zizi Zhou et al. BMC Surg. .

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.

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Conflict of interest statement

All the authors have read the manuscript and approved its submission. The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
A Characteristic facial features of Cushing syndrome. B Preoperative (up) and postoperative CT scans (Down). C Scar via subxiphoid approach. D resected mass. E Histological morphology and immunohistochemical images confirmed neuroendocrine tumor (carcinoid)
Fig. 2
Fig. 2
Changes in cortisol and ACTH levels after surgery
See this image and copyright information in PMC

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