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

Abstract

BackgroundEctopic ACTH-producing thymic carcinoid tumors are rare, but often behave aggressively with local invasion and distant metastasis. We herein report a case of ACTH-producing thymic typical carcinoid tumor with lymph node metastasis treated by surgery and postoperative radiation therapy.Case presentationA 61-year-old woman was admitted to be evaluated for hypoglycemia and hypokalemia. Laboratory data revealed elevation of serum cortisol and ACTH levels. Overnight administration of 8 mg dexamethasone did not suppress plasma ACTH. Chest CT demonstrated a tumor of 30 mm in diameter and enlargement of the lymph node at the anterior mediastinum. Ectopic ACTH syndrome was suspected and total thymectomy and lymph node dissection were performed. The histopathological examination indicated typical carcinoid tumor and mediastinal lymph node metastasis, and immunohistochemical staining was positive for ACTH. The plasma ACTH level decreased immediately after surgery. She received postoperative radiation therapy of 60 Gy.ConclusionEctopic ACTH-producing thymic typical carcinoid tumors are rare, and it is important to consider this disease and perform appropriate treatment.

ACTH‐producing thymic neuroendocrine tumor initially presenting as psychosis: A case report and literature review

“…The serum cortisol level at diagnosis in the present case was higher than in the previous case reports. We have summarized case reports and reviewed patients with ACTH‐producing thymic tumors over the past 10 years published in English, and the cortisol and ACTH levels are presented in Table . Serum cortisol and plasma ACTH levels in our patient were relatively high, and we speculated that the extremely high cortisol level could have been related to the development of severe psychosis.…”

Cushing’s Syndrome Due to a Functional Thymic Neuroendocrine Tumor in Multiple Endocrine Neoplasia Type 1 Syndrome

“…The clinical spectrum has now been expanded and tumors may develop in several endocrine and non-endocrine organs including foregut neuroendocrine tumors (NET) such as bronchial and thymic NET, duodenal gastrinoma, adrenal adenoma, meningioma, collagenomas, and thyroid tumors [2,3]. Thymic NET-associated tumor with MEN 1 syndrome is extremely rare, has an aggressive course, is usually nonfunctional and has a poor prognosis [3][4][5]. In rare cases, they may be functional and secrete adrenocorticotropic hormone (ACTH) leading to Cushing's syndrome [4,5].…”

“…Thymic NET-associated tumor with MEN 1 syndrome is extremely rare, has an aggressive course, is usually nonfunctional and has a poor prognosis [ 3 - 5 ]. In rare cases, they may be functional and secrete adrenocorticotropic hormone (ACTH) leading to Cushing’s syndrome [ 4 , 5 ]. We describe a case of MEN 1 syndrome associated with thymic carcinoid with an aggressive clinical course and rapid progression to a fulminant ectopic ACTH-dependent Cushing’s syndrome.…”