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

Asemota, Iriagbonse; Nwaichi, Chineme; Mbachi, Chimezie; Benjamín,

doi:10.7759/cureus.18590

Cited by 2 publications

(1 citation statement)

References 7 publications

(24 reference statements)

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“…Analysis of tumor tissue from a patient with MEN1 operated on twice for a thymic NET associated with the ectopic production of ACTH causing CS showed an initial Ki-67 labeling index of 5% but on re-operation the tumor was found to be invasive with a Ki-67 index of 30% ( 7 ). Another patient with germline mutation-proven MEN1 associated with thymic NET and ectopic CS was found at surgery to have an unresectable, invasive anterior mediastinal mass with a Ki-67 index of >50% ( 58 ). Although not well studied in the context of MEN1, such high Ki-67 indices and the increase in Ki-67 index noted on sequential operations likely represent the dominance over time of the more aggressive MEN1-null thymic neuroendocrine tumor clones.…”

Section: Cushing’s Syndrome Due To Ectopic Acth Secretion In Men1mentioning

confidence: 99%

Expressions of Cushing’s syndrome in multiple endocrine neoplasia type 1

Simonds

2023

Front. Endocrinol.

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Cushing’s syndrome (CS) resulting from endogenous hypercortisolism can be sporadic or can occur in the context of familial disease because of pituitary or extra-pituitary neuroendocrine tumors. Multiple endocrine neoplasia type 1 (MEN1) is unique among familial endocrine tumor syndromes because hypercortisolism in this context can result from pituitary, adrenal, or thymic neuroendocrine tumors and can therefore reflect either ACTH-dependent or ACTH-independent pathophysiologies. The prominent expressions of MEN1 include primary hyperparathyroidism, tumors of the anterior pituitary, gastroenteropancreatic neuroendocrine tumors, and bronchial carcinoid tumors along with several common non-endocrine manifestations such as cutaneous angiofibromas and leiomyomas. Pituitary tumors are present in about 40% of MEN1 patients, and up to 10% of such tumors secrete ACTH that can result in Cushing’s disease. Adrenocortical neoplasms occur frequently in MEN1. Although such adrenal tumors are mostly clinically silent, this category can include benign or malignant tumors causing hypercortisolism and CS. Ectopic tumoral ACTH secretion has also been observed in MEN1, almost exclusively originating from thymic neuroendocrine tumors. The range of clinical presentations, etiologies, and diagnostic challenges of CS in MEN1 are reviewed herein with an emphasis on the medical literature since 1997, when the MEN1 gene was identified.

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Section: Cushing’s Syndrome Due To Ectopic Acth Secretion In Men1mentioning

confidence: 99%

Expressions of Cushing’s syndrome in multiple endocrine neoplasia type 1

Simonds

2023

Front. Endocrinol.

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Ectopic ACTH Syndrome: Possibilities of Modern Diagnostics

Grineva,

Tsoy,

Ryzhkova

2024

Annals RAMS

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The ectopic ACTH syndrome (EAS) is a constellation of clinical signs and symptoms caused by chronic excess production of cortisol by the adrenal glands due to stimulation by adrenocorticotropic hormone (ACTH) of extrapituitary origin. EAS is a rare pathology, it accounts for approximately 20% of all cases of ACTH-dependent hypercortisolism and up to 10% of all causes of endogenous autonomic cortisol production, however, it is often associated with severe hypercortisolism and is a life-threatening condition. The sources of ectopic secretion of ACTH are neuroendocrine tumors (NET), which differ in histological type, malignant potential, clinical course, and also have different, often difficult to detect localization. Early identification of ACTH -producing NET reduces the risk of death of the patient both from severe hypercortisolism and the tumor itself. The paper describes the features of the course of EAS, the possibilities of its diagnosis, and presents an algorithm for EAS diagnosis, created on the basis of the latest achievements of Russian and world medicine.

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

Cited by 2 publications

References 7 publications

Expressions of Cushing’s syndrome in multiple endocrine neoplasia type 1

Expressions of Cushing’s syndrome in multiple endocrine neoplasia type 1

Ectopic ACTH Syndrome: Possibilities of Modern Diagnostics

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