Presentation of multiple endocrine neoplasia type 2A-associated ectopic cushing’s syndrome: case report and a systematic review

MEN 2-related ECS should be considered in differentials. Adrenalectomy typically achieved cure in most ECS due to PHEO, but vigilance is required for the double risk of both hypercatecholaminemia and hypercortisolism during the perioperative period. Whereas most ECS due to advanced-MTC eventually required BLA to improve symptoms, yet prognosis remained generally poor. TKIs might offer benefits in the management of both MTC and hypercortisolism. The integration of <i>RET</i> testing, early diagnosis, and precise treatment can help prevent ECS complications and improve outcomes.

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Publication Details

Journal
Frontiers in Endocrinology
Published
2025-11-11
DOI
https://doi.org/10.3389/fendo.2025.1644751
Primary Topic
Pituitary Gland Disorders and Treatments
Type
article
Field-Weighted Citation Impact
0.00

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article

Presentation of multiple endocrine neoplasia type 2A-associated ectopic cushing’s syndrome: case report and a systematic review

Xiao-Ping Qi, Hongyuan Yu, Feiping Li, Weitao Yao et al.
Frontiers in Endocrinology
Pituitary Gland Disorders and Treatments
article

Presentation of multiple endocrine neoplasia type 2A-associated ectopic cushing’s syndrome: case report and a systematic review

Xiao-Ping Qi, Hongyuan Yu, Feiping Li, Weitao Yao, Meixian Zhang, Zhen‐Yu Chen, Wang Jun-wei, Wei Wang, Weiying Chen, Jianqiang Zhao, Jun Lü, Zhi-Lie Cao
article en

Abstract

MEN 2-related ECS should be considered in differentials. Adrenalectomy typically achieved cure in most ECS due to PHEO, but vigilance is required for the double risk of both hypercatecholaminemia and hypercortisolism during the perioperative period. Whereas most ECS due to advanced-MTC eventually required BLA to improve symptoms, yet prognosis remained generally poor. TKIs might offer benefits in the management of both MTC and hypercortisolism. The integration of <i>RET</i> testing, early diagnosis, and precise treatment can help prevent ECS complications and improve outcomes.

Frontiers in EndocrinologyVol. 16
Wenzhou Medical University (CN), 117th Hospital of People's Liberation Army (CN), Zhejiang Cancer Hospital (CN), The First People's Hospital of Tianmen (CN), Third People's Hospital of Huzhou (CN), Zhejiang Taizhou Hospital (CN), Hangzhou Medical College (CN), Taizhou University (CN)
National Natural Science Foundation of China, Medical Science and Technology Project of Zhejiang Province, Science and Technology Program of Zhejiang Province
Openalex Percentile: Top 54%
Pituitary Gland Disorders and Treatments
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