SynthesisReviews in endocrine & metabolic disorders2021
Ectopic Cushing's syndrome due to thymic neuroendocrine tumours: a systematic review.
Synthesis in Reviews in endocrine & metabolic disorders, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 18 citations in OpenAlex.
- Thymic epithelial tumors: what's next?Cancer metastasis reviews · 2026Review
- Thymic neuroendocrine tumours: molecular landscape, immune microenvironment and therapeutic perspectives.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Use of PRRT and osilodrostat for ectopic Cushing syndrome secondary to thymic neuroendocrine neoplasm.Endocrinology, diabetes & metabolism case reports · 2026Article
- European Society of Neuroendocrine Tumors (ENETS) 2025 guidance paper for lung and thymic carcinoids.Journal of neuroendocrinology · 2026Article
- Ectopic Cushing Syndrome Due to a Large Mediastinal Neuroendocrine Tumor.JCEM case reports · 2026Article
- Thymic Neuroendocrine Tumors: Evolving Insights and Innovative Approaches.JTO clinical and research reports · 2026Review
- Update in the Management of ACTH-Secreting Gastroenteropancreatic and Thoracic Neuroendocrine Neoplasms.Current treatment options in oncology · 2025Review
- Recent Clinical and Molecular Advances in the Management of Thymic Carcinoids: A Comprehensive Review.Cancers · 2025Review
- Efficacy and safety of surufatinib in the treatment of thymic neuroendocrine tumors: a 102-case retrospective study.The oncologist · 2025Article
- Case Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing's syndrome.Frontiers in oncology · 2025Article
- Case report: A challenging case of severe Cushing's syndrome in the course of metastatic thymic neuroendocrine carcinoma with a synchronous adrenal tumor.Frontiers in endocrinology · 2024Article
- Gene fusions are frequent in ACTH-secreting neuroendocrine neoplasms of the pancreas, but not in their non-pancreatic counterparts.Virchows Archiv : an international journal of pathology · 2023Article
- Long-term Treatment With Metyrapone in a Man With Ectopic Cushing Syndrome.JCEM case reports · 2023Article
- Successful resolution of ectopic Cushing syndrome by minimally invasive thoracoscopic resection of the neuroendocrine tumor of the thymus: a rare case report.BMC surgery · 2022Article
- Ectopic adrenocorticotrophic hormone syndrome initially presenting as abnormal mental behavior caused by thymic carcinoid: a case report.Gland surgery · 2022Article
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Authors and funding
11 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Knowledge of ectopic Cushing's syndrome (CS) due to thymic neuroendocrine tumours (NETs) comes from short series or single cases. Our aim is to perform a systematic review using PubMed, Embase, Scopus, Ovid Medline and Biosis Previews of all cases with ectopic CS due to thymic NETs reported in the last 40 years and describe one illustrative patient attended in our institution. Search of literature: From 162 patients, 58.6% were male and mean age was 34.6 ± 13.9 years-old. Median of symptoms until diagnosis was 6 [2-24] months and 62% had aggressive CS. Imaging was positive in 93.7% (chest X-ray), 97.8% (computed tomography), 80.7% (somatostatin receptor scintigraphy) and median tumour size was 47 [25-68.5] mm. At presentation, 18% had localized disease, 26.2% locally invasive and 55.7% advanced. Eighty-eight present underwent surgery and histological subtypes were atypical (46.7%), typical (30.4%) and carcinoma (21.7%). Tumour persisted or recurred in 70.1%, 63% received radiotherapy and 45.2% chemotherapy. Follow-up median was 26.6 [14.5-57.5] months and mortality was reported in 35.8% with median survival of 38 [19-60] months. MEN-1 mutation was referred in 3.1%. Comparatively, carcinomas had aggressive CS more frequently while atypical showed advanced disease more often. In conclusion, thymic NETs causing ectopic CS are presented as aggressive hypercortisolism in the middle aged population. The disease is commonly extended at diagnosis and persists or recurs after surgery in most patients with a short term high mortality.
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