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Síndrome Cushing por secreção ectópica de ACTH
ORPHA:99889CID-10 · E24.3CID-11 · 5A70.1DOENÇA RARA

É um tipo de Síndrome de Cushing que acontece quando há uma produção excessiva do hormônio ACTH. Essa produção em excesso de ACTH é causada por um tumor que não fica na glândula hipófise, e que pode ser benigno (não canceroso) ou, mais frequentemente, maligno (canceroso).

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Introdução

O que você precisa saber de cara

📋

É um tipo de Síndrome de Cushing que acontece quando há uma produção excessiva do hormônio ACTH. Essa produção em excesso de ACTH é causada por um tumor que não fica na glândula hipófise, e que pode ser benigno (não canceroso) ou, mais frequentemente, maligno (canceroso).

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adult
+ elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: E24.3
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

📏
Crescimento
11 sintomas
🧠
Neurológico
8 sintomas
🧬
Pele e cabelo
7 sintomas
🫃
Digestivo
5 sintomas
🩸
Sangue
5 sintomas
🫁
Pulmão
4 sintomas

+ 34 sintomas em outras categorias

Características mais comuns

90%prev.
Fraqueza fatigável dos músculos proximais dos membros
Muito frequente (99-80%)
90%prev.
Aumento do nível urinário de cortisol
Muito frequente (99-80%)
90%prev.
Diabetes mellitus
Muito frequente (99-80%)
90%prev.
Aumento do nível circulante de ACTH
Muito frequente (99-80%)
90%prev.
Aumento do nível circulante de cortisol
Muito frequente (99-80%)
90%prev.
Aumento paradoxal da secreção de cortisol no teste de supressão por dexametasona
Muito frequente (99-80%)
83sintomas
Muito frequente (6)
Frequente (34)
Ocasional (26)
Muito raro (15)
Sem dados (2)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 83 características clínicas mais associadas, ordenadas por frequência.

Fraqueza fatigável dos músculos proximais dos membrosFatiguable weakness of proximal limb muscles
Muito frequente (99-80%)90%
Aumento do nível urinário de cortisolIncreased urinary cortisol level
Muito frequente (99-80%)90%
Diabetes mellitus
Muito frequente (99-80%)90%
Aumento do nível circulante de ACTHIncreased circulating ACTH level
Muito frequente (99-80%)90%
Aumento do nível circulante de cortisolIncreased circulating cortisol level
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa3
Últimos 10 anos200publicações
Pico202585 papers
Linha do tempo
2023Hoje · 2026🧪 2019Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
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2Fase 21
1Fase 11
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 2 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome Cushing por secreção ectópica de ACTH

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Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

Gallbladder neuroendocrine carcinoma: a rare cause of ectopic ACTH secretion.

BMJ case reports2026 Feb 10

This report presents a case of a middle-aged man who was incidentally diagnosed with a gallbladder (GB) mass. Though he had some features of Cushing syndrome (CS), including those due to mineralocorticoid effects (persistent hypokalaemia), the classical features were absent. Abdominal CT scan showed a large mass arising from the GB with liver and lymph node metastasis. Biochemical evaluation confirmed adrenocorticotropic hormone (ACTH)-dependent CS. A biopsy from the mass revealed a poorly differentiated neuroendocrine carcinoma. The patient was started on ketoconazole and appropriate management for other complications. Chemotherapy was poorly tolerated and hence discontinued. The patient eventually succumbed to his illness. This case illustrates how the absence of classical Cushingoid features can mask ectopic ACTH secretion, with persistent unexplained hypokalaemia serving as a key diagnostic clue in our case. It also emphasises the difficulty of identifying a neuroendocrine tumour arising from a rare, unsuspected primary site.

#2

Ectopic ACTH production from oesophageal neuroendocrine carcinoma causing Cushing syndrome.

BMJ case reports2026 Feb 04

Ectopic Cushing syndrome (ECS) occurs when a non-pituitary tumour secretes excess adrenocorticotropic hormone, leading to hypercortisolism. ECS is most commonly associated with small cell lung cancer and pancreatic neuroendocrine tumours, but rarely with oesophageal neuroendocrine carcinoma (NEC). We report the case of a man in the 60s initially treated for locally advanced large cell NEC of the oesophagus who later developed ECS. Oesophageal NECs account for less than 0.04% of all neuroendocrine tumours and carry a poor prognosis, with large cell subtype being particularly uncommon. Despite endocrine-directed therapy, the condition of the patient deteriorated rapidly due to aggressive disease. This case underscores the diagnostic challenges of ECS in rare tumour types and highlights the importance of early recognition and multidisciplinary management to optimise outcomes.

#3

The Clinical Spectrum and Management of Ectopic Cushing's Syndrome: A 12-Year Single-Center Experience.

Experimental and clinical endocrinology &amp; diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association2026 Feb

Ectopic Cushing's syndrome is a severe endocrine disorder with high morbidity and mortality, making timely diagnosis and effective cortisol control crucial. The aim of this study was to assess the 12-year experience at a tertiary centre.We retrospectively analyzed clinical, biochemical, imaging, treatment, and outcome data in this single-center, non-interventional cohort of patients treated from 2012 to 2024 at the University Hospital Centre Zagreb.Twelve patients were included (10/12 female, 83.3%; median age 43.5 years, range 22-74). Primary tumors were predominantly neuroendocrine (8/12, 66.7%), and 9/12 (75.0%) had metastatic disease at diagnosis. Common features were fat redistribution in 10/12 (83.3%), facial plethora in 8/12 (66.7%), hypertension in 8/12 (66.7%), and peripheral edema in 7/12 (58.3%). Frequent biochemical alterations were hypokalemia in 11/12 (91.7%) and hyperglycemia or new-onset/worsening diabetes in 10/12 (83.3%). Median 24-hour urinary free cortisol was 5,594 nmol/24 h (range: 196-72,954), and ACTH 52.4 pmol/L (range: 8-498). A urinary free cortisol level≥50×the upper limit of normal identified a high-risk group with shorter survival (Spearman ρ=-0.50). Biochemical remission was achieved in 1/12 (8.3%) patient through tumor resection, while the majority required bilateral adrenalectomy (8/12, 66.7%) due to inadequate response to medical therapy or as first-line treatment. At the time of last follow-up, 8/12 (66.7%) patients had died due to disease progression, with a median time to death of 12 months (range: 1-51). The median overall survival was 20 months (range: 1-154).Most patients presented with metabolic disturbances and limited response to medical therapy, leading to bilateral adrenalectomy. Early mortality was driven by uncontrolled hypercortisolism rather than tumor burden, and higher urinary free cortisol was associated with shorter survival. These findings highlight the prognostic relevance of cortisol burden and the importance of prompt cortisol control.

#4

Ectopic Cushing Syndrome Due to a Large Mediastinal Neuroendocrine Tumor.

JCEM case reports2026 Mar

Ectopic Cushing syndrome is an uncommon cause of hypercortisolism that needs rapid recognition and treatment to reduce complications. Here, we present the case of a 33-year-old man with a 1-year history of severe Cushing syndrome. Biochemical findings showed both high ACTH and 24-hour urine free cortisol, and nonsuppressed morning serum cortisol. The 18fluorine-1,4,7-triazacyclononane-1,4,7-triacetate-octreotide positron emission tomography/computed tomography revealed a large mediastinal tumor with high uptake. Initial biopsy reported a grade 1 neuroendocrine tumor with positive ACTH immunostaining. Treatment was initiated with ketoconazole and somatostatin analog. To achieve rapid Cushing syndrome control, etomidate intravenous infusion was started. After multidisciplinary assessment and because of high surgical risk (concern for airway compromise from tumor location above the trachea, size, and possible mechanical lung compression with laparoscopic adrenalectomy) debulking surgery of the primary tumor was performed first, followed by bilateral adrenalectomy. Pathology findings of the R2 tumor showed a higher grade tumor than previously reported and an 18 Fluorodeoxyglucose positron emission tomography/computed tomography demonstrated distant metastatic disease. In summary, severe hypercortisolism usually occurs in the setting of ectopic production, it can be debilitating with increased mortality, and in this case, tumor aggressiveness and location made management particularly challenging, requiring a multidisciplinary approach.

#5

Challenges of pediatric ectopic ACTH syndrome from sacrococcygeal teratoma.

Journal of pediatric endocrinology &amp; metabolism : JPEM2026 Feb 26

Ectopic ACTH syndrome (EAS) is a rare cause of Cushing's syndrome, especially in children, and has been only exceptionally linked to sacrococcygeal teratomas (SCTs). We report a pediatric case of SCT-associated EAS and review the literature to highlight diagnostic challenges and management strategies. A 13-year-old girl presented with hypertension, hypokalemia, hyperglycemia, and rapid weight gain. Biochemical testing confirmed ACTH-dependent hypercortisolism, while pituitary MRI was negative. Pelvic CT revealed a 5.1 cm presacral mass. PET/CT based on ^18F-FDG showed only mild glucose uptake (SUVmax 3.26) in the mass, whereas ^68Ga-DOTA-NOC PET/CT demonstrated intense somatostatin receptor (SSTR) expression (SUVmax 55.86). Laparoscopic resection identified a mature teratoma containing a well-differentiated neuroendocrine tumor (NET G1) positive for ACTH and SSTR2. Postoperatively, cortisol/ACTH levels normalized, and metabolic abnormalities resolved with 9-month follow-up showing no recurrence. This report describes the first adolescent case of EAS due to a neuroendocrine tumor within a sacrococcygeal teratoma. Complementary PET/CT on FDG metabolism and SSTR expression is critical for localizing occult neuroendocrine components. Complete tumor resection and multidisciplinary collaboration are key to favorable outcomes, expanding the spectrum of pediatric EAS and providing valuable insight into its evaluation and management.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC6 artigos no totalmostrando 198

2026

Gallbladder neuroendocrine carcinoma: a rare cause of ectopic ACTH secretion.

BMJ case reports
2026

The Clinical Spectrum and Management of Ectopic Cushing's Syndrome: A 12-Year Single-Center Experience.

Experimental and clinical endocrinology &amp; diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
2026

Ectopic Cushing Syndrome Due to a Large Mediastinal Neuroendocrine Tumor.

JCEM case reports
2026

Challenges of pediatric ectopic ACTH syndrome from sacrococcygeal teratoma.

Journal of pediatric endocrinology &amp; metabolism : JPEM
2026

A rare case of ectopic cushing's syndrome caused by renal neuroendocrine tumor.

Journal of diabetes and metabolic disorders
2026

Ectopic Adrenocorticotropic Hormone (ACTH) Syndrome Caused by Mediastinal Carcinoma: A Report of a Rare Case.

Cureus
2026

[68Ga]Ga-DOTATATE PET/CT and PET/MR enhances the detection of pituitary ACTH-secreting adenomas in cushing's disease.

Frontiers in endocrinology
2026

Ectopic maxillary ACTH-secreting adenoma complicated by Nelson's syndrome: a case report.

Annales d'endocrinologie
2026

Ectopic ACTH production from oesophageal neuroendocrine carcinoma causing Cushing syndrome.

BMJ case reports
2026

Disease activity and maternal-fetal outcomes in pregnant women with cushing's syndrome: a systematic review and meta‑analysis.

Reviews in endocrine &amp; metabolic disorders
2026

[Pheochromocytoma or Cushing's syndrome? About one case].

Annales de biologie clinique
2026

A positive corticotropin-releasing hormone test may obviate the need for petrosal sinus sampling in patients with cushing's disease.

Journal of endocrinological investigation
2025

Case Report: Ectopic Cushing's syndrome caused by a pancreatic neuroendocrine tumor managed through dual steroidogenesis inhibition and endoscopic ultrasound-guided ethanol ablation.

Frontiers in endocrinology
2025

Diabetic Ketoacidosis as the First Manifestation of Ectopic Cushing's Syndrome.

Cureus
2025

Hypokalemic metabolic alkalosis as a clinical clue to ectopic ACTH syndrome: two cases of neuroendocrine carcinoma.

Endocrine regulations
2025

Ectopic Adrenocorticotropic Hormone (ACTH)-Dependent Cushing Syndrome Secondary to Olfactory Neuroblastoma: A Rare Entity.

AACE endocrinology and diabetes
2025

Role of 68Ga-DOTATOC Positron Emission Tomography in Locating Pulmonary Neuroendocrine Tumor Presenting with ACTH-Dependent Cushing's Syndrome: A Case Report.

Journal of clinical medicine
2025

A Rare Case of Adrenocorticotropic Hormone (ACTH)-Secreting Pheochromocytoma Presenting With Severe Cushing Syndrome (CS) and Dual Hormonal Secretion.

Cureus
2025

A Second Look at Refractory Edema: Delayed Diagnosis of Paraneoplastic Cushing's Syndrome in Small Cell Lung Cancer.

Cureus
2026

Crooke Cell Adenoma Presenting With Refractory Hypokalemia and Uncontrolled Hypertension.

JCEM case reports
2026

Multicenter 30-Year Data on the 4-Mg Intravenous Dexamethasone Suppression Test in the Diagnosis of Cushing Syndrome.

Journal of the Endocrine Society
2026

18 F-FDG PET/CT and 68 Ga-DOTA-NOC PET/MRI in an Adolescent With Ectopic Adrenocorticotropic Hormone Syndrome From a Presacral Teratoma.

Clinical nuclear medicine
2025

A Difficult Case of Ectopic ACTH Syndrome: Is Treatment Possible Even Without Accurate Localization?

Journal of the ASEAN Federation of Endocrine Societies
2025

Ultrasound-Guided Jugular Vein Access for Inferior Petrosal Sinus Sampling: A Safe and Feasible Technique.

Cureus
2025

Case Report: ACTH- and CRH-secreting pheochromocytoma as a very rare cause of ectopic Cushing syndrome.

Frontiers in endocrinology
2025

Revamped perspective on conventional interpretation: the foreboding prognostic significance of low-lateralization in inferior petrosal sinus sampling for diagnosis of Cushing's disease.

BMC endocrine disorders
2025

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

Frontiers in endocrinology
2025

A Young Woman with Paraneoplastic Cushing's Syndrome Due to a Pulmonary Carcinoid.

Reports (MDPI)
2025

Clinical management of ectopic Cushing Syndrome in neuroendocrine neoplasms: a national survey.

Frontiers in endocrinology
2026

ACTH-secreting atypical carcinoid lung tumour expanding the Lynch syndrome spectrum.

Journal of medical genetics
2025

Cycle characterisation and clinical complications in patients with cyclic Cushing's syndrome: insights from an international retrospective cohort study.

The lancet. Diabetes &amp; endocrinology
2025

[Aggressive course of ectopic ACTH syndrome due to adrenal medulla hyperplasia. Case report].

Terapevticheskii arkhiv
2025

Severe Cushing Syndrome From Ectopic Adrenocorticotropin Secretion In Metastatic Prostate Cancer Treated With Osilodrostat.

JCEM case reports
2025

Three Cases of Ectopic, Cyclic Cushing Syndrome: A New Square Wave Variant.

JCEM case reports
2025

Clinical efficacy and safety of fluconazole treatment in patients with Cushing's syndrome.

Therapeutic advances in endocrinology and metabolism
2026

A case of prolonged adrenal insufficiency following osilodrostat discontinuation with a review of the literature.

Endocrine journal
2025

Diagnosis of Cushing's Disease.

Endocrinology and metabolism clinics of North America
2025

A comparison of bilateral internal jugular vein sampling with bilateral inferior petrosal sinus sampling at Mayo Clinic for the subtype evaluation of corticotropin-dependent cushing syndrome.

Pituitary
2025

Complete surgical resection of cortisol secreting neuroendocrine thymic tumour: a case report.

Journal of cardiothoracic surgery
2025

Co-occurrence of Ectopic Adrenocorticotropic Hormone (ACTH) Secretion and Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH) in Small Cell Lung Cancer: A Case Report.

Cureus
2025

Evaluating prolactin levels during bilateral inferior petrosal sinus sampling: a potential solution to false-negative results in Cushing's disease diagnosis.

Endocrine
2025

Ectopic Cushing's syndrome in a patient with DIPNECH and metastatic lung carcinoid.

Endocrinologia, diabetes y nutricion
2026

Osilodrostat-associated Adrenal Gland Shrinkage: A Case Series of Patients With ACTH-dependent Cushing's Syndrome.

The Journal of clinical endocrinology and metabolism
2025

Cushing Disease Mimicking Ectopic Adrenocorticotropic Hormone-Secreting Disease.

AACE endocrinology and diabetes
2025

Update in the Management of ACTH-Secreting Gastroenteropancreatic and Thoracic Neuroendocrine Neoplasms.

Current treatment options in oncology
2025

Peptide Receptor Radionuclide Therapy-Induced Hypercortisolemic Crisis in Ectopic Cushing Syndrome.

JCEM case reports
2025

A Silent Invader: Asymptomatic Rhodococcus Infection Unmasked in A Patient with Ectopic ACTH-Dependent Cushing's Syndrome.

European journal of case reports in internal medicine
2025

Case Report: Ectopic CRH production by adrenal adenoma as a unique cause of the ACTH-dependent Cushing's syndrome.

Frontiers in endocrinology
2025

Salient Observations from Clinical Profile and Long-Term Outcome of a Cohort of Patients with Ectopic Cushing's Syndrome.

Indian journal of endocrinology and metabolism
2025

Efficacy and Hepatotoxicity During Rapid Titration of Ketoconazole and/or Metyrapone in Patients With Cushing Syndrome.

Journal of the Endocrine Society
2025

Rapid Adrenal Atrophy Following Excision of an Ectopic Adrenocorticotropin-Secreting Lung Carcinoid Tumor.

JCEM case reports
2025

Relapse of Cyclic Cushing Syndrome With a 19-Year Remission: Potential Involvement of COVID-19 in the Relapse.

JCEM case reports
2025

Prolactin-adjusted inferior petrosal sinus sampling: Pituitary and ectopic adrenocorticotropic hormone-dependent Cushing syndrome.

Journal of neuroendocrinology
2025

Episodic Severe Ectopic Adrenocorticotropic Hormone Syndrome by Metastatic Appendiceal Neuroendocrine Tumor.

JCEM case reports
2025

A Case of Micro-medullary Thyroid Carcinoma Presenting as Cancer of Unknown Primary.

Cureus
2025

Bilateral Adrenal Ablation for the Treatment of Ectopic ACTH Syndrome With Severe Hypokalemia: A Case Report and a Literature Review.

Clinical case reports
2025

[A unique case of severe ACTH-dependent Cushing's syndrome due to ectopic corticotropin production by medullary thyroid carcinoma].

Problemy endokrinologii
2025

Ectopic ACTH Secretion Induced by an Olfactory Neuroblastoma: A Case Report.

Case reports in endocrinology
2025

Advances in the medical management of hypercortisolism: current strategies, monitoring, and emerging therapies.

Current opinion in endocrinology, diabetes, and obesity
2025

Ectopic Cushing Syndrome due to Metastatic Medullary Thyroid Cancer.

AACE endocrinology and diabetes
2025

Radiofrequency Ablation: Alternative Treatment of Ectopic Adrenocorticotropic Syndrome Secondary to Lung Carcinoid Tumor.

JCEM case reports
2025

Case Report: Refractory hypokalemia as a clinical manifestation of Crooke's cell adenoma.

Frontiers in medicine
2025

Differences in clinical, hormonal and radiological profile between Cushing's disease and ectopic Cushing's syndrome. Series of 37 cases.

Annales d'endocrinologie
2024

A RARE CASE OF CATASTROPHIC CUSHING'S SYNDROME DUE TO ACTH-SECRETING PHEOCHROMOCYTOMA: CHALLENGES IN THE DIAGNOSTIC JOURNEY.

Acta endocrinologica (Bucharest, Romania : 2005)
2025

Ectopic adrenocorticotropic hormone syndrome due to olfactory neuroblastoma: A case report and literature review.

Endocrinologia, diabetes y nutricion
2025

Corrigendum: Case report: Pheochromocytoma-induced pseudo-Cushing's syndrome.

Frontiers in endocrinology
2025

A Secret Revealed: The Coexistence of a Pheochromocytoma and Independent Adrenocorticotropic Hormone-Secreting Cushing Syndrome Within the Same Adrenal Gland.

Cureus
2025

Survival probabilities in patients with ectopic Cushing's syndrome-a systematic review and a single-arm meta-analysis.

European journal of endocrinology
2025

Ectopic Cushing's Syndrome in Advanced Small-Cell Lung Cancer (SCLC): Clinical Challenges and Therapeutic Insights.

Cancers
2025

Cushing's Syndrome due to a Renal Neuroendocrine Tumor: A Case Report.

Case reports in oncology
2025

Ectopic Adrenocorticotropic Hormone Production in a Stage IV Neuroendocrine Tumor: A Rare Presentation of Cushing's Syndrome.

Cureus
2025

Efficacy of Propranolol Combined with Different Modalities of Unilateral Adrenalectomy in a Case Series of 4 ARMC5-mutated Patients.

Experimental and clinical endocrinology &amp; diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
2025

Is Ectopic Cushing Syndrome Commonly Associated with Small Cell Lung Cancer (SCLC)? Critical Review of the Literature and ACTH Expression in Resected SCLC.

Endocrine pathology
2025

A novel molecular imaging technique using [68Ga]Ga-mDesmo PET-CT for localizing tumors in Cushing's disease.

European journal of nuclear medicine and molecular imaging
2025

Neuroendocrine Breast Cancer-Associated Ectopic Adrenocorticotropic Hormone Syndrome Requiring Bilateral Adrenalectomy.

Current oncology (Toronto, Ont.)
2025

Primary Renal Neuroendocrine Tumor Presenting as Ectopic ACTH Syndrome.

JCEM case reports
2025

Hypercortisolism Due to Paraganglioma Secreting Adrenocorticotropin and Catecholamines.

JCEM case reports
2025

[Ectopic ACTH secretion by neuroendocrine carcinoma of the uterine cervix].

Medicina
2025

A Rare Case of Metastatic Adrenocorticotrophic Hormone - Secreting Pancreatic Neuroendocrine Tumor Causing Ectopic Cushing Syndrome in a 46-Year-Old Woman.

The American journal of case reports
2025

Ectopic ACTH-secreting pheochromocytoma without typical signs of Cushing syndrome.

Oxford medical case reports
2025

Ectopic ACTH syndrome secondary to small-cell oropharyngeal cancer.

BMJ case reports
2025

Osilodrostat Treatment for Adrenal and Ectopic Cushing Syndrome: Integration of Clinical Studies With Case Presentations.

Journal of the Endocrine Society
2025

Case Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing's syndrome.

Frontiers in oncology
2025

Prospective Evaluation of 68Ga-DOTATATE and 18F-DOPA PET Scans in Detecting Tumors Causing Ectopic ACTH Syndrome.

The Journal of clinical endocrinology and metabolism
2025

Unraveling hypertension through mineralocorticoid receptor activation in Cushing's syndrome: A case report.

Clinical nephrology
2025

Transcriptome-Wide Analysis of Pituitary and Ectopic Adrenocorticotropic Hormone-Secreting Tumors.

Cancers
2025

An ACTH-Producing Neuroendocrine Tumor: Clinical Course of Multidisciplinary Therapy Including Peptide Receptor Radionuclide Therapy - A Case Report.

Case reports in oncology
2025

A Pheochromocytoma With Adrenocorticotropic Hormone Secretion and Subsequent Fatal Outcome: A Case Report.

Cureus
2025

Ectopic Corticotropin-Releasing Hormone/Adrenocorticotropic Hormone-Co-Secreting Neuroendocrine Tumors Leading to Cushing's Disease: A Case Presentation and Literature Review.

Neuroendocrinology
2025

Challenges in diagnosis and treatment of cushing disease in a 12-year-old boy. Case report.

La Pediatria medica e chirurgica : Medical and surgical pediatrics
2025

Recognition and Management of Ectopic ACTH Secreting Tumors.

Journal of the Endocrine Society
2025

Finding the Culprit: Cushing Syndrome Secondary to Lung Carcinoid Tumor.

AACE clinical case reports
2024

Performance of Vasopressin Stimulated Bilateral Inferior Petrosal Sinus Sampling in Corticotropin Dependent Cushing's Syndrome with Negative or Equivocal 3 Tesla Contrast Enhanced Magnetic Resonance Imaging of Pituitary.

Indian journal of endocrinology and metabolism
2025

Leukocytosis in Cushing's syndrome persists post-surgical remission and could predict a lower remission prognosis in patients with Cushing's disease.

Journal of endocrinological investigation
2025

Successful Treatment of Severe Ectopic ACTH-Dependent Cushing Syndrome Complicated by Hypocalcemia With Osilodrostat.

JCEM case reports
2024

Case report: Pheochromocytoma-induced pseudo-Cushing's syndrome.

Frontiers in endocrinology
2024

Ectopic ACTH syndrome caused by thymic neuroendocrine tumour - stages of treatment.

Endokrynologia Polska
2024

Severe Ectopic Adrenocorticotropic Hormone Syndrome Due to Pulmonary Carcinoid Tumor: A Case Report and Literature Review.

AACE clinical case reports
2024

The diagnostic value of prolactin adjustment in bilateral inferior petrosal sinus sampling for differentiating Cushing's disease from the ectopic ACTH syndrome: a systematic review and meta-analysis.

Pituitary
2025

Desmopressin is a safe and effective secretagogue to replace corticotropin-releasing hormone in petrosal sinus sampling.

Annales d'endocrinologie
2025

Is there still a place for etomidate in the management of Cushing's syndrome? The experience of a single center of low-dose etomidate and combined etomidate-osilodrostat treatment in severe hypercortisolemia.

Endocrine
2024

Complete Tumor Resection and Radical Lymphadenectomy: Potential Cure for Adrenocorticotropic Hormone (ACTH)-Dependent Pulmonary Carcinoid.

Cureus
2024

Addisonian Pigmentation - The Great Mimicker - A Review.

Indian journal of dermatology
2024

Malignant thyroid neoplasm with ectopic Cushing's syndrome.

BMJ case reports
2024

Co-Existing Ectopic Cortisol-Producing Adenoma and Retroperitoneal Schwannoma, a Rare Case Report.

Diabetes, metabolic syndrome and obesity : targets and therapy
2024

Extra-adrenal adrenocortical cancer associated with multiple endocrine neoplasia type 1.

Endocrinology, diabetes &amp; metabolism case reports
2024

Ectopic adrenocorticotrophic hormone syndrome in a 10-year-old girl with a thymic neuroendocrine tumor: a case report.

BMC endocrine disorders
2024

Ectopic ACTH-Dependent Cushing's Syndrome Emerging at a Late Stage of a Mixed Histology Neuroendocrine Neoplasm: A Case Report.

Case reports in oncology
2024

Challenging Diagnostic Workup of a 22-year-old Patient With Primary Pigmented Nodular Adrenocortical Disease.

JCEM case reports
2024

Frequency of clinical signs in patients with Cushing's syndrome and mild autonomous cortisol secretion: overlap is common.

European journal of endocrinology
2025

Cushing syndrome in paediatric population: who and how to screen.

Journal of endocrinological investigation
2024

Comparison of clinical, hormonal, pathological and treatment outcomes of ectopic Cushing's syndrome by sex: results of a multicenter study.

Endocrine
2024

Cushing syndrome from an ACTH-producing pheochromocytoma or paraganglioma: structured review of 94 cases.

Endocrine-related cancer
2024

Cushing's Syndrome: Rapid Evidence Review.

American family physician
2024

A Rare Case of Ectopic Adrenocorticotropic Hormone Syndrome (EAS) in an Adolescent Girl With a Thymic Neuroendocrine Tumour.

Cureus
2025

An aggressive Cushing's syndrome originating from a rare thymic neuroendocrine tumor, controlled successfully with fluconazole and octreotide therapy before surgery.

Journal of cancer research and therapeutics
2024

Understanding negative feedback: Changes in high-molecular-weight adrenocorticotropic hormone in adrenocorticotropic hormone-independent Cushing's syndrome.

Journal of neuroendocrinology
2025

Assessment of aortic perivascular and renal sinus fat in endogenous cortisol excess of different etiology.

Hormones (Athens, Greece)
2024

Ectopic Cushing Syndrome Secondary to Diffuse Idiopathic Neuroendocrine Cell Hyperplasia-A Report of 2 Cases.

JCEM case reports
2024

Unusual Presentation of Case of a Lung Carcinoid Tumor With Ectopic Adrenocorticotropic Secretion (EAS) Associated With Acute Weight Gain and Peripheral Edema.

Cureus
2023

[Fulminant hypercorticism due to ACTG producing pheochromocytoma].

Problemy endokrinologii
2024

Ectopic Cushing's Syndrome in Pediatric Age.

Endocrine, metabolic &amp; immune disorders drug targets
2024

Diagnostic Accuracy of the Desmopressin Stimulation Test in the Comprehensive Assessment of ACTH-Dependent Cushing's Syndrome: A Comparative Analysis with BIPSS and TSS.

Endocrine research
2024

Ectopic Cushing syndrome in metastatic castration‑resistant prostate cancer: A case report and review of literature.

Oncology letters
2024

ACTH-producing adrenocortical carcinoma: an exceedingly rare diagnosis.

Journal of the Egyptian National Cancer Institute
2024

Diagnostic performance of various imaging modalities in localizing ectopic ACTH syndrome: A systematic review.

Annales d'endocrinologie
2024

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
2024

Evaluating the usefulness of plasma chromogranin A measurement in cyclic ACTH-dependent Cushing's syndrome.

Endocrine journal
2024

A Rare Cyclic Cushing Syndrome Mystery Illustrates Diagnostic Principles.

JCEM case reports
2024

Ectopic adrenocortical adenoma characterized by hypogonadism: a case report and review of the literature.

Journal of medical case reports
2024

A Cadaveric Case of Bilateral Adrenal Hyperplasia With Lung Cancer: A Paraneoplastic Syndrome?

Cureus
2024

Complete remission of hypercortisolemia in a patient with severe ectopic Cushing syndrome caused by small-cell lung cancer.

Polish archives of internal medicine
2024

Cushing Syndrome Due to a Corticotropin-Releasing Hormone- and Adrenocorticotropic Hormone-Secreting Silent Pheochromocytoma.

AACE clinical case reports
2024

Difficult Diagnosis and Management of Concealed Cushing.

Endocrine, metabolic &amp; immune disorders drug targets
2025

Biochemical Control in Cushing's Syndrome: Outcomes of the Treatment in a Large Single Center Cohort.

The Journal of clinical endocrinology and metabolism
2024

[A rare cause of hypercorticism: ACTH-secreting pheochromocytoma (a case report)].

The Pan African medical journal
2024

A Case Report of Bilateral Adrenal Gland Stereotactic Body Radiotherapy to Manage Hypercortisolemia in a Patient With Ectopic Adrenocorticotropic Hormone (ACTH) Production From a Metastatic Pancreatic Neuroendocrine Tumor.

Cureus
2024

Hypokalemia Due to Ectopic Adrenocorticotropic Hormone.

WMJ : official publication of the State Medical Society of Wisconsin
2024

A Case of Pancreatic Neuroendocrine Tumor with Liver Metastases Demonstrating the Possibility of Enhanced ACTH Production by the SACI Test.

Case reports in endocrinology
2024

Olfactory Neuroblastoma Causing Cushing's Syndrome Due to the Ectopic Adrenocorticotropic Hormone (ACTH) Secretion: A Case Report.

Cureus
2024

Personalized Noninvasive Diagnostic Algorithms Based on Urinary Free Cortisol in ACTH-dependant Cushing's Syndrome.

The Journal of clinical endocrinology and metabolism
2024

Bilateral inferior petrosal sinus sampling in the differential diagnosis of ACTH-dependent Cushing's syndrome: A reappraisal.

Journal of internal medicine
2024

Bilateral Inferior Petrosal Sinus Sampling Without Lateralization Is Less Accurate for the Diagnosis of Cushing Disease.

Journal of the Endocrine Society
2024

Subclinical Cushing's Disease with High-Molecular-Weight Forms of Adrenocorticotropic Hormone Production.

Case reports in endocrinology
2024

Paraneoplastic Syndromes in Neuroendocrine Prostate Cancer: A Systematic Review.

Current oncology (Toronto, Ont.)
2024

[Hypertensive Emergency as the Debut of Paraneoplastic Cushing Syndrome].

Hipertension y riesgo vascular
2025

Machine Learning May Be an Alternative to BIPSS in the Differential Diagnosis of ACTH-dependent Cushing Syndrome.

The Journal of clinical endocrinology and metabolism
2024

A challenging case of ectopic ACTH-dependent Cushing's syndrome due to medullary thyroid carcinoma.

Endokrynologia Polska
2024

Delayed Diagnosis of Ectopic Cushing Syndrome.

JCEM case reports
2024

Pheochromocytoma With High Adrenocorticotropic Hormone Production Capacity Without Pigmentation and Cushingoid Symptoms: A Case Report With a Literature Review.

Cureus
2024

[Machine learning methods in differential diagnosis of ACTH-dependent hypercortisolism].

Problemy endokrinologii
2024

Fulminant ectopic Cushing's syndrome caused by metastatic small intestine neuroendocrine tumour - a case report and review of the literature.

Acta gastro-enterologica Belgica
2024

Clinical features and risk factors for postoperative recurrence in patients with Cushing's syndrome of different etiologies.

Scientific reports
2024

Accuracy of the 10 μg desmopressin test for differential diagnosis of Cushing syndrome: a systematic review and meta-analysis.

Frontiers in endocrinology
2024

Ectopic adrenocorticotropic hormone-secreting pheochromocytoma with severe metabolic disturbances: A case report.

International journal of surgery case reports
2024

[Diagnostic value of bilateral inferior petrosal sinus sampling in various modifications and methods of radiation and radionuclide imaging in the diagnosis and differential diagnosis of ACTH-dependent endogenous hypercortisolism].

Problemy endokrinologii
2024

Diverse presentations of Cushing's syndrome during pregnancy - A case series.

The Australian &amp; New Zealand journal of obstetrics &amp; gynaecology
2024

A Case of an Ectopic ACTH-Producing Tumor With Adrenal Shrinkage During Osilodrostat Administration.

JCEM case reports
2024

Second Malignancy Masquerading as Recurrence of Neuroendocrine Tumor.

JCEM case reports
2024

Two cases of pancreatic neuroendocrine tumors with ectopic ACTH syndrome during their disease course.

Clinical journal of gastroenterology
2024

Is [68Ga]Ga-DOTA-TATE PET/CT useful in the diagnosis of patients suspected of ectopic Cushing's syndrome?

Endokrynologia Polska
2024

High prevalence of venous thrombotic events in Cushing's syndrome: data from ERCUSYN and details in relation to surgery.

European journal of endocrinology
2024

A clinical perspective on ectopic Cushing's syndrome.

Trends in endocrinology and metabolism: TEM
2023

Adrenocorticotropin-Dependent Ectopic Cushing's Syndrome: A Case Report.

Cureus
2024

Prolonged adrenal insufficiency following discontinuation of osilodrostat treatment for intense hypercortisolism.

European journal of endocrinology
2023

Cushing's syndrome caused by ACTH precursors secreted from a pancreatic yolk sac tumor in an adult-a case report and literature review.

Frontiers in medicine
2024

Paediatric Cushing syndrome: a prospective, multisite, observational cohort study.

The Lancet. Child &amp; adolescent health
2025

False-Negative Inferior Petrosal Sinus Sampling in Young-Onset Cushing Disease: What Happens Next.

Hormone research in paediatrics
2023

Ectopic Hormonal Syndromes: Cushing's Syndrome and Acromegaly.

Archives of medical research
2025

A Challenging Case of Ectopic Adrenocorticotropin Hormone Syndrome with Bronchial Carcinoid and Literature Review.

Journal of clinical research in pediatric endocrinology
2023

Adrenocorticotropic hormone-secreting phaeochromocytoma as a cause of treatment-resistant hypertension and recurrent pulmonary emboli.

The journal of the Royal College of Physicians of Edinburgh
2023

Management of small cell lung cancer complicated with paraneoplastic Cushing's syndrome: a systematic literature review.

Frontiers in endocrinology
2023

Successful Management of Cushing Syndrome From Ectopic ACTH Secretion in an Adolescent With Osilodrostat.

JCEM case reports
2023

Cushing Syndrome due to a Pancreatic Neuroendocrine Tumor Treated With Radiofrequency Ablation.

JCEM case reports
2023

The Elusive Neuroendocrine Tumor: Finding the Ectopic ACTH Source 16 Years After the Diagnosis of Cushing Syndrome.

JCEM case reports
2023

Long-term Treatment With Metyrapone in a Man With Ectopic Cushing Syndrome.

JCEM case reports
2023

Pancreatic ACTH Hypersecretion and Pituitary Macroadenoma.

JCEM case reports
2023

Clival Ectopic Pituitary Adenoma Causing Cushing Syndrome.

JCEM case reports
2023

Bilateral inferior petrosal sinus sampling: validity, diagnostic accuracy in lateralization of pituitary microadenoma, and treatment in eleven patients with Cushing's syndrome - a single-center retrospective cohort study.

BMC endocrine disorders
2023

Ectopic Adrenocorticotropic Hormone-Producing Metastatic Gastrinoma: A Case Report.

Cureus
2023

Sertoli leydig cell tumor of the ovary in a woman with cushing syndrome: A case report.

Gynecologic oncology reports
2023

Paediatric and adolescent ectopic Cushing's syndrome: systematic review.

European journal of endocrinology
2023

ACTH-Producing Neuroendocrine Carcinoma of the Liver with Cushing's Syndrome.

Case reports in oncological medicine
2023

Ectopic adrenocorticotropic hormone (ACTH) syndrome: two case reports.

Neuro endocrinology letters
2023

ACTH-producing small cell neuroendocrine carcinoma from the gallbladder: a case report and literature review.

Frontiers in endocrinology
2023

Ectopic Cushing's syndrome from a corticotropin-releasing hormone-secreting medullary thyroid carcinoma: a rare pitfall of inferior petrosal sinus sampling.

Endocrinology, diabetes &amp; metabolism case reports
2023

Pulmonary Mucoepidermoid Carcinoma With Multiple Paraneoplastic Syndromes.

Cureus
2023

Impact of Cushing's syndrome on the gonadotrope axis and testicular functions in men.

Human reproduction (Oxford, England)
2023

Elevated Serum Androstenedione Level in a Patient With Ectopic Adrenocorticotropic Hormone Syndrome.

AACE clinical case reports
2023

Heterogeneity of TPIT expression in ACTH-secreting extra-pituitary neuroendocrine tumors (NETs) supports the existence of different cellular programs in pancreatic and pulmonary NETs.

Virchows Archiv : an international journal of pathology
2024

Toward Optimized and Cost-Efficient Protocols for Inferior Petrosal Sinus Sampling in the Diagnosis of Cushing Disease.

Neurosurgery
2023

ACTH-secreting pancreatic neuroendocrine neoplasms: A case-series.

Journal of neuroendocrinology
2023

A Double-Headed Physiologic Monster: A Case Report and Literature Review.

Cureus
2023

[Infectious pneumonia favored by paraneoplastic Cushing syndrome in a pulmonary carcinoid tumor].

Revue des maladies respiratoires
2023

Enhancing Cushing's disease diagnosis: exploring the impact of desmopressin on ACTH gradient during BIPSS.

Frontiers in endocrinology
2023

Emerging diagnostic methods and imaging modalities in cushing's syndrome.

Frontiers in endocrinology
2023

Ovine CRH Stimulation and 8 mg Dexamethasone Suppression Tests in 323 Patients With ACTH-Dependent Cushing's Syndrome.

The Journal of clinical endocrinology and metabolism
2023

Cushing's syndrome as a paraneoplastic feature of renal cell carcinoma: A case report.

Urology case reports

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Gallbladder neuroendocrine carcinoma: a rare cause of ectopic ACTH secretion.
    BMJ case reports· 2026· PMID 41802720mais citado
  2. Ectopic ACTH production from oesophageal neuroendocrine carcinoma causing Cushing syndrome.
    BMJ case reports· 2026· PMID 41638770mais citado
  3. The Clinical Spectrum and Management of Ectopic Cushing's Syndrome: A 12-Year Single-Center Experience.
    Experimental and clinical endocrinology &amp; diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association· 2026· PMID 41786303mais citado
  4. Ectopic Cushing Syndrome Due to a Large Mediastinal Neuroendocrine Tumor.
    JCEM case reports· 2026· PMID 41767198mais citado
  5. Challenges of pediatric ectopic ACTH syndrome from sacrococcygeal teratoma.
    Journal of pediatric endocrinology &amp; metabolism : JPEM· 2026· PMID 41762714mais citado
  6. A rare case of ectopic cushing's syndrome caused by renal neuroendocrine tumor.
    J Diabetes Metab Disord· 2026· PMID 41757383recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:99889(Orphanet)
  2. MONDO:0020527(MONDO)
  3. GARD:19698(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q32136799(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Síndrome Cushing por secreção ectópica de ACTH
Compêndio · Raras BR

Síndrome Cushing por secreção ectópica de ACTH

ORPHA:99889 · MONDO:0020527
Prevalência
Unknown
Herança
Not applicable
CID-10
E24.3 · Síndrome do ACTH ectópico
CID-11
Início
Adult, Elderly
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0001231
EuropePMC
Wikidata
DiscussaoAtiva

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