O tumor endócrino tímico é uma neoplasia tímica primária, maligna, rara, originada de células neuroendócrinas, apresentando-se como uma massa no mediastino anterior. Os pacientes geralmente apresentam sintomas inespecíficos, como dor torácica, tosse, falta de ar ou, em alguns casos, síndrome da veia cava superior, embora os pacientes possam ser assintomáticos durante os estágios iniciais ou apresentar neoplasia endócrina múltipla tipo I. A produção ectópica de ACTH e serotonina pode levar à síndrome de Cushing e à síndrome carcinoide, respectivamente.
Introdução
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O tumor endócrino tímico é uma neoplasia tímica primária, maligna, rara, originada de células neuroendócrinas, apresentando-se como uma massa no mediastino anterior. Os pacientes geralmente apresentam sintomas inespecíficos, como dor torácica, tosse, falta de ar ou, em alguns casos, síndrome da veia cava superior, embora os pacientes possam ser assintomáticos durante os estágios iniciais ou apresentar neoplasia endócrina múltipla tipo I. A produção ectópica de ACTH e serotonina pode levar à síndrome de Cushing e à síndrome carcinoide, respectivamente.
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Publicações mais relevantes
Brief Report: Androgen Receptor Expression Is Associated With Male Predominance in Thymic Neuroendocrine Neoplasms.
Neuroendocrine neoplasms represent a rare and poorly understood collection of malignancies. A better understanding of their biology is needed to improve treatment options. Male predominance to the incidence of neuroendocrine neoplasms of the thymus suggests a biological basis for this observation. This single-institution, retrospective cohort study evaluated androgen receptor (AR) expression and other clinicopathologic features in thymic neuroendocrine neoplasms compared with thymic epithelial tumors and neuroendocrine neoplasms of other sites. Expression of neuroendocrine and prostate markers was also assessed. For further molecular characterization, copy number analysis of AR and other relevant genes was assessed by single nucleotide polymorphism array. Last, analysis of single genes and thymic epithelial gene sets was performed using published thymic tumor transcriptomes. A cohort was assembled from 17 patients with neuroendocrine malignancies of primary thymic origin with assessable tissue for immunohistochemical analysis. The cohort was predominantly male (12 males, five females). Immunohistochemical analysis of the AR demonstrated positive staining in nine cases, all of which were male. Compared with thymic neuroendocrine neoplasms, AR expression is considerably less frequent in thymomas, thymic carcinoma, and neuroendocrine neoplasms of other sites of origin. No relevant copy number changes in the AR were identified. Further molecular characterization revealed expression of ASCL1 and limited expression of common prostate lineage and differentiation markers within our cohort, including thymic neuroendocrine epithelial gene set enrichment in published transcriptomes of thymic neuroendocrine neoplasms. The AR is expressed in a considerable fraction of thymic neuroendocrine neoplasms and is associated with male predominance. This observation has implications for investigation of androgen deprivation and receptor blockade.
Successful Management of Ectopic Adrenocorticotropin-Secreting Thymic Carcinoid With Mitotane: A New Look at an Old Drug.
Ectopic adrenocorticotropin syndrome (EAS) is usually associated with severe multiple complications and high mortality. Several adrenal steroidogenesis inhibitors can be used to control hypercortisolism when curative surgery is not feasible, but with different availability worldwide. It was long considered that mitotane (MTT) was not suitable for severe Cushing syndrome (CS) due to its delayed onset of action. We present a case of a 17-year-old girl with rapid-onset CS and an extremely high 24-hour urinary free cortisol (UFC) level (>300 times the upper limit of normal). An anterior mediastinal nodule with contrast enhancement was identified in computed tomography, with positive 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography with computed tomography (PET/CT) uptake (maximum standardized uptake value = 10.1), suggestive of a thymic neuroendocrine tumor as the most likely cause of EAS. Preoperative MTT monotherapy titrated to 2 g/day reduced UFC by 85% within 13 days without adverse effects, stabilized severe neuropsychiatric disturbances and opportunistic infections, thus enabling successful thymectomy. The tumor turned out to be an adrenocorticotropin-secreting thymic typical carcinoid. Other EAS cases treated with MTT reported in the literature were reviewed, and the time needed to control hypercortisolism using MTT was shorter than previously reported. Instead of an "add-on drug," we should reconsider the role of MTT in the treatment of severe hypercortisolism in EAS.
Correction to "Thymic Neuroendocrine Tumor Presenting with Cervical Lymphadenopathy: A Case Report and Diagnostic Challenge".
[This corrects the article DOI: 10.1002/ccr3.71580.].
Postoperative adrenal crisis and atrial tachycardia associated with an ectopic ACTH-secreting thymic neuroendocrine tumor: a case report and literature review.
Thymic neuroendocrine tumors associated with ectopic adrenocorticotropic hormone (ACTH) syndrome are rare, and postoperative adrenal crisis presenting with atrial tachycardia as the initial manifestation is even more uncommon. As a result, evidence to guide early recognition and management remains limited. This article reports a case of an ectopic ACTH-secreting thymic neuroendocrine tumor, complicated by postoperative adrenal crisis, with recurrent atrial tachycardia. Following tumor resection, the patient developed profound shock accompanied by recurrent atrial tachycardia, which improved significantly after prompt initiation of glucocorticoid administration and ventricular rate control. Adrenal crisis often presents with nonspecific clinical symptoms, predisposing it to delayed or missed diagnosis. However, during the perioperative management of ectopic ACTH-secreting neuroendocrine tumors, failure to administer glucocorticoids promptly and adequately can trigger an adrenal crisis that may rapidly progress to life-threatening complications, including atrial tachycardia, shock, and acute respiratory failure.Therefore, clinicians should aim to recognize this risk at the earliest possible stage. A multidisciplinary approach during the perioperative period remains essential to ensure timely and individualized glucocorticoid replacement therapy, thereby optimizing patient outcomes. Not applicable. The online version contains supplementary material available at 10.1186/s12902-026-02171-8.
Two novel multiple endocrine neoplasia type 1 variants caused thymic neuroendocrine tumor: a case report.
Publicações recentes
Brief Report: Androgen Receptor Expression Is Associated With Male Predominance in Thymic Neuroendocrine Neoplasms.
Successful Management of Ectopic Adrenocorticotropin-Secreting Thymic Carcinoid With Mitotane: A New Look at an Old Drug.
Two novel multiple endocrine neoplasia type 1 variants caused thymic neuroendocrine tumor: a case report.
Postoperative adrenal crisis and atrial tachycardia associated with an ectopic ACTH-secreting thymic neuroendocrine tumor: a case report and literature review.
📚 EuropePMC33 artigos no totalmostrando 63
Brief Report: Androgen Receptor Expression Is Associated With Male Predominance in Thymic Neuroendocrine Neoplasms.
JTO clinical and research reportsSuccessful Management of Ectopic Adrenocorticotropin-Secreting Thymic Carcinoid With Mitotane: A New Look at an Old Drug.
JCEM case reportsTwo novel multiple endocrine neoplasia type 1 variants caused thymic neuroendocrine tumor: a case report.
Hereditary cancer in clinical practicePostoperative adrenal crisis and atrial tachycardia associated with an ectopic ACTH-secreting thymic neuroendocrine tumor: a case report and literature review.
BMC endocrine disordersThymic Neuroendocrine Tumor Presenting With Cervical Lymphadenopathy: A Case Report and Diagnostic Challenge.
Clinical case reportsPeptide Receptor Radionuclide Therapy-Induced Hypercortisolemic Crisis in Ectopic Cushing Syndrome.
JCEM case reportsMedical Practice Guidelines for Lung Cancers including malignant pleural mesotheliomas and thymic tumors according to evidence-based medicine. 2024. Edited by the Japan Lung Cancer Society. Chapter 3 Guidelines for thymic epithelial tumors - Summary of recommendations.
Respiratory investigationAtypical Carcinoid of the Thymus: Early Diagnosis in a Case Report.
Medical sciences (Basel, Switzerland)Recent Clinical and Molecular Advances in the Management of Thymic Carcinoids: A Comprehensive Review.
CancersUtility of gel filtration chromatography in evaluating successful resection of ectopic adrenocorticotropic hormone-producing tumor: a case report and literature review.
Endocrine journalCharacterization, Prognosis, and Treatment of Patients With Locally Advanced or Metastatic Thymic Neuroendocrine Tumor: A Retrospective Study of the French GTE, ENDOCAN RENATEN, and RYTHMIC Networks.
Journal of thoracic oncology : official publication of the International Association for the Study of Lung CancerCase Report: Thymic neuroendocrine tumor with metastasis to the breast causing ectopic Cushing's syndrome.
Frontiers in oncologyEctopic adrenocorticotrophic hormone syndrome in a 10-year-old girl with a thymic neuroendocrine tumor: a case report.
BMC endocrine disordersAn 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 therapeuticsSuccessful management of thymic neuroendocrine tumour with paraneoplastic limbic encephalitis.
Interdisciplinary cardiovascular and thoracic surgeryImaging and Histopathological Features Of Primary Thymic Neuroendocrine Tumor.
Current medical imagingIncidence of second malignancies in patients with thymic carcinoma and thymic neuroendocrine tumor.
Journal of cancer research and clinical oncologyLong-term Treatment With Metyrapone in a Man With Ectopic Cushing Syndrome.
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GenesThe International Association for the Study of Lung Cancer Thymic Epithelial Tumor Staging Project: A Re-Assessment of the International Thymic Malignancy Interest Group/International Association for the Study of Lung Cancer Lymph Node Map for Thymic Epithelial Tumors for the Forthcoming Ninth Edition of the TNM Classification of Malignant Tumors.
Journal of thoracic oncology : official publication of the International Association for the Study of Lung CancerThe International Association for the Study of Lung Cancer Thymic Epithelial Tumor Staging Project: Proposal for the T Component for the Forthcoming (Ninth) Edition of the TNM Classification of Malignant Tumors.
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MedicineEfficacy and Safety of [177Lu]Lu-DOTA-TATE in Adults with Inoperable or Metastatic Somatostatin Receptor-Positive Pheochromocytomas/Paragangliomas, Bronchial and Unknown Origin Neuroendocrine Tumors, and Medullary Thyroid Carcinoma: A Systematic Literature Review.
Biomedicines[Thymic Neuroendocrine Tumor Associated with Multiple Endocrine Neoplasia Type 1].
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Journal of the College of Physicians and Surgeons--Pakistan : JCPSPPrognostic value of systemic inflammatory markers and the nutrition status in thymic epithelial tumors with complete resection.
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Annals of palliative medicineDual-scopic robotic thymectomy for a large thymic malignant tumor.
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Annals of surgical oncologyTrends in the incidence of thymoma, thymic carcinoma, and thymic neuroendocrine tumor in the United States.
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Endocrinology, diabetes & metabolism case reportsCapecitabine and Temozolomide as a Promising Therapy for Advanced Thymic Atypical Carcinoid.
The oncologistEvaluation of the prognostic value of surgery and postoperative radiotherapy for patients with thymic neuroendocrine tumors: A propensity-matched study based on the SEER database.
Thoracic cancerJLCS medical practice guidelines for thymic tumors: summary of recommendations.
Japanese journal of clinical oncologyCapecitabine and Celecoxib as a Promising Therapy for Thymic Neoplasms.
American journal of clinical oncology[Thymic neuroendocrine tumor: about a case and review of the literature].
The Pan African medical journalWhole exome sequencing of thymic neuroendocrine tumor with ectopic ACTH syndrome.
European journal of endocrinologyInvasive atypical thymic carcinoid: three case reports and literature review.
OncoTargets and therapySurgical treatment of ectopic adrenocorticotropic hormone syndrome with intra-thoracic tumor.
Journal of thoracic disease[Thymic neuroendocrine carcinoma with Pompe's disease of the adult].
MedicinaImpact of Positive Nodal Metastases in Patients with Thymic Carcinoma and Thymic Neuroendocrine Tumors.
Journal of thoracic oncology : official publication of the International Association for the Study of Lung CancerEctopic ACTH syndrome caused by desmopressin-responsive thymic neuroendocrine tumor.
Endocrine journalThymic Origin Neuroendocrine Carcinoma Metastasizing to the Orbit in an Otherwise Asymptomatic Patient.
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Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Brief Report: Androgen Receptor Expression Is Associated With Male Predominance in Thymic Neuroendocrine Neoplasms.
- Successful Management of Ectopic Adrenocorticotropin-Secreting Thymic Carcinoid With Mitotane: A New Look at an Old Drug.
- Correction to "Thymic Neuroendocrine Tumor Presenting with Cervical Lymphadenopathy: A Case Report and Diagnostic Challenge".
- Postoperative adrenal crisis and atrial tachycardia associated with an ectopic ACTH-secreting thymic neuroendocrine tumor: a case report and literature review.
- Two novel multiple endocrine neoplasia type 1 variants caused thymic neuroendocrine tumor: a case report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:97289(Orphanet)
- MONDO:0019964(MONDO)
- GARD:19361(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55789019(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.
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