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thymic neuroendocrine tumor - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

thymic neuroendocrine tumor

MONDO:0019964

Also known as:neuroendocrine neoplasm of thymusthymus NETthymus neuroendocrine neoplasm

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Overview

Thymic endocrine tumor is a rare, malignant, primary thymic neoplasm originating from neuroendocrine cells, presenting as a mass within the anterior mediastinum. Patients typically present with nonspecific symptoms, such as chest pain, cough, shortness of breath, or in some cases, superior vena cava syndrome, although patients could be asymptomatic during the early stages or present with multiple endocrine neoplasia type I. Ectopic production of ACTH and serotonin can lead to Cushing syndrome and carcinoid sydrome, respectively.

Clinical Trials1 registered

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include always present findings: Neoplasm of the thymus and Neuroendocrine neoplasm; and very common findings: Abnormal mediastinum morphology and Mediastinal lymphadenopathy. 24 total HPO annotations.

Organ System	Phenotype Count	Example Features
Hormones	5	Increased circulating cortisol level, Neoplasm of the endocrine system, Pituitary adenoma

Diagnosis

Biomarker and diagnostic research for thymic neuroendocrine tumor has been reported in the published literature.

Prognosis

Phenotype severity distribution: 2 always present features, 2 very common features, 11 common features.

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

1 clinical trial registered. Interventions under study include drug therapy, other interventions, and procedural interventions. Pipeline includes 1 PHASE3. Research is primarily sponsored by academic and government institutions.

117 publications have been identified in PubMed for thymic neuroendocrine tumor. Research spans Case Report / Case Series (25%), Basic Science / Preclinical (22%), and Review / Meta-Analysis (15%).

Research Type	Count	% of Total
Patient case studies	29

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 4:31 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:97289

GARD

Genetic and Rare Diseases Info Center

GARD:19361

Questions for Your Care Team

→Are any of the 1 active clinical trial a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Kisho

thymic neuroendocrine tumor

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

Latest News & Developments

Clinicopathological features and outcomes of immature PIT-1 lineage tumors: A high-risk pituitary neuroendocrine tumor/pituitary adenoma subtype per WHO 2022.

Use of PRRT and osilodrostat for ectopic Cushing syndrome secondary to thymic neuroendocrine neoplasm.

Life-Threatening Radiation Necrosis After Complete Response to Pembrolizumab in a Patient With a Metastatic Silent PIT1 Pituitary Neuroendocrine Tumor (PitNET).

thymic neuroendocrine tumor

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

Latest News & Developments

Clinicopathological features and outcomes of immature PIT-1 lineage tumors: A high-risk pituitary neuroendocrine tumor/pituitary adenoma subtype per WHO 2022.

Use of PRRT and osilodrostat for ectopic Cushing syndrome secondary to thymic neuroendocrine neoplasm.

Life-Threatening Radiation Necrosis After Complete Response to Pembrolizumab in a Patient With a Metastatic Silent PIT1 Pituitary Neuroendocrine Tumor (PitNET).