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Thymic epithelial neoplasm

Orpha number ORPHA3398
Synonym(s) TEN
Thymic epithelial tumor
Prevalence Unknown
Inheritance
  • Autosomal recessive
  • Sporadic
Age of onset Adulthood
ICD-10
  • C37
  • D15.0
OMIM -
UMLS
  • C1266101
MeSH
  • C536905
MedDRA -
SNOMED CT
  • 128621008

Summary

Thymic epithelial neoplasms (TEN) are rare malignancies arising from the epithelium of the thymic gland. They comprise three sub-types: thymoma, thymic carcinoma, and thymic neuroendocrine carcinoma (see these terms).

TENs have an annual incidence of approximately 1/588,000. Thymoma is the most frequent subtype, while thymic carcinoma and thymic neuroendocrine carcinoma represent 1% and 2-4% of the anterior mediastinal tumors, respectively. The two latter have a higher malignant potential than thymoma. TENs usually occur in adults from 30 to 80 years of age, with a median age of 60 years, but they can also occur during childhood.

Most adult patients are asymptomatic, while in children nearly two thirds present with symptoms that include cough, chest pain, weight loss, fatigue and dyspnea. Many autoimmune syndromes are associated with TENs. Myasthenia gravis (see this term) is observed in 30-50% of thymoma cases. Phrenic nerve palsy and superior vena cava syndrome are usually observed in cases of thymic carcinoma.

Etiology is unknown.

Expert reviewer(s)

  • Dr Saul SUSTER

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