[Thymic epithelial neoplasms: updates on diagnosis, staging, biology and management in France]

Bull Cancer. 2012 Nov;99(11):1045-55. doi: 10.1684/bdc.2012.1659.
[Article in French]

Abstract

Thymic epithelial neoplasms are rare malignancies with about 250 new incident cases in France every year. The WHO histologic classification distinguishes thymoma and thymic carcinoma which are tumors with different biological and clinical behaviors and outcomes. The Masaoka-Koga staging system is considered as a reference and is also of prognosis value. Diagnosis, multimodal treatment and follow-up of thymic epithelial neoplasms require a multidisciplinary approach where surgery is the cornerstone treatment. A national expert center coordinates thymic epithelial neoplasms management with 12 other regional expert centers through the French organization named RYTHMIC (www.rythmic.org). Patient's files have to be discussed at regional or national multidisciplinary staff. A group of expert pathologists will centrally review tumors when the diagnosis or classification is a matter of controversy. Among its objectives, RYHTMIC has to promote medical education, patient's information and research. This review focuses on RYTHMIC guidelines and data regarding multimodal management and targeted therapies in epithelial thymic neoplasms.

Publication types

  • Review

MeSH terms

  • ErbB Receptors / metabolism
  • France
  • Genes, erbB-1
  • Humans
  • Molecular Targeted Therapy / methods
  • Myasthenia Gravis / etiology
  • Neoplasm Staging / methods
  • Neoplasms, Glandular and Epithelial* / diagnosis
  • Neoplasms, Glandular and Epithelial* / metabolism
  • Neoplasms, Glandular and Epithelial* / pathology
  • Neoplasms, Glandular and Epithelial* / therapy
  • Neovascularization, Pathologic / therapy
  • Paraneoplastic Syndromes / etiology
  • Practice Guidelines as Topic*
  • Prognosis
  • Proto-Oncogene Proteins c-kit / metabolism
  • Rare Diseases* / diagnosis
  • Rare Diseases* / metabolism
  • Rare Diseases* / pathology
  • Rare Diseases* / therapy
  • Thymoma* / diagnosis
  • Thymoma* / metabolism
  • Thymoma* / pathology
  • Thymoma* / therapy
  • Thymus Neoplasms* / diagnosis
  • Thymus Neoplasms* / metabolism
  • Thymus Neoplasms* / pathology
  • Thymus Neoplasms* / therapy

Substances

  • ErbB Receptors
  • Proto-Oncogene Proteins c-kit

Supplementary concepts

  • Thymic epithelial tumor