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Genetics of Endocrine and Neuroendocrine Neoplasias (PDQ®)

  • Last Modified: 11/06/2014

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Changes to This Summary (11/06/2014)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Multiple Endocrine Neoplasia Type 2 (MEN2)

Revised text to state that the use of vandetanib and cabozantinib is approved by the U.S. Food and Drug Administration for adult patients with progressive metastatic medullary thyroid cancer who are ineligible for surgery; a phase III study found that progression-free survival was longer in adults who received vandetanib than in those who received placebo. Also added that a phase I/II study of children with MEN2B found an objective partial response rate of 47% with vandetanib (cited Fox et al. as reference 218).

Added level of evidence 4.

Familial Paraganglioma (FPGL) Syndrome

Added text to state that paragangliomas (PGLs) found in the skull base or head and neck region are usually from parasympathetic paraganglia and rarely secrete catecholamines. Also revised text to state that representing 3% of all PGLs, the tumors in the cervical region typically arise in the glomus cells, near the aortic body, along the vagal nerve, in the temporal bone, in the jugular fossa (middle ear space), or in the nose and parasinus.

Revised text to state that PGLs arise from cells involved in the metabolism of catecholamines, but approximately 95% of tumors located in the head and neck region are parasympathetic and nonsecretory.

Added Hussain et al. as reference 23.

Added text to state that FPGL syndromes are among the rare inherited diseases in which genomic imprinting occurs.

This summary is written and maintained by the PDQ Cancer Genetics Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.