Solid Tumour Section

نویسندگان

  • Yash Somnay
  • David Schneider
  • Haggi Mazeh
چکیده

Note Medullary thyroid cancers (MTC) are rare tumors of neuroendocrine origin that arise from parafollicular C cells which secrete a variety of peptides and hormones including calcitonin. As opposed to the more common papillary and follicular thyroid cancer subtypes, MTC represents a rare and under-characterized form of cancer, and can cause death if untreated (Taccaliti et al., 2011). MTC can be either sporadic, usually isolated to one thyroid lobe, or familial, the latter of which is defined as the cancer syndrome known as Multiple Endocrine Neoplasia type 2 (MEN2) (Frank-Raue et al., 2010). MEN2 is the result of the autosomally dominant missense gain of function mutation in the RET (Rearranged during Transfection) proto-oncogene. MEN2 can be further subclassified into MEN2A, MEN2B and Familial Medullary Thyroid Carcinoma (FMTC). MEN2A is defined by the occurrence of medullary thyroid carcinoma (MTC), in conjunction with pheochromocytomas and primary hyperparathyroidism. MEN2B, is definied by the presence of MTC, pheochromocytomas, ganglioneuromatosis of the gastrointestinal tract, mucosal neuromas of the lips and tongue, and a Marfanoid body habitus (Frank-Raue et al., 2010). FMTC occurs when MTC is the only clinical feature, rarely with other endocrine neoplasias. Offspring of affected carriers of the RET mutation have a 50% chance of inheriting the mutation. Clinics and pathology

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تاریخ انتشار 2013