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6 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
MEN2 is an inherited condition, one of the multiple endocrine neoplasia syndromes, in which a faulty RET gene causes medullary thyroid cancer in almost every carrier, often with adrenal tumours and overactive parathyroids. Because the thyroid cancer is so predictable, children who inherit the gene have the thyroid removed at an age set by which RET mutation they carry.
MEN2 is caused by germline activating mutations in the RET proto-oncogene. The American Thyroid Association guideline divides it into MEN2A, with medullary thyroid carcinoma, phaeochromocytoma and primary hyperparathyroidism (and the variants with cutaneous lichen amyloidosis or Hirschsprung disease, and familial medullary thyroid carcinoma, now classed as an MEN2A variant), and MEN2B, with earlier and more aggressive medullary thyroid carcinoma, phaeochromocytoma, mucosal neuromas and a marfanoid habitus, almost always from the RET M918T mutation (Wells 2015). The WHO endocrine classification lists MEN2 among the genetic tumour syndromes.
How it differs from its parent: MEN1 is a menin syndrome centred on parathyroid, pancreas and pituitary; MEN2 is a RET syndrome whose defining cancer is medullary thyroid carcinoma, which the corpus covers on its own page. The guideline stratifies RET mutations into highest risk (M918T), high risk (codon 634 and A883F) and moderate risk, and times prophylactic thyroidectomy accordingly: in the first year of life for the highest-risk group, at or before age five for the high-risk group, and by calcitonin surveillance for the moderate group; phaeochromocytoma is excluded before any operation (Wells 2015).
| Setting | Approach | Guideline |
|---|---|---|
| Gene carriers | Prophylactic thyroidectomy timed by RET risk category, after excluding phaeochromocytoma (ATA 2015). | not mapped |
| Established medullary thyroid carcinoma | Treated as the medullary thyroid cancer page describes; selpercatinib for advanced RET-driven disease (LIBRETTO-531). | not mapped |