Endocrine surgical aspects of multiple endocrine neoplasia syndromes in children.
BACKGROUND
All patients diagnosed with medullary thyroid carcinoma (MTC) should undergo RET mutation analysis to exclude familial disease - multiple endocrine neoplasia (MEN)-2A and -2B and familial medullary thyroid carcinoma (FMTC). In young patients at risk of genetically determined MTC, the key to a good outcome is an appropriate first operation, and this will depend upon the codon mutation, patient age, calcitonin level and disease extent at presentation. When MTC has already developed, a therapeutic intervention is required.


CONCLUSIONS
The thyroid, pituitary, adrenal, parathyroid and pancreatic components of MEN-1 and -2 require close collaboration of a specialist and experienced multidisciplinary team.
Harrison
Barney
B
Royal Hallamshire Hospital, Sheffield, UK. harind@pediatrics@ohio-state.edu
D018276:Carcinoma, Medullary / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D002648:Child; D004252:DNA Mutational Analysis; D003937:Diagnosis, Differential; D013507:Endocrine Surgical Procedures; D006801:Humans; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D018813:Multiple Endocrine Neoplasia Type 2a / Q000601:surgery; D018814:Multiple Endocrine Neoplasia Type 2b / Q000601:surgery; D009154:Mutation; D051096:Proto-Oncogene Proteins c-ret / Q000235:genetics; D013964:Thyroid Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000601:surgery
D051096:Proto-Oncogene Proteins c-ret; C099282:RET protein, human