Initial surgical management of sporadic medullary thyroid cancer: Guidelines based optimal care ‐ A systematic review

Abstract

IntroductionMedullary thyroid carcinoma (MTC) is a rare neuroendocrine tumor from parafollicular cells that produce calcitonin (Ct). Despite several existing guidelines for the surgical management of sporadic MTC (sMTC), optimal initial surgical management of the thyroid, the central and the lateral neck remains a matter of debate.MethodsA systematic review in PubMed and Scopus for current guidelines addressing the surgical management of sMTC and its referenced citations was conducted as per the PRISMA guidelines.ResultsTwo‐hundred and one articles were identified, of which 7 met the inclusion criteria. Overall, guidelines vary significantly in their recommendations for the surgical management of sMTC. Only one guideline recommended partial thyroidectomy for limited disease, but the possibility to avoid completion thyroidectomy in selected cases is acknowledged in 42% (3/7) of the remaining guidelines. The majority of guidelines (71.4%; 5/7) recommended prophylactic central neck dissection (CND) for all patients while the remaining two guidelines recommended CND based on Ct level and tumor size. The role of prophylactic lateral neck dissection based on preoperative Ct levels was recommended by 42% (3/7) of guidelines. Overall, these guidelines are based on low‐quality evidence, mostly single‐center retrospective series, some of which are over 20 years old.ConclusionCurrent surgical management guidelines of sMTC should be revised, and ought to be based on updated data challenging current recommendations, which are based on historic, low‐quality evidence. Partial thyroidectomy may become a viable option for small, limited tumors. Prospective, multi‐center studies may be useful to conclude whether prophylactic ND is necessary in all sMTC patients.

Critically evaluated key points on hereditary medullary thyroid carcinoma

“…In tumour tissue in which no RET mutation is found, a RAS mutation can often be detected. Detection of the somatic RET mutation has prognostic significance and is a prerequisite for treatment with selective RET tyrosine kinase inhibitors (LOXO-292, BLU-667) ( 15 ).…”

“…As there is no clear evidence of benefit in early stages of the disease, treatment with TKIs is currently indicated for high tumour burden and progressive (RECIST criteria, short tumour marker doubling time) as well as pronounced symptomatic disease when local treatment measures have been exhausted. To date, no TKI has been shown to prolong survival in metastasised MTC ( 4 , 15 , 28 ).…”