Medullary thyroid cancer surgery
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Surgery is the mainstay of treatment for medullary thyroid carcinoma.
- In case of tumors < 1 cm in diameter or unilateral thyroid disease, total thyroidectomy with neck dissection (level VI) is indicated.
- In case of tumors ≥ 1 cm in diameter or bilateral thyroid disease, total thyroidectomy with bilateral central neck dissection (level VI) is indicated.
- The treatment of choice for primary medullary thyroid carcinoma, both sporadic or hereditary, is total thyroidectomy with systematic dissection of all lymph nodes of the central compartment. Total thyroidectomy is necessary as medullary thyroid carcinoma is multicentric in 65–90% of patients in multiple endocrine neoplasia type 2 and extensive central lymph node dissection has been reported to improve survival and recurrence rates compared to less aggressive procedures. Lymph node dissection of laterocervical compartments is reserved till the neck ultrasound suggests the presence of metastatic nodes.
Prophylactic or Precocious Thyroidectomy in RET Gene Carrier
- Prophylactic thyroidectomy is advised in gene carriers to guarantee a definitive cure in these subjects.
- During the Seventh International Multiple Endocrine Neoplasia Meeting in Gubbio in 1999, the risk of medullary thyroid carcinoma has been stratified in three categories according to the mutations of c-RET as following.
|Children with MEN2B and/or c-RET codon 883, 918,
|Highest risk of aggressive medullary thyroid carcinoma||Total thyroidectomy with
central node dissection, within the first six months.
|Children with any c-RET codon 611, 618, 620 or 634
|High risk of medullary thyroid carcinoma||Total thyroidectomy should be performed before age of
five years, with or without central node dissection.
|Children with c-RET codon 609, 768, 790, 791, 804
and 891 mutations
|Less aggressive and slowly growing medullary thyroid carcinoma||Operated at a later stage|
- The time of surgical treatment could be personalized and safely planned when the stimulated serum calcitonin becomes positive at the annual control, independently from the type of RET mutation and its associated level of risk. The follow-up controls should be more or less frequent in cysteine or noncysteine RET-mutated gene carriers, respectively. This strategy obviously implies a high compliance of the RET gene carriers to the scheduled follow-up with the advantage that young children can be treated later, sometime even after the puberty, close to the adulthood.
- The following flowchart depicts the surgical management of medullary thyroid cancer.
- Thyroxine should be supplemented for patients undergoing total thyroidectomy.
- Serum calcitonin and carcinoembryonic antigen doubling time (CEA DT) are measured during post surgical follow-up.
- Provacative pentagastrin or calcium test is administered and serum calcitonin level is measured.
- If there is no significant elevation in serum calcitonin level, serum calcitonin is measured every 6 months for 2-3 years and then yearly.
- If the calcitonin is below 150 pg/ml, ultrasound neck is recommended.
- If the basal serum calcitonin is above 150 pg/ml, screening for distant metastasis is recommended.
- The following flowchart depicts the post surgical management of medullary thyroid cancer.
- "www.nccn.org" (PDF).
- Machens A, Hauptmann S, Dralle H (2007). "Increased risk of lymph node metastasis in multifocal hereditary and sporadic medullary thyroid cancer". World J Surg. 31 (10): 1960–5. doi:10.1007/s00268-007-9185-1. PMID 17665245.
- Russell CF, Van Heerden JA, Sizemore GW, Edis AJ, Taylor WF, ReMine WH; et al. (1983). "The surgical management of medullary thyroid carcinoma". Ann Surg. 197 (1): 42–8. PMC 1352852. PMID 6128962.
- Pacini F, Castagna MG, Brilli L, Pentheroudakis G, ESMO Guidelines Working Group (2012). "Thyroid cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up". Ann Oncol. 23 Suppl 7: vii110–9. doi:10.1093/annonc/mds230. PMID 22997443.