Multiple endocrine neoplasia type 2 surgery: Difference between revisions
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[[File:Medullary thyroid cancer.png|thumb|center|500px|ESMO clinical practice guidelines for treatment of medullary cell carcinoma]] | [[File:Medullary thyroid cancer.png|thumb|center|500px|ESMO clinical practice guidelines for treatment of medullary cell carcinoma]] | ||
====Post Surgery==== | ====Post Surgery==== | ||
* Thyroid should supplemented be supplemented for patients undergoing total thyroidectomy. | * Thyroid should supplemented be supplemented for patients undergoing total thyroidectomy. | ||
* Serum calcitonin and CEA DT are measured during post surgical follow-up. | * Serum calcitonin and CEA DT are measured during post surgical follow-up. | ||
Revision as of 17:00, 23 September 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
Surgery
Prophylactic or Precocious Thyroidectomy in RET Gene Carrier
- Prophylactic thyroidectomy is advised in gene carriers to guarantee a definitive cure in these subjects. Four different risk levels (from A, the lowest, to D the highest) for RET mutations have been suggested by the American Thyroid Association task force, which developed the most recent guidelines for the management of MTC patients.[1] According to these guidelines, these levels of risk, which are related to the clinical aggressiveness of the corresponding MTC, should be taken into consideration when planning surgical treatment. In particular patients with a level D, RET mutation (i.e., Met918Thr) should be treated as soon as possible in the first year of life; patients with level B and C mutations (located in exons 10, 11, 13, 14, and 15) should be operated on before 5 years of age; only for patients with a level A mutation (exon 8 and 5 mutations), total thyroidectomy can be delayed after five years of age or until the CT positivity.
- Recently, some evidences in big series of RET gene carriers demonstrated that gene carriers with undetectable levels of basal CT have an almost null risk to have already developed the MTC.[2][3] Moreover, a serum Ct <30–40 pg/mL is always associated to an intrathyroidal micro-MTC without any evidence of lymph node metastases. Taking into account these observation, Elisei et al. [4] designed a study in which they operated on only RET gene carriers on the basis of basal and stimulated CT. According to their results, the time of surgical treatment could be personalized and safely planned when the stimulated serum CT becomes positive at the annual control, independently from the type of RET mutation and its associated level of risk. Of course, both cysteine RET mutations and older age are risk factors for having an earlier positive result for either basal or Pg-stimulated serum CT. For these reasons, 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 theRET gene carriers to the scheduled followup with the advantage that young children can be treated later, sometime even after the puberty, close to the adulthood.
Post Surgery
- Thyroid should supplemented be supplemented for patients undergoing total thyroidectomy.
- Serum calcitonin and 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, USG neck is recommended.
- If the basal serum calcitonin is above 150 pg/ml, screening for distant metastasis is recommeneded.
Reference
- ↑ American Thyroid Association Guidelines Task Force. Kloos RT, Eng C, Evans DB, Francis GL, Gagel RF; et al. (2009). "Medullary thyroid cancer: management guidelines of the American Thyroid Association". Thyroid. 19 (6): 565–612. doi:10.1089/thy.2008.0403. PMID 19469690.
- ↑ Lau GS, Lang BH, Lo CY, Tso A, Garcia-Barcelo MM, Tam PK; et al. (2009). "Prophylactic thyroidectomy in ethnic Chinese patients with multiple endocrine neoplasia type 2A syndrome after the introduction of genetic testing". Hong Kong Med J. 15 (5): 326–31. PMID 19801688.
- ↑ Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID http://dx.doi.org/10.1210/jc.2010-1234 Check
|pmid=value (help). - ↑ Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID http://dx.doi.org/10.1210/jc.2011-2046 Check
|pmid=value (help).