Medullary thyroid cancer surgery: Difference between revisions
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{{CMG}}; {{AE}} {{Ammu}} | {{CMG}}; {{AE}} {{Ammu}} | ||
==Overview== | ==Overview== | ||
[[Surgery]] is the mainstay of treatment for medullary thyroid carcinoma. | |||
==Surgery== | |||
* [[Surgery]] is the mainstay of treatment for medullary thyroid carcinoma.<ref name="urlwww.nccn.org">{{cite web |url=https://www.nccn.org/professionals/physician_gls/pdf/thyroid.pdf |title=www.nccn.org |format= |work= |accessdate=}}</ref> | |||
==Indications== | |||
* In case of [[tumors]] < 1 cm in diameter or unilateral thyroid disease, total [[thyroidectomy]] with [[neck]] dissection (level VI) is indicated.<ref name="urlwww.nccn.org">{{cite web |url=https://www.nccn.org/professionals/physician_gls/pdf/thyroid.pdf |title=www.nccn.org |format= |work= |accessdate=}}</ref> | |||
* In case of [[tumors]] ≥ 1 cm in diameter or bilateral thyroid disease, total [[thyroidectomy]] with bilateral central neck dissection (level VI) is indicated. | |||
====Conventional Therapy==== | ====Conventional Therapy==== | ||
* The treatment of choice for primary [[medullary thyroid carcinoma]], both sporadic or hereditary, is total [[thyroidectomy]] with systematic dissection of all [[lymph node]]s 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.<ref name="pmid17665245">{{cite journal| author=Machens A, Hauptmann S, Dralle H| title=Increased risk of lymph node metastasis in multifocal hereditary and sporadic medullary thyroid cancer. | journal=World J Surg | year= 2007 | volume= 31 | issue= 10 | pages= 1960-5 | pmid=17665245 | doi=10.1007/s00268-007-9185-1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17665245 }}</ref><ref name="pmid6128962">{{cite journal| author=Russell CF, Van Heerden JA, Sizemore GW, Edis AJ, Taylor WF, ReMine WH et al.| title=The surgical management of medullary thyroid carcinoma. | journal=Ann Surg | year= 1983 | volume= 197 | issue= 1 | pages= 42-8 | pmid=6128962 | doi= | pmc=PMC1352852 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6128962 }} </ref> [[Lymph node]] dissection of laterocervical compartments is | * The treatment of choice for primary [[medullary thyroid carcinoma]], both sporadic or hereditary, is total [[thyroidectomy]] with systematic dissection of all [[lymph node]]s 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.<ref name="pmid17665245">{{cite journal| author=Machens A, Hauptmann S, Dralle H| title=Increased risk of lymph node metastasis in multifocal hereditary and sporadic medullary thyroid cancer. | journal=World J Surg | year= 2007 | volume= 31 | issue= 10 | pages= 1960-5 | pmid=17665245 | doi=10.1007/s00268-007-9185-1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17665245 }}</ref><ref name="pmid6128962">{{cite journal| author=Russell CF, Van Heerden JA, Sizemore GW, Edis AJ, Taylor WF, ReMine WH et al.| title=The surgical management of medullary thyroid carcinoma. | journal=Ann Surg | year= 1983 | volume= 197 | issue= 1 | pages= 42-8 | pmid=6128962 | doi= | pmc=PMC1352852 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6128962 }} </ref> [[Lymph node]] dissection of laterocervical compartments is reserved till the neck [[ultrasound]] suggests the presence of metastatic nodes. | ||
[[File:Diagram showing after surgery for medullary thyroid cancer with the central lymph nodes and the thyroid gland removed CRUK 092.png|thumb|center|500px|ESMO clinical practice guidelines for treatment of medullary cell carcinoma]] | |||
====Prophylactic or Precocious Thyroidectomy in RET Gene Carrier==== | ====Prophylactic or Precocious Thyroidectomy in RET Gene Carrier==== | ||
* Prophylactic [[thyroidectomy]] is advised in [[gene]] carriers to guarantee a definitive cure in these subjects. | * 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 | * 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. | ||
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center | {| style="border: 0px; font-size: 90%; margin: 3px;" align=center | ||
|+'''''Prophylactic Thyroidectomy''''' | |+'''''Prophylactic Thyroidectomy''''' | ||
| Line 34: | Line 38: | ||
! style="background: #F5F5F5;" | Operated at a later stage | ! style="background: #F5F5F5;" | 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. | * The following flowchart depicts the surgical management of medullary thyroid cancer. | ||
[[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]] | ||
| Line 46: | Line 50: | ||
* The following flowchart depicts the post surgical management of medullary thyroid cancer. | * The following flowchart depicts the post surgical management of medullary thyroid cancer. | ||
[[File:Post surgical follow up MEN 2.png|thumb|center|500px|ESMO clinical practice guidelines for treatment of medullary cell carcinoma]] | [[File:Post surgical follow up MEN 2.png|thumb|center|500px|ESMO clinical practice guidelines for treatment of medullary cell carcinoma]] | ||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
[[Category:Endocrine system]] | [[Category:Endocrine system]] | ||
[[Category:Genetic disorders]] | |||
[[Category:Endocrinology]][[Category:Endocrine system]] | |||
[[Category:Genetic disorders]] | [[Category:Genetic disorders]] | ||
[[Category:Endocrinology]] | [[Category:Endocrinology]] | ||
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Latest revision as of 20:59, 20 August 2019
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Medullary thyroid cancer Microchapters |
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Differentiating Medullary thyroid cancer from other Diseases |
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Medullary thyroid cancer surgery On the Web |
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American Roentgen Ray Society Images of Medullary thyroid cancer surgery |
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Risk calculators and risk factors for Medullary thyroid cancer surgery |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
Surgery is the mainstay of treatment for medullary thyroid carcinoma.
Surgery
Indications
- In case of tumors < 1 cm in diameter or unilateral thyroid disease, total thyroidectomy with neck dissection (level VI) is indicated.[1]
- In case of tumors ≥ 1 cm in diameter or bilateral thyroid disease, total thyroidectomy with bilateral central neck dissection (level VI) is indicated.
Conventional Therapy
- 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.[2][3] 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.
| Gene | Risk | Treatment |
|---|---|---|
| Children with MEN2B and/or c-RET codon 883, 918,
922 |
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
mutations |
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.
Post Surgery
- Thyroxine should be supplemented for patients undergoing total thyroidectomy.[4]
- 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.
References
- ↑ 1.0 1.1 "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.