Multiple endocrine neoplasia type 2 natural history: Difference between revisions
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==Natural History== | ==Natural History== | ||
Multiple endocrine neoplasia type 2 has a variable natural history. Specific c-''[[RET gene|RET]]'' [[mutation]]s, are associated to peculiar clinical [[phenotype]]s and thus to different courses of the [[disease]]. Multiple endocrine neoplasia type 2 can vary from a dormant type after the surgical removal of [[medullary thyroid carcinoma]] to a rapidly progressive one with [[metastatic]] complications resulting in death. | Multiple endocrine neoplasia type 2 has a [[variable]] natural history. Specific c-''[[RET gene|RET]]'' [[mutation]]s, are associated to peculiar [[clinical]] [[phenotype]]s and thus to different courses of the [[disease]]. Multiple endocrine neoplasia type 2 can vary from a dormant type after the [[Surgery|surgical]] removal of [[medullary thyroid carcinoma]] to a rapidly progressive one with [[metastatic]] [[Complication (medicine)|complications]] resulting in death.<ref name="MariniFalchetti2006">{{cite journal|last1=Marini|first1=Francesca|last2=Falchetti|first2=Alberto|last3=Del Monte|first3=Francesca|last4=Carbonell Sala|first4=Silvia|last5=Tognarini|first5=Isabella|last6=Luzi|first6=Ettore|last7=Brandi|first7=Maria|journal=Orphanet Journal of Rare Diseases|volume=1|issue=1|year=2006|pages=45|issn=17501172|doi=10.1186/1750-1172-1-45}}</ref> | ||
==Complications== | ==Complications== | ||
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==Prognosis== | ==Prognosis== | ||
* [[Prognosis]] of multiple endocrine neoplasia type 2 is mainly related to the stage-dependent [[prognosis]] of [[medullary thyroid cancer]] indicating the necessity of a complete [[Thyroidectomy|thyroid surgery]] for index cases with [[medullary thyroid cancer]] and the early [[thyroidectomy]] for screened at risk subjects. The 10 year survival of [[medullary thyroid cancer]] of multiple endocrine neoplasia type 2 is 61-76%. Multiple endocrine neoplasia type 2A has a better [[prognosis]] among the [[tumor]]s of [[multiple endocrine neoplasia]]. Specific c-''[[RET gene|RET]]'' [[mutations]], in fact, are associated to peculiar clinical [[phenotype]]s and thus to different course and [[prognosis]] of the [[disease]]. The following factors influencing survival rate of [[medullary thyroid carcinoma]]. | * [[Prognosis]] of multiple endocrine neoplasia type 2 is mainly related to the stage-dependent [[prognosis]] of [[medullary thyroid cancer]] indicating the necessity of a complete [[Thyroidectomy|thyroid surgery]] for index cases with [[medullary thyroid cancer]] and the early [[thyroidectomy]] for screened at risk subjects.<ref name="RaueFrank-Raue2018">{{cite journal|last1=Raue|first1=Friedhelm|last2=Frank-Raue|first2=Karin|title=Update on Multiple Endocrine Neoplasia Type 2: Focus on Medullary Thyroid Carcinoma|journal=Journal of the Endocrine Society|volume=2|issue=8|year=2018|pages=933–943|issn=2472-1972|doi=10.1210/js.2018-00178}}</ref> | ||
*The 10 year survival of [[medullary thyroid cancer]] of multiple endocrine neoplasia type 2 is 61-76%.<ref name="RaueFrank-Raue2015">{{cite journal|last1=Raue|first1=Friedhelm|last2=Frank-Raue|first2=Karin|title=Risk-stratified follow-up of patients with medullary thyroid carcinoma|journal=International Journal of Endocrine Oncology|volume=2|issue=4|year=2015|pages=249–252|issn=2045-0869|doi=10.2217/ije.15.23}}</ref> | |||
*Multiple endocrine neoplasia type 2A has a better [[prognosis]] among the [[tumor]]s of [[multiple endocrine neoplasia]]. Specific c-''[[RET gene|RET]]'' [[mutations]], in fact, are associated to peculiar clinical [[phenotype]]s and thus to different course and [[prognosis]] of the [[disease]]. The following factors influencing survival rate of [[medullary thyroid carcinoma]]. | |||
:* Stage of [[disease]] at [[diagnosis]] | :* Stage of [[disease]] at [[diagnosis]] | ||
:* Size of the [[tumor]] | :* Size of the [[tumor]] | ||
:* Age of the patient | :* Age of the [[patient]] | ||
:* Sex of the patient | :* Sex of the [[patient]] | ||
:* [[Calcitonin]] doubling time | :* [[Calcitonin]] doubling time | ||
* The following table depicts the stage dependent prognosis of medullary thyroid cancer. | * The following table depicts the stage dependent prognosis of medullary thyroid cancer.<ref>Thyroid cancer: Stage and prognosis. American association of endocrine surgeons (30.10.2015). http://endocrinediseases.org/thyroid/cancer_prognosis.shtml Accessed on October, 30, 2015</ref><ref name="RomanLin2006">{{cite journal|last1=Roman|first1=Sanziana|last2=Lin|first2=Rong|last3=Sosa|first3=Julie Ann|title=Prognosis of medullary thyroid carcinoma|journal=Cancer|volume=107|issue=9|year=2006|pages=2134–2142|issn=0008543X|doi=10.1002/cncr.22244}}</ref> | ||
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|+'''''Stage dependant prognosis of medullary thyroid cancer''''' | |+'''''Stage dependant prognosis of medullary thyroid cancer''''' | ||
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Latest revision as of 14:21, 26 June 2019
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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
Multiple endocrine neoplasia type 2 has a variable natural history. Life threatening complications of multiple endocrine neoplasia type 2 include malignant hypertension, megacolon, and metastasis. Prognosis of multiple endocrine neoplasia type 2 is mainly related to the stage-dependent prognosis of medullary thyroid cancer.
Natural History
Multiple endocrine neoplasia type 2 has a variable natural history. Specific c-RET mutations, are associated to peculiar clinical phenotypes and thus to different courses of the disease. Multiple endocrine neoplasia type 2 can vary from a dormant type after the surgical removal of medullary thyroid carcinoma to a rapidly progressive one with metastatic complications resulting in death.[1]
Complications
- Common complications of multiple endocrine neoplasia type 2 include the following:[2]
Systemic Complications
Endocrine Complications
Genitourinary System Complications
Gastrointestinal System Complications
- Obstruction
- Infection
- Stricture
- Fistula
- Constipation
- Fecal incontinence
- Diverticulum
- Gastrointestinal ganglioneuromatosis
- Megacolon
- Colitis
Prognosis
- Prognosis of multiple endocrine neoplasia type 2 is mainly related to the stage-dependent prognosis of medullary thyroid cancer indicating the necessity of a complete thyroid surgery for index cases with medullary thyroid cancer and the early thyroidectomy for screened at risk subjects.[3]
- The 10 year survival of medullary thyroid cancer of multiple endocrine neoplasia type 2 is 61-76%.[4]
- Multiple endocrine neoplasia type 2A has a better prognosis among the tumors of multiple endocrine neoplasia. Specific c-RET mutations, in fact, are associated to peculiar clinical phenotypes and thus to different course and prognosis of the disease. The following factors influencing survival rate of medullary thyroid carcinoma.
| Stage | Prognosis |
|---|---|
| Stage 1 | Best Prognosis and low chance of recurrence |
| Stage 2 | Good Prognosis and low chance of recurrence |
| Stage 3 | Bad Prognosis and high chance of recurrence |
| Stage 4 | Bad Prognosis and high chance of recurrence |
References
- ↑ Marini, Francesca; Falchetti, Alberto; Del Monte, Francesca; Carbonell Sala, Silvia; Tognarini, Isabella; Luzi, Ettore; Brandi, Maria (2006). Orphanet Journal of Rare Diseases. 1 (1): 45. doi:10.1186/1750-1172-1-45. ISSN 1750-1172. Missing or empty
|title=(help) - ↑ Cohen MS, Phay JE, Albinson C, DeBenedetti MK, Skinner MA, Lairmore TC; et al. (2002). "Gastrointestinal manifestations of multiple endocrine neoplasia type 2". Ann Surg. 235 (5): 648–54, discussion 654-5. PMC 1422490. PMID 11981210.
- ↑ Raue, Friedhelm; Frank-Raue, Karin (2018). "Update on Multiple Endocrine Neoplasia Type 2: Focus on Medullary Thyroid Carcinoma". Journal of the Endocrine Society. 2 (8): 933–943. doi:10.1210/js.2018-00178. ISSN 2472-1972.
- ↑ Raue, Friedhelm; Frank-Raue, Karin (2015). "Risk-stratified follow-up of patients with medullary thyroid carcinoma". International Journal of Endocrine Oncology. 2 (4): 249–252. doi:10.2217/ije.15.23. ISSN 2045-0869.
- ↑ Thyroid cancer: Stage and prognosis. American association of endocrine surgeons (30.10.2015). http://endocrinediseases.org/thyroid/cancer_prognosis.shtml Accessed on October, 30, 2015
- ↑ Roman, Sanziana; Lin, Rong; Sosa, Julie Ann (2006). "Prognosis of medullary thyroid carcinoma". Cancer. 107 (9): 2134–2142. doi:10.1002/cncr.22244. ISSN 0008-543X.