Multiple endocrine neoplasia type 2 natural history: Difference between revisions

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Revision as of 14:17, 26 June 2019

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

Kidney stones

Gastrointestinal System Complications

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 of disease at diagnosis
Size of the tumor
Age of the patient
Sex of the patient
Calcitonin doubling time

The following table depicts the stage dependent prognosis of medullary thyroid cancer.^[5]

**Stage dependant prognosis of medullary thyroid cancer**
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

Template:WikiDoc Sources

[MariniFalchetti2006-1] 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)

[pmid11981210-2] 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.

[RaueFrank-Raue2018-3] 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.

[RaueFrank-Raue2015-4] 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.

[5] 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

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@@ Line 34: / Line 34: @@
 ==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.<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%. 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]].
+*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]]