Multiple endocrine neoplasia type 2 natural history

	Multiple endocrine neoplasia type 2 Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Multiple endocrine neoplasia type 2 from other Diseases
Epidemiology & Demographics
Risk Factors
Screening
Natural History, Complications & Prognosis
Diagnosis
Diagnostic Criteria
History & Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X Ray
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Multiple endocrine neoplasia type 2 natural history On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Multiple endocrine neoplasia type 2 natural history All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Multiple endocrine neoplasia type 2 natural history
CDC on Multiple endocrine neoplasia type 2 natural history
Multiple endocrine neoplasia type 2 natural history in the news
Blogs on Multiple endocrine neoplasia type 2 natural history
Directions to Hospitals Treating Multiple endocrine neoplasia type 2
Risk calculators and risk factors for Multiple endocrine neoplasia type 2 natural history

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.

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]

Common complications of multiple endocrine neoplasia type 2 include the following:^[2]

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.

The following table depicts the stage dependent prognosis of medullary thyroid cancer.^[5]^[6]

**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

↑ 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.