Multiple endocrine neoplasia type 2 natural history
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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.
- The following table depicts the stage dependent prognosis of medullary thyroid cancer.[5]
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
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(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