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Spontaneous Murine Model of Anaplastic Thyroid Cancer
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Pediatric Medullary Thyroid Carcinoma.

Dmytro Starenki1, Jong-In Park1

  • 1Department of Biochemistry, Medical College of Wisconsin, Milwaukee, WI 53226, USA.

Journal of Pediatric Oncology
|March 26, 2016
PubMed
Summary
This summary is machine-generated.

Medullary thyroid carcinoma (MTC) is a rare cancer originating from thyroid C cells. Most pediatric MTC cases are hereditary, linked to RET proto-oncogene mutations, and often require early surgical intervention.

Keywords:
MEN2AMEN2BMedullary thyroid carcinomaRET

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Area of Science:

  • Oncology
  • Genetics
  • Endocrinology

Background:

  • Medullary thyroid carcinoma (MTC) originates from thyroid parafollicular C cells, representing 3-5% of thyroid malignancies.
  • MTC can be sporadic or inherited, often as part of Multiple Endocrine Neoplasia (MEN) syndromes.
  • A strong genotype-phenotype correlation exists between hereditary MTC and germline RET proto-oncogene mutations.

Purpose of the Study:

  • To summarize the characteristics, diagnosis, and treatment of Medullary Thyroid Carcinoma (MTC).
  • To highlight the genetic basis and management strategies for hereditary MTC, particularly in pediatric cases.
  • To discuss current therapeutic options for advanced MTC.

Main Methods:

  • Review of existing literature on Medullary Thyroid Carcinoma.
  • Analysis of genotype-phenotype correlations in hereditary MTC.
  • Summary of standard and advanced treatment modalities for MTC.

Main Results:

  • Most pediatric MTC cases are hereditary, linked to RET mutations, and diagnosed during familial genetic investigations.
  • Standard treatment involves total thyroidectomy and central neck node dissection.
  • Prophylactic thyroidectomy is recommended for presymptomatic individuals with hereditary MTC.
  • Targeted therapies like vandetanib and cabozantinib are used for advanced MTC but have variable efficacy.

Conclusions:

  • Medullary thyroid carcinoma, especially in children, is frequently hereditary and associated with RET mutations.
  • Early diagnosis and intervention, including prophylactic surgery, are crucial for managing hereditary MTC.
  • Treatment for advanced MTC relies on tyrosine kinase inhibitors, necessitating ongoing research for improved therapies.