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Familial thyroid cancer
1Clinical Fellow-Endocrine Surgical Oncology, and Chief, Professor and Vice Chair, Department of Surgery, University of California, San Francisco/Mount Zion Medical Center, San Francisco, California, USA. alsaneao@surgery.scsf.edu
Current Opinion in Oncology
|January 10, 2001
Summary
Familial thyroid cancer has two types: medullary and nonmedullary. Genetic testing and timely surgery are key for managing familial medullary thyroid cancer (FMTC) and related syndromes.
Area of Science:
- Endocrinology
- Oncology
- Genetics
Background:
- Familial thyroid cancer presents as familial medullary thyroid cancer (FMTC) or familial nonmedullary thyroid cancer (FNMTC).
- FMTC can be isolated or part of multiple endocrine neoplasia (MEN) syndromes (types IIA and IIB).
- FNMTC is associated with papillary or Hurthle cell cancers and other genetic syndromes.
Purpose of the Study:
- To outline the genetic basis and management strategies for familial thyroid cancer.
- To differentiate between medullary and nonmedullary familial thyroid cancers.
- To emphasize the importance of genetic testing and surgical interventions.
Main Methods:
- Genetic testing for RET mutations in chromosome 10 for FMTC.
- Prophylactic total thyroidectomy in asymptomatic gene carriers.
- Surgical interventions including central neck dissection and modified radical neck dissection based on clinical presentation and tumor size.
Main Results:
- RET mutation testing identifies carriers for FMTC and MEN syndromes.
- Prophylactic surgery in gene carriers before age 6 (FMTC, MEN IIA) or after age 1 (MEN IIB) is recommended.
- Postoperative calcitonin levels indicate surgical success.
- FNMTC, while more aggressive than sporadic forms, is less aggressive than FMTC.
Conclusions:
- Early genetic identification and prophylactic thyroidectomy are crucial for managing FMTC and MEN syndromes.
- Total thyroidectomy, neck dissection, radioactive iodine ablation, and hormone suppression are effective for FNMTC.
- Awareness of ethical and lifestyle issues is vital for affected families.