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Results of a screening program for multiple endocrine neoplasia type II
Surgery, Gynecology & Obstetrics
|August 1, 1984
Summary
Early screening for Multiple Endocrine Neoplasia type IIa (MEN IIa) is crucial. Continuous surveillance of all family members, even after treatment, is vital for reducing mortality from medullary thyroid carcinoma and pheochromocytoma.
Area of Science:
- Endocrinology
- Genetics
- Oncology
Background:
- Multiple Endocrine Neoplasia type IIa (MEN IIa) is a hereditary condition.
- Early diagnosis and intervention are critical for managing MEN IIa.
Purpose of the Study:
- To evaluate the effectiveness of a screening program for MEN IIa in a large British family.
- To assess methods for early detection of medullary thyroid carcinoma (MCT) and pheochromocytoma.
Main Methods:
- Screening of 180 family members using plasma calcitonin and urinary catecholamine tests.
- Analysis of screening results and subsequent diagnostic procedures.
Main Results:
- Abnormal screening tests were identified in 14 individuals.
- Medullary thyroid carcinoma was confirmed in two patients, and pheochromocytoma in one.
- Persistent elevation of calcitonin levels post-thyroidectomy indicated residual C cells and ongoing risk.
Conclusions:
- Variable plasma calcitonin levels necessitate established normal ranges to prevent unnecessary surgery.
- Pheochromocytoma diagnosis remains challenging; pentagastrin-stimulated catecholamines warrant further evaluation.
- Continuous, lifelong screening of all family members is essential for reducing mortality and managing MEN IIa effectively.
