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| DOI | 10.4067/S0370-41062014000300012 | ||||
| Año | 2014 | ||||
| Tipo | reporte de caso |
Citas Totales
Autores Afiliación Chile
Instituciones Chile
% Participación
Internacional
Autores
Afiliación Extranjera
Instituciones
Extranjeras
Introduction: Papillary thyroid cancer (PTC) is the most common endocrine malignancy, representing 80% of all thyroid cancers; only 10% of cases are manifested before age 21 and have an estimated incidence of 0.54 cases per 100,000 people. In children it is diagnosed at a more advanced stage of the disease but with good therapeutic response and very low mortality. Objective: To present four family cases with PTC, discuss the particular characteristics and the importance of early diagnosis. Case reports: 4 families with members affected by family papillary thyroid carcinoma are presented, all pediatric cases were manifested after a direct member adult case was diagnosed, therefore pediatric patients were early detected, but despite a timely treatment, the disease was advanced at the time of diagnosis. The pediatric cases are 3 females and 1 male with an average age of 12 ½ years old at diagnosis. Discussion: The family variety of papillary thyroid carcinoma (2 or more direct members affected), represents 5% of papillary cancers. It is transmitted through autosomal dominant inheritance with incomplete penetrance and variable expressivity. It occurs at a younger age than the sporadic type, and it is more aggressive with greater local invasion (32%), recurrence (20-50%) and lymphatic metastases (57 %), and it is associated with benign thyroid diseases and often, it is multifocal. Conclusion: The family papillary thyroid cancer is a disease with worse prognosis than the sporadic variety; therefore, a high index of suspicion is required in affected families for early diagnosis and treatment.
| Ord. | Autor | Género | Institución - País |
|---|---|---|---|
| 1 | FERRADA C, CLARITA | Mujer |
Pontificia Universidad Católica de Chile - Chile
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| 2 | GODOY C, CLAUDIA | Mujer |
Pontificia Universidad Católica de Chile - Chile
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| 3 | Martinez-Aguayo, Alejandro G. | Hombre |
Pontificia Universidad Católica de Chile - Chile
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| 4 | GARCIA-LOVAZZANO, HERNAN | Hombre |
Pontificia Universidad Católica de Chile - Chile
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