Clinical characteristics and prognosis of familial nonmedullary thyroid carcinoma
Joaquín de Carlos Artajo 1 , Ana Irigaray Echarri 2 , Javier García Torres 2 , José Javier Pineda Arribas 2 , Ander Ernaga Lorea 2 , Nerea Eguílaz Esparza 2 , Juan Manuel Zubiría Gortázar 3 , Emma Anda Apiñániz 2
Introduction: Familial non-medullary thyroid carcinoma (FNMTC) is defined by the presence of 2 or more first-degree family members with differentiated thyroid carcinoma (DTC). The aim of this study is to compare clinicopathological features and prognosis of FNMTC and sporadic carcinoma (SC).
Materials and methods: Retrospective study of DTC included in the hospital database during the period 1990-2018.
Results: A total of 927 patients were analyzed, 61 of them were FNMTC, with a mean follow-up of 9.7 ± 6.5 years. The prevalence of FNMTC was 6.6%, with a lower TNM staging presentation (P = 0.003) consequence of a higher proportion of tumors smaller than 2 cm (P = 0.003), combined with a greater multifocality (P = 0.034) and papillary histologic subtype (P = 0.022) compared to SC. No significant differences in age at diagnosis (P = 0.347), gender (P = 0.406), neither in other aggressiveness markers (bilaterality, extrathyroidal extension, lymph node involvement and metástasis) were detected. Rate of persistence/recurrence (P = 0.656), disease-free survival (P = 0.929) and mortality caused by the tumor itself (P = 0.666) were comparable. Families with ≥3 affected relatives, had smaller tumors (P = 0.005), more multifocality (P = 0.040) and bilaterality (P = 0.002), as well as a higher proportion of males (P = 0.020). Second generation patients present earlier FNMTC compared to those of the first generation (P = 0.001).
Conclusion: In our study FNMTC presents a lower TNM staging, higher multifocality and papillary variant, with similar aggressiveness and prognosis compared to SC.
CITA DEL ARTÍCULO Endocrinol Diabetes Nutr (Engl Ed). 2022 Apr;69(4):262-270. doi: 10.1016/j.endien.2022.03.003. Epub 2022 Mar 16.