M Capezzone1, C Secchi1, N Fralassi1, S Cantara1, L Brilli1, C Ciuoli1, T Pilli1, F Maino1, R Forleo1, F Pacini1, M G Castagna2. 1. Section of Endocrinology and Metabolism, Department of Medical, Surgical and Neurological Sciences, University of Siena, Policlinico Santa Maria alle Scotte, Viale Bracci 1, 53100, Siena, Italy. 2. Section of Endocrinology and Metabolism, Department of Medical, Surgical and Neurological Sciences, University of Siena, Policlinico Santa Maria alle Scotte, Viale Bracci 1, 53100, Siena, Italy. m.g.castagna@ao-siena.toscana.it.
Abstract
PURPOSE: An increased aggressiveness of familial papillary thyroid carcinoma (FPTC) compared with sporadic form has been reported. On the contrary, the biological behavior of familial microPTC (FmPTC) is still debated. To assess if familial diseases should be considered as a negative prognostic factor in mPTC, the clinical presentation and outcome of FmPTC and sporadic mPTC (SmPTC) were compared. METHODS: We retrospectively analyzed 291 mPTC (SmPTC n = 248, FmPTC n = 43) patients followed for a median follow-up of 8.3 years. FmPTC was defined as the presence of PTC in two or more first-degree relatives, after excluding hereditary syndromes associated with PTC. RESULTS: FmPTC patients had more frequently bilateral tumor (32.6% versus 16.5%, p = 0.01) and lymph node metastases at diagnosis (30.2% versus 14.9%, p = 0.02). At the first follow-up, FmPTC patients had a higher rate of structural disease and a lower rate of remission compared to SmPTC (p = 0.01). Also in a multivariate model, using a "CHAID tree-building algorithm", familial disease correlated with a worse clinical presentation and outcome of mPTC patients. Familial disease was associated with a higher rate of intermediate risk patients in non incidental mPTC and with a higher rate of structural incomplete response in mPTC without lymph node metastases (p = 0.01). CONCLUSIONS: Like in macroPTC, the familial form of the diseases has been shown to be a negative prognostic factor also in mPTC, therefore, it should be highly regarded in the management of mPTC patients.
PURPOSE: An increased aggressiveness of familial papillary thyroid carcinoma (FPTC) compared with sporadic form has been reported. On the contrary, the biological behavior of familial microPTC (FmPTC) is still debated. To assess if familial diseases should be considered as a negative prognostic factor in mPTC, the clinical presentation and outcome of FmPTC and sporadic mPTC (SmPTC) were compared. METHODS: We retrospectively analyzed 291 mPTC (SmPTC n = 248, FmPTC n = 43) patients followed for a median follow-up of 8.3 years. FmPTC was defined as the presence of PTC in two or more first-degree relatives, after excluding hereditary syndromes associated with PTC. RESULTS: FmPTC patients had more frequently bilateral tumor (32.6% versus 16.5%, p = 0.01) and lymph node metastases at diagnosis (30.2% versus 14.9%, p = 0.02). At the first follow-up, FmPTC patients had a higher rate of structural disease and a lower rate of remission compared to SmPTC (p = 0.01). Also in a multivariate model, using a "CHAID tree-building algorithm", familial disease correlated with a worse clinical presentation and outcome of mPTCpatients. Familial disease was associated with a higher rate of intermediate risk patients in non incidental mPTC and with a higher rate of structural incomplete response in mPTC without lymph node metastases (p = 0.01). CONCLUSIONS: Like in macroPTC, the familial form of the diseases has been shown to be a negative prognostic factor also in mPTC, therefore, it should be highly regarded in the management of mPTCpatients.
Authors: A Ríos; M A Rodríguez; J A Puñal; P Moreno; E Mercader; E Ferrero; J Ruiz-Pardo; M A Morlán; J Martín; M Durán-Poveda; J M Bravo; D Casanova; M P Salvador Egea; N M Torregrosa; A Exposito-Rodríguez; G Martínez-Fernández; A M Carrión; O Vidal; F Herrera; G Ruiz-Merino; J M Rodríguez Journal: Langenbecks Arch Surg Date: 2022-10-17 Impact factor: 2.895