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M Capezzone, Endocrinology, University of Siena, Siena, Italy
S Marchisotta, Endocrinology, University of Siena, Siena, Italy
S Cantara, Endocrinology, University of Siena, Siena, Italy
G Busonero, Endocrinology, University of Siena, Siena, Italy
L Brilli, Endocrinology, University of Siena, Siena, Italy
K Pazaitou-Panayiotou, Endocrine oncology, University of Tessaloniki, Tessaloniki, Greece
A Carli, Endocrine surgery, University of Siena, Siena, Italy
G Caruso, Otorinolaringoiatry, University of Siena, Siena, Italy
P Toti, Human Pathology and Oncology, University of Siena, Siena, Italy
S Capitani, Surgery, University of Siena, Siena, Italy
A Pammolli, Surgery, University of Siena, Siena, Italy
F Pacini, Endocrinology, University of Siena, Siena, Italy

Correspondence: Furio Pacini, Email: pacini8{at}unisi.it

Abstract

Non-medullary thyroid carcinoma (NMTC) is mostly sporadic, but familial clustering is described. We aimed to compare the features of patients with sporadic and familial NMTC (FNMTC) patients and to assess whether FNMTC patients with parent-child relationship exhibit the anticipation phenomenon (earlier age at disease onset and increased severity in successive generations). Among 300 NMTCs followed in the Section of Endocrinology (University of Siena, Italy), 34 (11.3 %) patients, all with the papillary histotype, (16 kindred), met the criteria of FNMTC. Twenty seven of them (79.4 %) exhibited a parent-child relationship and 7 (20.6 %) a sibling relationship. These patients were compared to 235 sporadic PTCs. To analyze the features of FNMTC of the 1st and 2nd generation, we cumulated the series of Siena with 32 additional FNMTC patients (15 kindred) from the Department of Endocrinology-Endocrine Oncology, Thessaloniki, Greece. Significant difference between sporadic PTC and FNMTC patients included more frequent tumour multifocality (P=0.001) and worse final outcome in FNMTC patients (P=0.001). Among 47 FNMTC with parent-child relationship, we found an earlier age at disease presentation (P<0.0001), at diagnosis (P<0.0001) and at disease onset (P=0.04) in the 2nd generation compared to the 1st generation. Patients in the 2nd generation were more frequently males (P=0.02); their tumours were more frequently multifocal (P=0.003), and bilateral (P=0.01), had higher rate of lymph node metastases at surgery (P=0.02) and worse outcome (P=0.04) compared to the 1st generation. In conclusion, FNMTC displays the features of clinical anticipation, with the 2nd generation acquiring the disease at an earlier age and having more advanced disease at presentation.