ESPE Abstracts (2019) 92 RFC5.1

Hurthle Cell Carcinoma in Childhood: Retrospective Analysis of a Large Series

Giorgia Pepe1, Mariella Valenzise 1, Giuseppina Zirilli1, Laura Cannavò1, Gerdi Tuli2, Andrea Corrias2, Malgorzata Wasniewska1


1Department of Human Pathology of Adulthood and Childhood, University of Messina, Messina, Italy. 2Department of Pediatrics, Regina Margherita Children's Hospital, Turin, Italy


Background: in general population Hurthle cell cancer (HCC) accounts for 3-7% of all differentiated thyroid cancers (TC) with a more aggressive course, while its relative prevalence and behavior in childhood is uncertain due to the lack of specific literature reviews.

Objective: to describe the largest pediatric HCC cohort to date reported and to estimate its relative prevalence among TC variants in childhood.

Methods: study population included 5 patients <19 years, who were diagnosed with HCC during the period 2000-2018 in our Departments. Histologic diagnosis of HCC was based on the finding of at least 75% of Hurthle cells at post-surgical analysis. HCC course was retrospectively reconstructed with data recorded at diagnosis, at surgical resection and during a follow-up period ranging from 6.5 to 15 years. Patients' assessment included: clinical findings, thyroid function and autoimmunity tests, neck and chest imaging (ultrasound and computed tomography scan), cytologic and histologic analyses of the tumor.

Results: HCC occurred with a relative prevalence of 5.8% (5 of 86 young patients affected by TC, diagnosed in the same period and institutions), at a median chronological age of 12.5 years. All patients were biochemically euthyroid at HCC diagnosis and underwent both total thyroidectomy with central neck dissection and radioiodine therapy, with subsequent L-T4 thyroidal suppression. Low or intermediate risk level was observed at diagnosis, since none of our patients exhibited extensive lateral neck disease or distant metastases and all of them showed a persistent clinical, biochemical and imaging remission over time. Antecedents of other diseases were recorded in 3 patients (Hashimoto's thyroiditis, Neurofibromatosis type 1 and osteosarcoma respectively).

Conclusions: 1) in childhood the relative prevalence of HCC among TC histotypes is 5.8%, that is close to the one reported from literature both in general population (3-5.7%) and in young patients (2-7%); 2) HCC may develop even very early, at an age of 7 years; 3) in pediatric age HCC does not seem to have a more aggressive behavior than other TC histotypes; 4) antecedents of other diseases are not infrequent in the history of children with HCC.

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