Anaplastic thyroid carcinoma: a nationwide cohort study on incidence, treatment and survival in the Netherlands over 3 decades

Abstract

<jats:sec> <jats:title>Objective</jats:title> <jats:p>To perform a nationwide population based study in ATC on incidence, treatment and survival.</jats:p> </jats:sec> <jats:sec> <jats:title>Design</jats:title> <jats:p>Retrospective cohort study.</jats:p> </jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>All patients with primary ATC between 1989 and 2016 were identified in the Netherlands Cancer Registry (NCR). Of all these patients excerpts from the pathology reports from PALGA: Dutch Pathology registry were linked to the data of the NCR. Standardized incidences were calculated, survival was estimated using Kaplan–Meier method and univariable statistically significant factors were included in a multivariable regression model.</jats:p> </jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>In total, 812 patients were included. Mean standardized incidence rates were 0.18/100 000 (range 0.11–0.27/100 000) with a significant trend over the years with an estimated annual percentage change of 1.3% per year (95% CI 0.4–2.1%). Median overall survival was 2.2 months, and estimated 1-year survival was 12%. Patients without distant metastases at diagnosis had an estimated 1-year survival of 21.6%. Prognostic factors for prolonged survival were double or triple therapy, age below 65 years, M0-status and absence of bilateral lymph node metastases.</jats:p> </jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>ATC is rare, but often lethal, form of thyroid cancer, with a median survival of 2 months and 1-year survival of approximately 10%. The incidence is slightly rising in the Netherlands over the past 3 decades. There appears to be a subgroup of patients that survive longer, mainly those with relatively limited disease who underwent double or triple therapy. Further research is needed to define these patients more distinctively.</jats:p> </jats:sec>

Anaplastic Thyroid Carcinoma: An Update

“…ATC is a rare cancer as defined by the European Union rare cancer surveillance program (RARECARE) with an incidence far below six new cases per 100,000 person-years [ 15 ]. Indeed, recent epidemiological studies have confirmed an age-adjusted incidence in the US of 0.12 per 100,000 person-years (95% CI: 0.8–1.6) in 2014 in the Surveillance, Epidemiology, and End Results (SEER) database [ 16 ] and 0.1 to 0.3 per 100,000 person-years in Europe according to data from Denmark [ 4 ], Wales [ 17 ] and the Netherlands [ 18 ] registries. The SEER database and the Netherlands registries analyses have suggested an increase in the age-adjusted incidence with an average annual percent change of 3.0% per year (95% CI: 2.2–3.7%) and 1.3% per year (95% CI: 0.4–2.1%), respectively, over a 30–40-year period of time; this increase in incidence was not reported in the Danish and the Welsh databases [ 4 , 16 , 17 , 18 ].…”

Combination therapy of tyrosine kinase inhibitor sorafenib with the HSP90 inhibitor onalespib as a novel treatment regimen for thyroid cancer

“…However, the outcomes for ATC patients are less favorable as the disease is notoriously di cult to treat 6 . The median survival of ATC patients is less than six months post diagnosis and the 5-year overall survival rate falls below 10% 7 . In cases where the primary tumor is successfully resected, patients often present with metastasized disease either at diagnosis or following initial treatment 8 .…”

Complete pathological response following chemotherapy and radiotherapy in two cases of advanced anaplastic thyroid carcinoma

“…Anaplastic thyroid carcinoma (ATC) accounts for the highest short-term mortality rate of all thyroid cancer, with almost 40–50% of thyroid cancer deaths directly related to ATC and a historical median overall survival (OS) estimated between 2 and 6 months. However, slightly better outcomes have been reported in recent years through increased knowledge of the disease’s molecular landscape and immune microenvironment, as well as the emergence of targeted therapies/immunotherapy ( 1 , 2 , 3 , 4 ). Studying long-term cancer survivors is also a good way to improve the care of the patients.…”