Abstract

PurposePrimary thyroid lymphoma (PTL) is a rare malignancy, and the literature is limited to small case series and case reports. This study aimed to assess the epidemiologic characteristics, survival, and prognostic factors of patients with PTL.MethodsWe analyzed 2215 PTL patients from the Surveillance, Epidemiology, and End Results database medical records, between 1983 and 2015, as the training cohort. We enrolled 105 patients from the Cancer Hospital, Chinese Academy of Medical Sciences, for the external validation cohort. The nomograms for predicting the 1-, 5-, and 10-year overall survival (OS) and lymphoma-specific survival (LSS) were constructed.ResultsPTL incidence steadily increased from 1977 to 1994, with an annual percentage change of 3.2% (95% confidence interval [CI]: 1.2–5.2, P < 0.05). The 1-, 5-, and 10-year OS and LSS rates were 84.66%, 71.61%, and 55.95%; and 90.5%, 85.7%, and 82.2%, respectively. Multivariate Cox regression analysis revealed that shorter OS association with age ≥ 60 years (hazard ratio [HR], 3.94; 95% CI 3.31–4.69; P < 0.001), unmarried status (HR, 1.55; 95% CI 1.37–1.75; P < 0.001), Ann Arbor stage III-IV (HR, 1.55; 95% CI 1.37–1.75; P = 0.020), diffuse large B-cell lymphoma (HR, 2.60; 95% CI 1.15–5.87; P = 0.022), and T cell non–Hodgkin lymphoma (HR, 3.53; 95% CI 1.12–11.10; P = 0.031). In the multivariate competing-risk analyzes, age, stages III-IV, year of diagnosis, surgery, radiation, chemotherapy, and histology were strongly predictive of PTL-specific risk of death. To estimate the 1-, 5-, and 10-year LSS and OS rates, respectively, nomograms were built. In the validation cohort, the results also confirmed the utility.ConclusionsThis study presents the first prognostic model with an external validation that could help clinicians identify patients with high-risk PTL to improve their prognosis.

Highlights

  • Primary thyroid lymphoma (PTL) is an uncommon malignancy that arises in the thyroid gland with or without the presence of regional lymph nodes in the neck

  • We identified 2,215 PTL patients as a training cohort from the SEER database between 1983 and 2015

  • From 1977 to 1994, we found a steadily rising incidence with an annual percentage changes (APCs) of 3.2% (Supplemental file Fig. 2A)

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Summary

Introduction

Primary thyroid lymphoma (PTL) is an uncommon malignancy that arises in the thyroid gland with or without the presence of regional lymph nodes in the neck. This definition excludes lymphomas from other sites of the body that invade the thyroid gland. Owing to the size of the sample used to conduct immunohistochemistry for its precise subtype validation, more tissue is needed for diagnosing PTL than for diagnosing other thyroid tumors [6,7,8]. The prevalent histology of PTL is non-Hodgkin’s B-cell lymphoma and includes diffuse large B-cell lymphoma (DLBCL), mucosa-associated lymphoid tissue lymphoma (MALT), and mixed MALT and DLBCL [9].

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