Abstract
BackgroundPrimary thyroid Burkitt’s lymphoma (BL) is an extremely rare and highly aggressive form of non-Hodgkin’s lymphoma; only isolated case reports are available for patients with this disease.MethodsWe analyzed the clinicopathological features of thyroid BL by conducting a meta-analysis of 21 known patients (including ours) and compared them to those of extrathyroidal BL.ResultsThere were 13 men and 8 women with a median age of 39.3 years (range, 6–75 years). The median follow-up was 46.5 months (range, 0.5–361 months). Six patients (28.6%) had stage I disease, 2 (9.5%) had stage II, 2 (9.5%) had stage III, and 11 (52.4%) had stage IV. Five of 7 tested patients with thyroid BL (71.4%) had histological evidence of underlying Hashimoto’s thyroiditis. Ki-67 labeling indices exceeding 90% in all 19 patients tested (100%). Fluorescence in situ hybridization performed on 12 patient samples revealed that all (100%) had MYC rearrangement. Among the 16 patients for whom follow-up data were available, 4 died of disease-related causes. Kaplan-Meier analysis revealed that the 12- and 60-month overall survival rates for patients with thyroid BL were 87.5 and 70.7%, respectively.ConclusionsOurs was the largest study of thyroid BL and its detailed clinicopathological features to date. Thyroid BL is not associated with underlying Epstein-Barr virus infection but is closely linked to Hashimoto’s thyroiditis; patients generally have good overall survival and respond well to intensive chemotherapy. The correct pathological diagnosis is essential for treatment selection and outcome improvement.
Highlights
Primary thyroid Burkitt’s lymphoma (BL) is an extremely rare and highly aggressive form of nonHodgkin’s lymphoma; only isolated case reports are available for patients with this disease
We report a new patient with primary thyroid BL to help further characterize the clinicopathological and genetic features of this disease
Research design We reviewed 454 patients diagnosed with primary thyroid lymphoma at Kuma Hospital, Kagawa University, and Takamatsu Red Cross Hospital between 1996 and 2015
Summary
Primary thyroid Burkitt’s lymphoma (BL) is an extremely rare and highly aggressive form of nonHodgkin’s lymphoma; only isolated case reports are available for patients with this disease. Primary thyroid Burkitt’s lymphoma (BL) is a rare and highly aggressive form of non-Hodgkin’s lymphoma and comprises 1–2% of thyroid lymphomas [1]. This neoplasm is characterized by intermediate-sized lymphoid cells with a “starry sky” appearance and exhibits chromosomal translocations that activate the MYC oncogene [2, 3]. We report a new patient with primary thyroid BL to help further characterize the clinicopathological and genetic features of this disease. We performed a meta-analysis of all 21 patients known to date and compared their characteristics to those of patients with extrathyroidal BL
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