Extranodal natural killer/T-cell lymphoma of the breast: a retrospective clinicopathological analysis of a consecutive 11-year case series
Abstract Background Extranodal NK/T-cell lymphoma of the breast (ENKTL-Breast) is rarely detected in clinical practice, and its clinicopathological features remain unclear. Results A consecutive 11-year (2010–2020) ENKTL-Breast case series was retrospectively analyzed. Eight cases of ENKTL-Breast we...
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oai:doaj.org-article:241c8e423a8e48e89686a128b0103b382021-11-21T12:26:01ZExtranodal natural killer/T-cell lymphoma of the breast: a retrospective clinicopathological analysis of a consecutive 11-year case series10.1186/s13023-021-02110-x1750-1172https://doaj.org/article/241c8e423a8e48e89686a128b0103b382021-11-01T00:00:00Zhttps://doi.org/10.1186/s13023-021-02110-xhttps://doaj.org/toc/1750-1172Abstract Background Extranodal NK/T-cell lymphoma of the breast (ENKTL-Breast) is rarely detected in clinical practice, and its clinicopathological features remain unclear. Results A consecutive 11-year (2010–2020) ENKTL-Breast case series was retrospectively analyzed. Eight cases of ENKTL-Breast were selected, with three primary and five secondary lesions, accounting for 3.5% (8/228) of all breast lymphomas. All patients were female with a median age of 46 years. Lesions presented as solid breast masses (8/8, 100%) and were usually located in the upper outer quadrant of the breast (7/8, 87.5%). B-symptoms were observed in seven (7/8, 87.5%) cases. Two primary ENKTL-Breast cases showed concomitant diseases (IgA nephropathy and chronic active hepatitis B). Histological and immunohistochemical features of ENKTL-Breast were similar to those of ENKTL at other sites. T-cell receptor rearrangement revealed clonality in all examined primary cases (2/2, 100%), but only in one secondary case (1/5, 20%). The disease progressed rapidly in two primary cases and both patients died within 3 and 9 months. For secondary cases, the disease manifested as a disseminated disease, with a median survival time of 6 months. Conclusions Our data suggested that ENKTL-Breast clinically mimics breast cancer to some extent, though B-symptoms might serve as a distinguishing factor. ENKTL-Breast is highly aggressive and patients with this disease exhibit a short survival time. Primary ENKTL-Breast tends to originate from activated cytotoxic T-cells, and immune-related diseases may be involved in its pathogenesis and development.Wei LiuZihang ChenFanglan LiWenyan ZhangWeiping LiuSha ZhaoBMCarticleExtranodal natural killer/T-cell lymphomaBreast lymphomaT-cell lymphomaEpstein–Barr virusMedicineRENOrphanet Journal of Rare Diseases, Vol 16, Iss 1, Pp 1-11 (2021) |
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Extranodal natural killer/T-cell lymphoma Breast lymphoma T-cell lymphoma Epstein–Barr virus Medicine R |
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Extranodal natural killer/T-cell lymphoma Breast lymphoma T-cell lymphoma Epstein–Barr virus Medicine R Wei Liu Zihang Chen Fanglan Li Wenyan Zhang Weiping Liu Sha Zhao Extranodal natural killer/T-cell lymphoma of the breast: a retrospective clinicopathological analysis of a consecutive 11-year case series |
description |
Abstract Background Extranodal NK/T-cell lymphoma of the breast (ENKTL-Breast) is rarely detected in clinical practice, and its clinicopathological features remain unclear. Results A consecutive 11-year (2010–2020) ENKTL-Breast case series was retrospectively analyzed. Eight cases of ENKTL-Breast were selected, with three primary and five secondary lesions, accounting for 3.5% (8/228) of all breast lymphomas. All patients were female with a median age of 46 years. Lesions presented as solid breast masses (8/8, 100%) and were usually located in the upper outer quadrant of the breast (7/8, 87.5%). B-symptoms were observed in seven (7/8, 87.5%) cases. Two primary ENKTL-Breast cases showed concomitant diseases (IgA nephropathy and chronic active hepatitis B). Histological and immunohistochemical features of ENKTL-Breast were similar to those of ENKTL at other sites. T-cell receptor rearrangement revealed clonality in all examined primary cases (2/2, 100%), but only in one secondary case (1/5, 20%). The disease progressed rapidly in two primary cases and both patients died within 3 and 9 months. For secondary cases, the disease manifested as a disseminated disease, with a median survival time of 6 months. Conclusions Our data suggested that ENKTL-Breast clinically mimics breast cancer to some extent, though B-symptoms might serve as a distinguishing factor. ENKTL-Breast is highly aggressive and patients with this disease exhibit a short survival time. Primary ENKTL-Breast tends to originate from activated cytotoxic T-cells, and immune-related diseases may be involved in its pathogenesis and development. |
format |
article |
author |
Wei Liu Zihang Chen Fanglan Li Wenyan Zhang Weiping Liu Sha Zhao |
author_facet |
Wei Liu Zihang Chen Fanglan Li Wenyan Zhang Weiping Liu Sha Zhao |
author_sort |
Wei Liu |
title |
Extranodal natural killer/T-cell lymphoma of the breast: a retrospective clinicopathological analysis of a consecutive 11-year case series |
title_short |
Extranodal natural killer/T-cell lymphoma of the breast: a retrospective clinicopathological analysis of a consecutive 11-year case series |
title_full |
Extranodal natural killer/T-cell lymphoma of the breast: a retrospective clinicopathological analysis of a consecutive 11-year case series |
title_fullStr |
Extranodal natural killer/T-cell lymphoma of the breast: a retrospective clinicopathological analysis of a consecutive 11-year case series |
title_full_unstemmed |
Extranodal natural killer/T-cell lymphoma of the breast: a retrospective clinicopathological analysis of a consecutive 11-year case series |
title_sort |
extranodal natural killer/t-cell lymphoma of the breast: a retrospective clinicopathological analysis of a consecutive 11-year case series |
publisher |
BMC |
publishDate |
2021 |
url |
https://doaj.org/article/241c8e423a8e48e89686a128b0103b38 |
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