Primary Thymic Extranodal Marginal-Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Type Exhibits Distinctive Clinicopathological and Molecular Features
Extranodal marginal-zone B-cell lymphoma (MZBL) of mucosa-associated lymphoid tissue (MALT) arising in the thymus is rare, with the largest series in the literature including only three cases. In the present study, we investigated 15 cases of thymic MALT lymphoma to systematically characterize its c...
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| Veröffentlicht in: | The American journal of pathology 2002-04, Vol.160 (4), p.1435-1443 |
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| creator | Inagaki, Hiroshi Chan, John K.C. Ng, Josephine W.M. Okabe, Mitsukuni Yoshino, Tadashi Okamoto, Masataka Ogawa, Hiroshi Matsushita, Hiroshi Yokose, Tomoyuki Matsuno, Yoshihiro Nakamura, Naoya Nagasaka, Tetsuro Ueda, Ryuzo Eimoto, Tadaaki Nakamura, Shigeo |
| description | Extranodal marginal-zone B-cell lymphoma (MZBL) of mucosa-associated lymphoid tissue (MALT) arising in the thymus is rare, with the largest series in the literature including only three cases. In the present study, we investigated 15 cases of thymic MALT lymphoma to systematically characterize its clinical, histopathological, and molecular features. There was a marked female predilection (male:female = 1:4), with a mean age of 55 years at diagnosis. There was a strong association with autoimmune disease, especially Sjögren's syndrome. Histologically, the thymic lymphoma showed the characteristic morphological features of extranodal MZBL of MALT type. Cysts were common. Prominent lymphoepithelial lesions were formed by centrocyte-like cells infiltrating and expanding the Hassall's corpuscles and epithelium lining the cysts. Plasmacytic differentiation was apparent in all cases. Notably, 13 of 15 cases expressed immunoglobulin (Ig) A phenotype; IgA expression in thymic MALT lymphoma was in striking contrast with the IgM phenotype observed in most of the Sjögren's syndrome-associated MZBLs and MALT lymphomas at other sites. Epstein-Barr virus was absent, and
API2-MALT1 gene fusion, a recently reported MALT lymphoma-specific gene abnormality, was not detected in any case. Although one patient died of disease 85 months after the diagnosis, other patients were alive with overall 3-year and 5-year survival rates being 89% and 83%, respectively. Among the 22 patients reported previously and in the present series, at least 17 patients (77%) were Asians. These data indicate that thymic MALT lymphoma may represent a distinct subgroup of MALT lymphoma characterized by apparent predilection for Asians, a strong association with autoimmune disease, frequent presence of cysts, consistent plasma cell differentiation, tumor cells expressing IgA phenotype, and consistent lack of
API2-MALT1 gene fusion. |
| doi_str_mv | 10.1016/S0002-9440(10)62569-2 |
| format | Article |
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API2-MALT1 gene fusion, a recently reported MALT lymphoma-specific gene abnormality, was not detected in any case. Although one patient died of disease 85 months after the diagnosis, other patients were alive with overall 3-year and 5-year survival rates being 89% and 83%, respectively. Among the 22 patients reported previously and in the present series, at least 17 patients (77%) were Asians. These data indicate that thymic MALT lymphoma may represent a distinct subgroup of MALT lymphoma characterized by apparent predilection for Asians, a strong association with autoimmune disease, frequent presence of cysts, consistent plasma cell differentiation, tumor cells expressing IgA phenotype, and consistent lack of
API2-MALT1 gene fusion.</description><identifier>ISSN: 0002-9440</identifier><identifier>EISSN: 1525-2191</identifier><identifier>DOI: 10.1016/S0002-9440(10)62569-2</identifier><identifier>PMID: 11943727</identifier><identifier>CODEN: AJPAA4</identifier><language>eng</language><publisher>Bethesda, MD: Elsevier Inc</publisher><subject>Adult ; Aged ; Biological and medical sciences ; Female ; Hematologic and hematopoietic diseases ; Humans ; Immunohistochemistry ; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis ; Lymphoma, B-Cell, Marginal Zone - genetics ; Lymphoma, B-Cell, Marginal Zone - metabolism ; Lymphoma, B-Cell, Marginal Zone - pathology ; Male ; Medical sciences ; Middle Aged ; Regular ; Thymus Neoplasms - genetics ; Thymus Neoplasms - metabolism ; Thymus Neoplasms - pathology</subject><ispartof>The American journal of pathology, 2002-04, Vol.160 (4), p.1435-1443</ispartof><rights>2002 American Society for Investigative Pathology</rights><rights>2002 INIST-CNRS</rights><rights>Copyright American Society for Investigative Pathology Apr 2002</rights><rights>Copyright © 2002, American Society for Investigative Pathology</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c618t-105b5670cc780572e5a7f7e11301ba84188e272810b43098ffde2811081916933</citedby><cites>FETCH-LOGICAL-c618t-105b5670cc780572e5a7f7e11301ba84188e272810b43098ffde2811081916933</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC1867201/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://dx.doi.org/10.1016/S0002-9440(10)62569-2$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>230,314,727,780,784,885,3548,27922,27923,45993,53789,53791</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=13636254$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11943727$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Inagaki, Hiroshi</creatorcontrib><creatorcontrib>Chan, John K.C.</creatorcontrib><creatorcontrib>Ng, Josephine W.M.</creatorcontrib><creatorcontrib>Okabe, Mitsukuni</creatorcontrib><creatorcontrib>Yoshino, Tadashi</creatorcontrib><creatorcontrib>Okamoto, Masataka</creatorcontrib><creatorcontrib>Ogawa, Hiroshi</creatorcontrib><creatorcontrib>Matsushita, Hiroshi</creatorcontrib><creatorcontrib>Yokose, Tomoyuki</creatorcontrib><creatorcontrib>Matsuno, Yoshihiro</creatorcontrib><creatorcontrib>Nakamura, Naoya</creatorcontrib><creatorcontrib>Nagasaka, Tetsuro</creatorcontrib><creatorcontrib>Ueda, Ryuzo</creatorcontrib><creatorcontrib>Eimoto, Tadaaki</creatorcontrib><creatorcontrib>Nakamura, Shigeo</creatorcontrib><title>Primary Thymic Extranodal Marginal-Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Type Exhibits Distinctive Clinicopathological and Molecular Features</title><title>The American journal of pathology</title><addtitle>Am J Pathol</addtitle><description>Extranodal marginal-zone B-cell lymphoma (MZBL) of mucosa-associated lymphoid tissue (MALT) arising in the thymus is rare, with the largest series in the literature including only three cases. In the present study, we investigated 15 cases of thymic MALT lymphoma to systematically characterize its clinical, histopathological, and molecular features. There was a marked female predilection (male:female = 1:4), with a mean age of 55 years at diagnosis. There was a strong association with autoimmune disease, especially Sjögren's syndrome. Histologically, the thymic lymphoma showed the characteristic morphological features of extranodal MZBL of MALT type. Cysts were common. Prominent lymphoepithelial lesions were formed by centrocyte-like cells infiltrating and expanding the Hassall's corpuscles and epithelium lining the cysts. Plasmacytic differentiation was apparent in all cases. Notably, 13 of 15 cases expressed immunoglobulin (Ig) A phenotype; IgA expression in thymic MALT lymphoma was in striking contrast with the IgM phenotype observed in most of the Sjögren's syndrome-associated MZBLs and MALT lymphomas at other sites. Epstein-Barr virus was absent, and
API2-MALT1 gene fusion, a recently reported MALT lymphoma-specific gene abnormality, was not detected in any case. Although one patient died of disease 85 months after the diagnosis, other patients were alive with overall 3-year and 5-year survival rates being 89% and 83%, respectively. Among the 22 patients reported previously and in the present series, at least 17 patients (77%) were Asians. These data indicate that thymic MALT lymphoma may represent a distinct subgroup of MALT lymphoma characterized by apparent predilection for Asians, a strong association with autoimmune disease, frequent presence of cysts, consistent plasma cell differentiation, tumor cells expressing IgA phenotype, and consistent lack of
API2-MALT1 gene fusion.</description><subject>Adult</subject><subject>Aged</subject><subject>Biological and medical sciences</subject><subject>Female</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis</subject><subject>Lymphoma, B-Cell, Marginal Zone - genetics</subject><subject>Lymphoma, B-Cell, Marginal Zone - metabolism</subject><subject>Lymphoma, B-Cell, Marginal Zone - pathology</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Regular</subject><subject>Thymus Neoplasms - genetics</subject><subject>Thymus Neoplasms - metabolism</subject><subject>Thymus Neoplasms - pathology</subject><issn>0002-9440</issn><issn>1525-2191</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2002</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqFkk2P0zAQhiMEYpeFnwCykPg6BDzO9wW0lF1AagUS5cLFcp1JM5UTBzsp9OfwT3G31S5w4WSN55nX43cmih4Cfwkc8ldfOOcirtKUPwf-IhdZXsXiVnQKmchiARXcjk6vkZPonvebEOZJye9GJwBVmhSiOI1-fXbUKbdjy3bXkWYXP0enelsrwxbKralXJv5me2Rv4xkaw-a7bmhtp5ht2GLS1qv43HurSY1YH7NUsyV5PyFb7gYMki2taPTsHfmRej3SFtnMUE_aDmpsrbFr0uFB1ddsYQ3qySjHLlGNk0N_P7rTKOPxwfE8i75eXixnH-L5p_cfZ-fzWOdQjjHwbJXlBde6KHlWCMxU0RQIkHBYqTKFskRRiBL4Kk14VTZNjSECXgav8ipJzqLXB91hWnVYa-yDE0YOB3-kVST_zvTUyrXdSijzQnAIAk-PAs5-n9CPsiOvg2mqRzt5WUBWhQ724ON_wI2dXHDaSwFlVVU8qQKUHSDtrPcOm-tOgMv9BsirDZD78e6vrjZAilD36M9v3FQdRx6AJ0dA-eB6E8atyd9wSZ4ErTRwzw5cS-v2BzmUvlPGBFmQajNAzmUqIU2yQL45kBjGsyV00mvCXmMdqvQoa0v_afo38Ifcgw</recordid><startdate>20020401</startdate><enddate>20020401</enddate><creator>Inagaki, Hiroshi</creator><creator>Chan, John K.C.</creator><creator>Ng, Josephine W.M.</creator><creator>Okabe, Mitsukuni</creator><creator>Yoshino, Tadashi</creator><creator>Okamoto, Masataka</creator><creator>Ogawa, Hiroshi</creator><creator>Matsushita, Hiroshi</creator><creator>Yokose, Tomoyuki</creator><creator>Matsuno, Yoshihiro</creator><creator>Nakamura, Naoya</creator><creator>Nagasaka, Tetsuro</creator><creator>Ueda, Ryuzo</creator><creator>Eimoto, Tadaaki</creator><creator>Nakamura, Shigeo</creator><general>Elsevier Inc</general><general>ASIP</general><general>American Society for Investigative Pathology</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88I</scope><scope>8AF</scope><scope>8C1</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>LK8</scope><scope>M0S</scope><scope>M1P</scope><scope>M2P</scope><scope>M7P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>Q9U</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20020401</creationdate><title>Primary Thymic Extranodal Marginal-Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Type Exhibits Distinctive Clinicopathological and Molecular Features</title><author>Inagaki, Hiroshi ; Chan, John K.C. ; Ng, Josephine W.M. ; Okabe, Mitsukuni ; Yoshino, Tadashi ; Okamoto, Masataka ; Ogawa, Hiroshi ; Matsushita, Hiroshi ; Yokose, Tomoyuki ; Matsuno, Yoshihiro ; Nakamura, Naoya ; Nagasaka, Tetsuro ; Ueda, Ryuzo ; Eimoto, Tadaaki ; Nakamura, Shigeo</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c618t-105b5670cc780572e5a7f7e11301ba84188e272810b43098ffde2811081916933</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2002</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Biological and medical sciences</topic><topic>Female</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis</topic><topic>Lymphoma, B-Cell, Marginal Zone - genetics</topic><topic>Lymphoma, B-Cell, Marginal Zone - metabolism</topic><topic>Lymphoma, B-Cell, Marginal Zone - pathology</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Regular</topic><topic>Thymus Neoplasms - genetics</topic><topic>Thymus Neoplasms - metabolism</topic><topic>Thymus Neoplasms - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Inagaki, Hiroshi</creatorcontrib><creatorcontrib>Chan, John K.C.</creatorcontrib><creatorcontrib>Ng, Josephine W.M.</creatorcontrib><creatorcontrib>Okabe, Mitsukuni</creatorcontrib><creatorcontrib>Yoshino, Tadashi</creatorcontrib><creatorcontrib>Okamoto, Masataka</creatorcontrib><creatorcontrib>Ogawa, Hiroshi</creatorcontrib><creatorcontrib>Matsushita, Hiroshi</creatorcontrib><creatorcontrib>Yokose, Tomoyuki</creatorcontrib><creatorcontrib>Matsuno, Yoshihiro</creatorcontrib><creatorcontrib>Nakamura, Naoya</creatorcontrib><creatorcontrib>Nagasaka, Tetsuro</creatorcontrib><creatorcontrib>Ueda, Ryuzo</creatorcontrib><creatorcontrib>Eimoto, Tadaaki</creatorcontrib><creatorcontrib>Nakamura, Shigeo</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Science Database (Alumni Edition)</collection><collection>STEM Database</collection><collection>Public Health Database</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Natural Science Collection</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>ProQuest Biological Science Collection</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Science Database</collection><collection>Biological Science Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest Central Basic</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>The American journal of pathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Inagaki, Hiroshi</au><au>Chan, John K.C.</au><au>Ng, Josephine W.M.</au><au>Okabe, Mitsukuni</au><au>Yoshino, Tadashi</au><au>Okamoto, Masataka</au><au>Ogawa, Hiroshi</au><au>Matsushita, Hiroshi</au><au>Yokose, Tomoyuki</au><au>Matsuno, Yoshihiro</au><au>Nakamura, Naoya</au><au>Nagasaka, Tetsuro</au><au>Ueda, Ryuzo</au><au>Eimoto, Tadaaki</au><au>Nakamura, Shigeo</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Primary Thymic Extranodal Marginal-Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Type Exhibits Distinctive Clinicopathological and Molecular Features</atitle><jtitle>The American journal of pathology</jtitle><addtitle>Am J Pathol</addtitle><date>2002-04-01</date><risdate>2002</risdate><volume>160</volume><issue>4</issue><spage>1435</spage><epage>1443</epage><pages>1435-1443</pages><issn>0002-9440</issn><eissn>1525-2191</eissn><coden>AJPAA4</coden><abstract>Extranodal marginal-zone B-cell lymphoma (MZBL) of mucosa-associated lymphoid tissue (MALT) arising in the thymus is rare, with the largest series in the literature including only three cases. In the present study, we investigated 15 cases of thymic MALT lymphoma to systematically characterize its clinical, histopathological, and molecular features. There was a marked female predilection (male:female = 1:4), with a mean age of 55 years at diagnosis. There was a strong association with autoimmune disease, especially Sjögren's syndrome. Histologically, the thymic lymphoma showed the characteristic morphological features of extranodal MZBL of MALT type. Cysts were common. Prominent lymphoepithelial lesions were formed by centrocyte-like cells infiltrating and expanding the Hassall's corpuscles and epithelium lining the cysts. Plasmacytic differentiation was apparent in all cases. Notably, 13 of 15 cases expressed immunoglobulin (Ig) A phenotype; IgA expression in thymic MALT lymphoma was in striking contrast with the IgM phenotype observed in most of the Sjögren's syndrome-associated MZBLs and MALT lymphomas at other sites. Epstein-Barr virus was absent, and
API2-MALT1 gene fusion, a recently reported MALT lymphoma-specific gene abnormality, was not detected in any case. Although one patient died of disease 85 months after the diagnosis, other patients were alive with overall 3-year and 5-year survival rates being 89% and 83%, respectively. Among the 22 patients reported previously and in the present series, at least 17 patients (77%) were Asians. These data indicate that thymic MALT lymphoma may represent a distinct subgroup of MALT lymphoma characterized by apparent predilection for Asians, a strong association with autoimmune disease, frequent presence of cysts, consistent plasma cell differentiation, tumor cells expressing IgA phenotype, and consistent lack of
API2-MALT1 gene fusion.</abstract><cop>Bethesda, MD</cop><pub>Elsevier Inc</pub><pmid>11943727</pmid><doi>10.1016/S0002-9440(10)62569-2</doi><tpages>9</tpages><oa>free_for_read</oa></addata></record> |
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| source | MEDLINE; ScienceDirect Journals (5 years ago - present); EZB-FREE-00999 freely available EZB journals; PubMed Central |
| subjects | Adult Aged Biological and medical sciences Female Hematologic and hematopoietic diseases Humans Immunohistochemistry Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis Lymphoma, B-Cell, Marginal Zone - genetics Lymphoma, B-Cell, Marginal Zone - metabolism Lymphoma, B-Cell, Marginal Zone - pathology Male Medical sciences Middle Aged Regular Thymus Neoplasms - genetics Thymus Neoplasms - metabolism Thymus Neoplasms - pathology |
| title | Primary Thymic Extranodal Marginal-Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Type Exhibits Distinctive Clinicopathological and Molecular Features |
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