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Primary Histiocytic Sarcoma of the Spleen Associated with Erythrophagocytic Histiocytosis
We report an exceptional case of a histiocytic sarcoma presenting as a primary isolated spleen tumor in a 71-year-old woman. The neoplastic cells in the cords and sinuses of the red pulp formed multiple lobulated tumors, which were detected in vivo by ultrasound scan. The medium cells, large cells a...
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Published in: | Pathology, research and practice research and practice, 2003, Vol.199 (2), p.107-112 |
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description | We report an exceptional case of a histiocytic sarcoma presenting as a primary isolated spleen tumor in a 71-year-old woman. The neoplastic cells in the cords and sinuses of the red pulp formed multiple lobulated tumors, which were detected
in vivo by ultrasound scan. The medium cells, large cells and the giant cells expressed CD68, a histiocyte-associated marker, lysozyme and S100 protein. All these cells were negative for B- and T-cell markers, cytokeratins, melanosome markers (HMB45) and CD1a (Langerhans' cells). Many tumor cells displayed strong erythrophagocytosis and sometimes lymphocytophagocytosis. In addition, numerous histiocytes with morphology indistinguishable from reactive macrophages also exhibited a strong erythro-phagocytosis, and were found in the tumor as well as in the normal splenic parenchyma. Despite multi-agent chemotherapy, the patient suffered from a relapse in the liver, with a rapid fatal outcome. A literature review showed that such a primary splenic presentation with multiple tumors is rare. In contrast, in systemic malignant histiocytosis, secondary spleen involvement occurs more frequently but with diffuse infiltration. The association with a reactive histiocytosis with erythrophagocytosis corresponds to “histiocytic medullary reticulosis”, as previously described by Scott and Robb-Smith. |
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in vivo by ultrasound scan. The medium cells, large cells and the giant cells expressed CD68, a histiocyte-associated marker, lysozyme and S100 protein. All these cells were negative for B- and T-cell markers, cytokeratins, melanosome markers (HMB45) and CD1a (Langerhans' cells). Many tumor cells displayed strong erythrophagocytosis and sometimes lymphocytophagocytosis. In addition, numerous histiocytes with morphology indistinguishable from reactive macrophages also exhibited a strong erythro-phagocytosis, and were found in the tumor as well as in the normal splenic parenchyma. Despite multi-agent chemotherapy, the patient suffered from a relapse in the liver, with a rapid fatal outcome. A literature review showed that such a primary splenic presentation with multiple tumors is rare. In contrast, in systemic malignant histiocytosis, secondary spleen involvement occurs more frequently but with diffuse infiltration. The association with a reactive histiocytosis with erythrophagocytosis corresponds to “histiocytic medullary reticulosis”, as previously described by Scott and Robb-Smith.</description><identifier>ISSN: 0344-0338</identifier><identifier>EISSN: 1618-0631</identifier><identifier>DOI: 10.1078/0344-0338-00362</identifier><identifier>PMID: 12747473</identifier><language>eng</language><publisher>Germany: Elsevier GmbH</publisher><subject>Aged ; Biomarkers, Tumor ; Diagnosis, Differential ; Erythrophagocytic histiocytosis ; Female ; Histiocytic sarcoma ; Histiocytic Sarcoma - complications ; Histiocytic Sarcoma - metabolism ; Histiocytic Sarcoma - pathology ; Humans ; Immunohistochemistry ; Inflammation - metabolism ; Inflammation - pathology ; Liver Neoplasms - secondary ; Lymph Nodes - pathology ; Malignant histiocytosis ; Primary splenic sarcoma ; Sarcoma - complications ; Sarcoma - metabolism ; Sarcoma - pathology ; Sarcoma - secondary ; Splenic Neoplasms - complications ; Splenic Neoplasms - metabolism ; Splenic Neoplasms - pathology</subject><ispartof>Pathology, research and practice, 2003, Vol.199 (2), p.107-112</ispartof><rights>2003 Urban & Fischer Verlag</rights><rights>Copyright Urban & Fischer Verlag 2003</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c370t-5bfe60d69fc721e7da79ff5fd8a9ee1f2de9a411e52ca56dbc5c2a99a00415ca3</citedby><cites>FETCH-LOGICAL-c370t-5bfe60d69fc721e7da79ff5fd8a9ee1f2de9a411e52ca56dbc5c2a99a00415ca3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,4024,27923,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/12747473$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Audouin, Josée</creatorcontrib><creatorcontrib>Vercelli-Retta, J.</creatorcontrib><creatorcontrib>Le Tourneau, A.</creatorcontrib><creatorcontrib>Adida, C.</creatorcontrib><creatorcontrib>Camilleri-Broët, S.</creatorcontrib><creatorcontrib>Molina, T.</creatorcontrib><creatorcontrib>Diebold, J.</creatorcontrib><title>Primary Histiocytic Sarcoma of the Spleen Associated with Erythrophagocytic Histiocytosis</title><title>Pathology, research and practice</title><addtitle>Pathol Res Pract</addtitle><description>We report an exceptional case of a histiocytic sarcoma presenting as a primary isolated spleen tumor in a 71-year-old woman. The neoplastic cells in the cords and sinuses of the red pulp formed multiple lobulated tumors, which were detected
in vivo by ultrasound scan. The medium cells, large cells and the giant cells expressed CD68, a histiocyte-associated marker, lysozyme and S100 protein. All these cells were negative for B- and T-cell markers, cytokeratins, melanosome markers (HMB45) and CD1a (Langerhans' cells). Many tumor cells displayed strong erythrophagocytosis and sometimes lymphocytophagocytosis. In addition, numerous histiocytes with morphology indistinguishable from reactive macrophages also exhibited a strong erythro-phagocytosis, and were found in the tumor as well as in the normal splenic parenchyma. Despite multi-agent chemotherapy, the patient suffered from a relapse in the liver, with a rapid fatal outcome. A literature review showed that such a primary splenic presentation with multiple tumors is rare. In contrast, in systemic malignant histiocytosis, secondary spleen involvement occurs more frequently but with diffuse infiltration. The association with a reactive histiocytosis with erythrophagocytosis corresponds to “histiocytic medullary reticulosis”, as previously described by Scott and Robb-Smith.</description><subject>Aged</subject><subject>Biomarkers, Tumor</subject><subject>Diagnosis, Differential</subject><subject>Erythrophagocytic histiocytosis</subject><subject>Female</subject><subject>Histiocytic sarcoma</subject><subject>Histiocytic Sarcoma - complications</subject><subject>Histiocytic Sarcoma - metabolism</subject><subject>Histiocytic Sarcoma - pathology</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Inflammation - metabolism</subject><subject>Inflammation - pathology</subject><subject>Liver Neoplasms - secondary</subject><subject>Lymph Nodes - pathology</subject><subject>Malignant histiocytosis</subject><subject>Primary splenic sarcoma</subject><subject>Sarcoma - complications</subject><subject>Sarcoma - metabolism</subject><subject>Sarcoma - pathology</subject><subject>Sarcoma - 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complications</topic><topic>Histiocytic Sarcoma - metabolism</topic><topic>Histiocytic Sarcoma - pathology</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>Inflammation - metabolism</topic><topic>Inflammation - pathology</topic><topic>Liver Neoplasms - secondary</topic><topic>Lymph Nodes - pathology</topic><topic>Malignant histiocytosis</topic><topic>Primary splenic sarcoma</topic><topic>Sarcoma - complications</topic><topic>Sarcoma - metabolism</topic><topic>Sarcoma - pathology</topic><topic>Sarcoma - secondary</topic><topic>Splenic Neoplasms - complications</topic><topic>Splenic Neoplasms - metabolism</topic><topic>Splenic Neoplasms - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Audouin, Josée</creatorcontrib><creatorcontrib>Vercelli-Retta, J.</creatorcontrib><creatorcontrib>Le Tourneau, A.</creatorcontrib><creatorcontrib>Adida, C.</creatorcontrib><creatorcontrib>Camilleri-Broët, S.</creatorcontrib><creatorcontrib>Molina, T.</creatorcontrib><creatorcontrib>Diebold, J.</creatorcontrib><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>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>Research Library (Alumni Edition)</collection><collection>ProQuest Central (Alumni)</collection><collection>ProQuest Central</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>eLibrary</collection><collection>AUTh Library subscriptions: ProQuest Central</collection><collection>ProQuest 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>Research Library Prep</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>PML(ProQuest Medical Library)</collection><collection>Proquest Research Library</collection><collection>Science Database</collection><collection>Biological Science Database</collection><collection>Research Library (Corporate)</collection><collection>Research Library China</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 Basic</collection><collection>SIRS Editorial</collection><collection>MEDLINE - Academic</collection><jtitle>Pathology, research and practice</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Audouin, Josée</au><au>Vercelli-Retta, J.</au><au>Le Tourneau, A.</au><au>Adida, C.</au><au>Camilleri-Broët, S.</au><au>Molina, T.</au><au>Diebold, J.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Primary Histiocytic Sarcoma of the Spleen Associated with Erythrophagocytic Histiocytosis</atitle><jtitle>Pathology, research and practice</jtitle><addtitle>Pathol Res Pract</addtitle><date>2003</date><risdate>2003</risdate><volume>199</volume><issue>2</issue><spage>107</spage><epage>112</epage><pages>107-112</pages><issn>0344-0338</issn><eissn>1618-0631</eissn><abstract>We report an exceptional case of a histiocytic sarcoma presenting as a primary isolated spleen tumor in a 71-year-old woman. The neoplastic cells in the cords and sinuses of the red pulp formed multiple lobulated tumors, which were detected
in vivo by ultrasound scan. The medium cells, large cells and the giant cells expressed CD68, a histiocyte-associated marker, lysozyme and S100 protein. All these cells were negative for B- and T-cell markers, cytokeratins, melanosome markers (HMB45) and CD1a (Langerhans' cells). Many tumor cells displayed strong erythrophagocytosis and sometimes lymphocytophagocytosis. In addition, numerous histiocytes with morphology indistinguishable from reactive macrophages also exhibited a strong erythro-phagocytosis, and were found in the tumor as well as in the normal splenic parenchyma. Despite multi-agent chemotherapy, the patient suffered from a relapse in the liver, with a rapid fatal outcome. A literature review showed that such a primary splenic presentation with multiple tumors is rare. In contrast, in systemic malignant histiocytosis, secondary spleen involvement occurs more frequently but with diffuse infiltration. The association with a reactive histiocytosis with erythrophagocytosis corresponds to “histiocytic medullary reticulosis”, as previously described by Scott and Robb-Smith.</abstract><cop>Germany</cop><pub>Elsevier GmbH</pub><pmid>12747473</pmid><doi>10.1078/0344-0338-00362</doi><tpages>6</tpages></addata></record> |
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subjects | Aged Biomarkers, Tumor Diagnosis, Differential Erythrophagocytic histiocytosis Female Histiocytic sarcoma Histiocytic Sarcoma - complications Histiocytic Sarcoma - metabolism Histiocytic Sarcoma - pathology Humans Immunohistochemistry Inflammation - metabolism Inflammation - pathology Liver Neoplasms - secondary Lymph Nodes - pathology Malignant histiocytosis Primary splenic sarcoma Sarcoma - complications Sarcoma - metabolism Sarcoma - pathology Sarcoma - secondary Splenic Neoplasms - complications Splenic Neoplasms - metabolism Splenic Neoplasms - pathology |
title | Primary Histiocytic Sarcoma of the Spleen Associated with Erythrophagocytic Histiocytosis |
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