{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,7]],"date-time":"2026-02-07T09:29:12Z","timestamp":1770456552880,"version":"3.49.0"},"reference-count":23,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,7,22]],"date-time":"2021-07-22T00:00:00Z","timestamp":1626912000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Reports"],"published-print":{"date-parts":[[2022,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Histiocytic sarcoma is a very rare monocyte\/macrophage\u2010derived hematopoietic system tumor with a poor prognosis whose diagnosis is pathologically challenging due to its extreme rarity and histological overlap with various mimicking entities in which histiocytes also predominate.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case<\/jats:title><jats:p>We report the case of a 33\u2010year\u2010old male patient with hemophagocytic lymphohistiocytosis, purpuric syndrome, and significant splenomegaly. The patient underwent splenectomy; subsequent macroscopic examination revealed a spleen weighing 2065 grams with hyperemic red pulp and multiple infarcts at the periphery. The histological and immunohistochemical study established a diagnosis of primary splenic histiocytic sarcoma with frequent hemophagocytosis. Next\u2010generation sequencing demonstrated mutations in <jats:italic>FLT3, NOTCH2<\/jats:italic>, and <jats:italic>KMT2A,<\/jats:italic> microsatellite stability, and a tumor mutational burden of 2 mut\/Mb. The patient's condition deteriorated clinically from the appearance of the first symptoms and he died 6 months later from multi\u2010organ failure.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Primary splenic histiocytic sarcoma is one of the rarest tumors of the hematopoietic system. We report the first case with mutations in <jats:italic>FLT3, NOTCH2<\/jats:italic>, and <jats:italic>KMT2A,<\/jats:italic> and associated hemophagocytic lymphohistiocytosis.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cnr2.1496","type":"journal-article","created":{"date-parts":[[2021,7,22]],"date-time":"2021-07-22T15:53:47Z","timestamp":1626969227000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Primary splenic histiocytic sarcoma associated with hemophagocytic lymphohistiocytosis: A case report and review of literature of <scp>next\u2010generation<\/scp> sequencing involving <scp><i>FLT3<\/i><\/scp>, <scp><i>NOTCH2<\/i><\/scp>, and <scp><i>KMT2A<\/i><\/scp> mutations"],"prefix":"10.1002","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2342-9120","authenticated-orcid":false,"given":"Nelson","family":"Montalvo","sequence":"first","affiliation":[{"name":"Facultad de Ciencias M\u00e9dicas de la Salud y la Vida, Escuela de Medicina, Departamento de Docencia e Investigaci\u00f3n Universidad Internacional del Ecuador, Av. Sim\u00f3n Bol\u00edvar y Jorge Fern\u00e1ndez  Quito Ecuador"},{"name":"Servicio de Patolog\u00eda Hospital Metropolitano, Av. Mariana de Jes\u00fas s\/n y Nicol\u00e1s Arteta  Quito Ecuador"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jorge","family":"Lara\u2010Endara","sequence":"additional","affiliation":[{"name":"Facultad de Ciencias M\u00e9dicas de la Salud y la Vida, Escuela de Medicina, Departamento de Docencia e Investigaci\u00f3n Universidad Internacional del Ecuador, Av. Sim\u00f3n Bol\u00edvar y Jorge Fern\u00e1ndez  Quito Ecuador"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ligia","family":"Redrob\u00e1n","sequence":"additional","affiliation":[{"name":"Servicio de Patolog\u00eda Hospital Metropolitano, Av. 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Sim\u00f3n Bol\u00edvar y Jorge Fern\u00e1ndez  Quito Ecuador"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,7,22]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-01-643569"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-10-812495"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-01-690636"},{"key":"e_1_2_11_5_1","volume-title":"World Health Organization Classification of Tumours","author":"Swerdlow SH","year":"2008"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2019.230375"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-12-893917"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/1476-4598-9-292"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/jlb.65.3.372"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/ni.1615"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-02-410241"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13000-019-0804-6"},{"issue":"2","key":"e_1_2_11_13_1","first-page":"279","article-title":"Precision medicine for human cancers with notch signaling dysregulation (review)","volume":"45","author":"Katoh M","year":"2020","journal-title":"Int J Mol Med"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/82_2012_235"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1006193"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/apm.12906"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.284109.116"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2018-0349-RS"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1186\/1746-1596-7-143"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1078\/0344-0338-00362"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0344-0338(88)80165-1"},{"key":"e_1_2_11_22_1","first-page":"271","volume-title":"Diagnostic Pathology of Hematopoietic Disorders of Spleen and Liver","author":"Tuzuner N","year":"2020"},{"key":"e_1_2_11_23_1","first-page":"356","article-title":"A case report and review of the literature","volume":"39","author":"Paik JH","year":"2005","journal-title":"Korean J Pathol"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2016.05.001"}],"container-title":["Cancer Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cnr2.1496","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/cnr2.1496","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cnr2.1496","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,25]],"date-time":"2023-08-25T07:22:48Z","timestamp":1692948168000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/cnr2.1496"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,7,22]]},"references-count":23,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2022,5]]}},"alternative-id":["10.1002\/cnr2.1496"],"URL":"https:\/\/doi.org\/10.1002\/cnr2.1496","archive":["Portico"],"relation":{},"ISSN":["2573-8348","2573-8348"],"issn-type":[{"value":"2573-8348","type":"print"},{"value":"2573-8348","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,7,22]]},"assertion":[{"value":"2021-04-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-06-17","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-07-22","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e1496"}}