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Abdominal manifestations of RDD are extremely rare.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case presentation<\/jats:title><jats:p>In August 2018, a 42-year-old man underwent an abdominal\u00a0ultrasonography\u00a0examination due to his weight loss of 10\u00a0kg in only three months and found a giant solid tumor was found in his spleen. Then, he was admitted to our hospital and diagnosed as a splenic mass via abdominal enhanced CT and MRI. Laparoscopic splenectomy was administrated within six days of admission due to the clear surgical indications. The pathogenesis of RDD remained poorly understood and the disease should be diagnosed based on histopathology and immunohistochemistry (IHC). The mutations in<jats:italic>ATM<\/jats:italic>and<jats:italic>NFKBIA<\/jats:italic>were observed using next generation sequencing (NGS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We reported a case of splenic involvement of RDD with NGS genetic testing, indicating the difficulty of making a diagnosis before surgery. This extremely rare case offers new references for the understanding of abdominal viscera RDD.<\/jats:p><\/jats:sec>","DOI":"10.1186\/s12893-020-01014-0","type":"journal-article","created":{"date-parts":[[2021,1,6]],"date-time":"2021-01-06T21:03:31Z","timestamp":1609967011000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["An extremely rare case of Rosai-Dorfman disease in the spleen"],"prefix":"10.1186","volume":"21","author":[{"given":"Xuewu","family":"Yang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chuang","family":"Fang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuanpu","family":"Sha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qi","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xing","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Furong","family":"Du","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3595-7059","authenticated-orcid":false,"given":"Weijun","family":"Tian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,1,6]]},"reference":[{"key":"1014_CR1","first-page":"63","volume":"87","author":"J Rosai","year":"1969","unstructured":"Rosai J, Dorfman RF. 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Informed consent was obtained from the family of the patient.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to publication"}},{"value":"Data supporting the present findings can be obtained, after obtaining permission from the Tianjin Medical University General Hospital. The data can be obtained by corresponding author.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Availability of data and material"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"24"}}