{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T09:38:54Z","timestamp":1792143534005,"version":"4.4.0"},"reference-count":8,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2021,2,15]],"date-time":"2021-02-15T00:00:00Z","timestamp":1613347200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Histiocytic sarcoma (HS) is a rare hematopoietic neoplasm derived from non-Langerhans histiocytic cells of the monocyte\/macrophage system. With an incidence of 0.17\/million individuals and a slight male preference, HS presents with a wide age distribution. Most commonly, it occurs as a primary malignancy. In approximately 25% of the cases a presumed transdifferentiation of a preexisting hematolymphoid disorder can be demonstrated. The clinical presentation varies from a localized solitary mass to severe disseminated disease often with extranodal involvement including skin, soft tissue, the gastrointestinal tract and the hematopoietic system. Systemic symptoms in terms of weight loss, fever and night sweats often occur. The diagnostic work-up of HS is extremely challenging due to the rarity of the disease as well as a wide differential diagnosis in terms of a histologic overlap with diverse mimics. No standardized treatment for HS exists and especially in a disseminated disease the clinical course is overly aggressive with a dismal outcome. The median overall survival from the time of diagnosis is approximately six months. We report a 43-year-old previously healthy Caucasian male admitted to our hospitals with abdominal pain and a feeling of fatigue. We demonstrate both the challenges of a correct diagnosis and an effective treatment as well as the aggressive nature of histiocytic sarcoma.<\/jats:p>","DOI":"10.3390\/diagnostics11020310","type":"journal-article","created":{"date-parts":[[2021,2,15]],"date-time":"2021-02-15T02:35:23Z","timestamp":1613356523000},"page":"310","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Histiocytic Sarcoma: Challenging Course, Dismal Outcome"],"prefix":"10.3390","volume":"11","author":[{"given":"Kim Francis","family":"Andersen","sequence":"first","affiliation":[{"name":"Department of Clinical Physiology, Nuclear Medicine &amp; PET, Rigshospitalet, Copenhagen University Hospital, Blegdamsvej 9, DK-2100 Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lene Dissing","family":"Sj\u00f6","sequence":"additional","affiliation":[{"name":"Department of Pathology, Rigshospitalet, Copenhagen University Hospital, Blegdamsvej 9, DK-2100 Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Kampmann","sequence":"additional","affiliation":[{"name":"Department of Haematology, Rigshospitalet, Copenhagen University Hospital, Blegdamsvej 9, DK-2100 Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3274-5325","authenticated-orcid":false,"given":"Torben Bridstrup","family":"Pedersen","sequence":"additional","affiliation":[{"name":"Department of Surgery, Herlev Hospital, Borgmester Ib Juuls Vej 1, DK-2730 Herlev, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,15]]},"reference":[{"key":"ref_1","unstructured":"Swerdlow, S.H., Campo, E., Harris, N.L., Jaffe, E.S., Pileri, S.A., Stein, H., and Thiele, J. (2017). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC. [4th ed.]."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"165","DOI":"10.2169\/internalmedicine.47.0386","article-title":"Histiocytic sarcoma: Identification of its histiocytic origin using immunohistochemistry","volume":"47","author":"Yoshida","year":"2008","journal-title":"Intern. Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1133","DOI":"10.1097\/01.pas.0000131541.95394.23","article-title":"Extranodal Histiocytic Sarcoma: Clinicopathologic Analysis of 14 Cases of a Rare Epithelioid Malignancy","volume":"28","author":"Hornick","year":"2004","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1182\/blood-2017-10-812495","article-title":"Histiocytic sarcoma: A population-based analysis of incidence, demographic disparities, and long-term outcomes","volume":"131","author":"Kommalapati","year":"2018","journal-title":"Blood"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"98723","DOI":"10.18632\/oncotarget.21920","article-title":"Prognostic factors for histiocytic and dendritic cell neoplasms","volume":"8","author":"Shimono","year":"2017","journal-title":"Oncotarget"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2413","DOI":"10.1016\/j.ejca.2015.06.109","article-title":"Impact of surgery, radiation and systemic therapy on the outcomes of patients with dendritic cell and histiocytic sarcomas","volume":"51","author":"Gounder","year":"2015","journal-title":"Eur. J. Cancer"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"650","DOI":"10.5858\/arpa.2018-0349-RS","article-title":"Histiocytic Sarcoma","volume":"144","author":"Hung","year":"2019","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3960\/jslrt.53.1","article-title":"Histiocytic Sarcoma: An Updated Literature Review Based on the 2008 WHO Classification","volume":"53","author":"Takahashi","year":"2013","journal-title":"J. Clin. Exp. Hematop."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/11\/2\/310\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T05:24:15Z","timestamp":1760160255000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/11\/2\/310"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,2,15]]},"references-count":8,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2021,2]]}},"alternative-id":["diagnostics11020310"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics11020310","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,2,15]]}}}