{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T03:54:10Z","timestamp":1777607650720,"version":"3.51.4"},"reference-count":16,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2022,4,18]],"date-time":"2022-04-18T00:00:00Z","timestamp":1650240000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hematology Reports"],"abstract":"<jats:p>Myeloid sarcomas (MS) are rare extramedullary (EM) hematological tumors that generally arise during the natural course of acute myeloid leukemia (AML), occurring concomitantly with the onset of systemic leukemia; it can also occur following onset but rarely before. Common sites of EM involvement include the lymph nodes, skin, soft tissue, bone and peritoneum. Herein, we report the case of a 63-year-old man who presented EM AML upon initial diagnosis involving the bone marrow, lymph nodes and skin (leukemia cutis). A diagnosis was made based on immunohistochemistry (IHC). This case presents a diagnostic dilemma due to its atypical presentation and the sites involved. It also highlights the importance of IHC in the diagnosis of EM AML. The potential role of hypomethylating agents and Venetoclax in cases not eligible for hematopoietic stem cell transplant are also discussed.<\/jats:p>","DOI":"10.3390\/hematolrep14020021","type":"journal-article","created":{"date-parts":[[2022,4,20]],"date-time":"2022-04-20T02:04:22Z","timestamp":1650420262000},"page":"143-148","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Extramedullary Acute Leukemia\u2014Still an Unforeseen Presentation"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9965-7173","authenticated-orcid":false,"given":"Dina","family":"Rochate","sequence":"first","affiliation":[{"name":"Divino Esp\u00edrito Santo\u2019s Hospital, 9500-370 Ponta Delgada, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolina","family":"Pav\u00e3o","sequence":"additional","affiliation":[{"name":"Divino Esp\u00edrito Santo\u2019s Hospital, 9500-370 Ponta Delgada, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rui","family":"Amaral","sequence":"additional","affiliation":[{"name":"Divino Esp\u00edrito Santo\u2019s Hospital, 9500-370 Ponta Delgada, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolina","family":"Viveiros","sequence":"additional","affiliation":[{"name":"Divino Esp\u00edrito Santo\u2019s Hospital, 9500-370 Ponta Delgada, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9","family":"Cabe\u00e7adas","sequence":"additional","affiliation":[{"name":"Portuguese Institute of Oncology, 1070-212 Lisbon, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vitor","family":"Carneiro","sequence":"additional","affiliation":[{"name":"Divino Esp\u00edrito Santo\u2019s Hospital, 9500-370 Ponta Delgada, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristina","family":"Fraga","sequence":"additional","affiliation":[{"name":"Divino Esp\u00edrito Santo\u2019s Hospital, 9500-370 Ponta Delgada, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1016\/j.blre.2016.04.001","article-title":"Extramedullary acute myelogenous leukemia","volume":"30","author":"Solh","year":"2016","journal-title":"Blood Rev."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1309\/AJCPO58YWIBUBESX","article-title":"Extramedullary Manifestations of Myeloid Neoplasms","volume":"144","author":"Wilson","year":"2015","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/j.clml.2017.02.027","article-title":"Myeloid Sarcoma: Presentation, Diagnosis, and Treatment","volume":"17","author":"Almond","year":"2017","journal-title":"Clin. Lymphoma Myeloma Leuk."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1309\/AJCP6GR8BDEXPKHR","article-title":"An Updated Approach to the Diagnosis of Myeloid Leukemia Cutis","volume":"132","author":"Cronin","year":"2009","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"3785","DOI":"10.1182\/blood-2011-04-347229","article-title":"How I treat extramedullary acute myeloid leukemia","volume":"118","author":"Bakst","year":"2011","journal-title":"Blood"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2375","DOI":"10.1182\/blood-2016-01-643569","article-title":"The 2016 revision of the World Health Organization classification of lymphoid neoplasms","volume":"127","author":"Swerdlow","year":"2016","journal-title":"Blood"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1007\/s11912-020-00919-6","article-title":"Diagnostic and Therapeutic Considerations for Extramedullary Leukemia","volume":"22","author":"Bakst","year":"2020","journal-title":"Curr. Oncol. Rep."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"3544","DOI":"10.1200\/JCO.2016.67.5892","article-title":"Extramedullary Disease in Adult Acute Myeloid Leukemia Is Common but Lacks Independent Significance: Analysis of Patients in ECOG-ACRIN Cancer Research Group Trials, 1980-2008","volume":"34","author":"Ganzel","year":"2016","journal-title":"J. Clin. Oncol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"909","DOI":"10.1002\/dc.22859","article-title":"Acute myeloid leukemia with leukemic pleural effusion","volume":"41","author":"Chang","year":"2013","journal-title":"Diagn. Cytopathol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"70","DOI":"10.4103\/1658-5127.186327","article-title":"Aleukemic granulocytic sarcoma and leukemia cutis: A report of two rare cases and review of literature","volume":"7","author":"Anand","year":"2016","journal-title":"J. Appl. Hematol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1","DOI":"10.7175\/cmi.v14i1.1460","article-title":"Myeloid Sarcoma Involving Kidneys: From Diagnosis to Treatment. Case Report and Literature Review","volume":"14","author":"Guidotti","year":"2020","journal-title":"Clin. Manag. Issues"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1080\/10245332.2017.1333275","article-title":"Clinical characteristics, molecular profile and outcomes of myeloid sarcoma: A single institution experience over 13 years","volume":"23","author":"Kaur","year":"2018","journal-title":"Hematology"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1165","DOI":"10.6004\/jnccn.2012.0120","article-title":"Management of extramedullary leukemia as a presentation of acute myeloid leukemia","volume":"10","author":"Slomowitz","year":"2012","journal-title":"J. Natl. Compr. Cancer Netw."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"2020","DOI":"10.1080\/10428194.2020.1742908","article-title":"The efficacy of venetoclax and hypomethylating agents in acute myeloid leukemia with extramedullary involvement","volume":"61","author":"Otoukesh","year":"2020","journal-title":"Leuk. Lymphoma"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1182\/blood-2018-08-868752","article-title":"Venetoclax combined with decitabine or azacitidine in treatment-naive, elderly patients with acute myeloid leukemia","volume":"133","author":"Dinardo","year":"2019","journal-title":"Blood"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"E253","DOI":"10.1002\/ajh.25567","article-title":"Association of leukemia genetics with response to venetoclax and hypomethylating agents in relapsed\/refractory acute myeloid leukemia","volume":"94","author":"Aldoss","year":"2019","journal-title":"Am. J. Hematol."}],"container-title":["Hematology Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2038-8330\/14\/2\/21\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:55:51Z","timestamp":1760136951000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2038-8330\/14\/2\/21"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,18]]},"references-count":16,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2022,6]]}},"alternative-id":["hematolrep14020021"],"URL":"https:\/\/doi.org\/10.3390\/hematolrep14020021","relation":{},"ISSN":["2038-8330"],"issn-type":[{"value":"2038-8330","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,18]]}}}