{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,2]],"date-time":"2026-03-02T11:03:17Z","timestamp":1772449397170,"version":"3.50.1"},"reference-count":23,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2025,4,10]],"date-time":"2025-04-10T00:00:00Z","timestamp":1744243200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Current Oncology"],"abstract":"<jats:p>Pseudomyogenic haemangioendotheliomas (PMH) are exceedingly rare, mostly occurring in soft tissue, with malignant cases even more uncommon. In this report, we present a case of a 28-year-old male initially suspected of having a fibroblastic osteosarcoma of the right femur, which was then correctly diagnosed as a primary pseudomyogenic hemangioendothelioma of the bone with synchronous metastases to other skeletal segments. Molecular analysis through targeted RNA sequencing confirmed the correct diagnosis, revealing a fusion transcript ACTB::FOSB. To our knowledge, this is one of the few reported cases of suffering from multiple pathological fractures. The rapid skeletal progression and the onset of distant metastases in this case is highly unusual considering the typically indolent clinical course commonly reported in the literature for this tumor.<\/jats:p>","DOI":"10.3390\/curroncol32040219","type":"journal-article","created":{"date-parts":[[2025,4,10]],"date-time":"2025-04-10T07:41:51Z","timestamp":1744270911000},"page":"219","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["A Rare Malignant Case of a Primary Pseudomyogenic Haemangioendothelioma of the Bone"],"prefix":"10.3390","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9128-3186","authenticated-orcid":false,"given":"Annabella","family":"Di Mauro","sequence":"first","affiliation":[{"name":"Pathology Unit, Istituto Nazionale Tumori, IRCCS\u2014Fondazione G. Pascale, 80131 Napoli, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3019-354X","authenticated-orcid":false,"given":"Salvatore","family":"Tafuto","sequence":"additional","affiliation":[{"name":"Sarcomas and Rare Tumors Unit, Istituto Nazionale Tumori, IRCCS\u2014Fondazione G. Pascale, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9993-2013","authenticated-orcid":false,"given":"Lucia","family":"Cannella","sequence":"additional","affiliation":[{"name":"Sarcomas and Rare Tumors Unit, Istituto Nazionale Tumori, IRCCS\u2014Fondazione G. Pascale, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Collina","sequence":"additional","affiliation":[{"name":"Pathology Unit, Istituto Nazionale Tumori, IRCCS\u2014Fondazione G. Pascale, 80131 Napoli, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Neri","sequence":"additional","affiliation":[{"name":"Sarcomas and Rare Tumors Unit, Istituto Nazionale Tumori, IRCCS\u2014Fondazione G. Pascale, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0569-8570","authenticated-orcid":false,"given":"Ottavia","family":"Clemente","sequence":"additional","affiliation":[{"name":"Sarcomas and Rare Tumors Unit, Istituto Nazionale Tumori, IRCCS\u2014Fondazione G. Pascale, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Imma","family":"D\u2019Arbitrio","sequence":"additional","affiliation":[{"name":"Pathology Unit, Istituto Nazionale Tumori, IRCCS\u2014Fondazione G. Pascale, 80131 Napoli, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Ricci","sequence":"additional","affiliation":[{"name":"Nuclear Medicine Unit, Istituto Nazionale Tumori di Napoli, IRCCS G. Pascale, 80031, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6649-9903","authenticated-orcid":false,"given":"Secondo","family":"Lastoria","sequence":"additional","affiliation":[{"name":"Nuclear Medicine Unit, Istituto Nazionale Tumori di Napoli, IRCCS G. Pascale, 80031, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gerardo","family":"Ferrara","sequence":"additional","affiliation":[{"name":"Pathology Unit, Istituto Nazionale Tumori, IRCCS\u2014Fondazione G. Pascale, 80131 Napoli, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annarosaria","family":"De Chiara","sequence":"additional","affiliation":[{"name":"Histopathology of Lymphomas and Sarcomas SSD, Istituto Nazionale Tumori, IRCCS\u2014Fondazione G. Pascale, 80131, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,4,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1097\/00000478-200301000-00006","article-title":"Epithelioid sarcoma-like hemangioendothelioma","volume":"27","author":"Billings","year":"2003","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1097\/PAS.0b013e3181ff0901","article-title":"Pseudomyogenic hemangioendothelioma: A distinctive, often multicentric tumor with indolent behavior","volume":"35","author":"Hornick","year":"2011","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/j.humpath.2017.10.023","article-title":"Pseudomyogenic hemangioendothelioma of skin, bone and soft tissue-a clinicopathological, immunohistochemical, and fluorescence in situ hybridization study","volume":"71","author":"Pradhan","year":"2018","journal-title":"Hum. Pathol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1097\/PAS.0000000000000613","article-title":"Primary Pseudomyogenic Hemangioendothelioma of Bone","volume":"40","author":"Inyang","year":"2016","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1016\/j.anndiagpath.2019.06.003","article-title":"Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma of bone: Clinicopathologic features of 5 cases","volume":"41","author":"Kosemehmetoglu","year":"2019","journal-title":"Ann. Diagn. Pathol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"e26781","DOI":"10.1002\/pbc.26781","article-title":"Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma","volume":"65","author":"Gabor","year":"2018","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_7","unstructured":"Hornick, J.L., Fletcher, C.D.M., Mertens Fletcher, C.D., Bridge, J.A., Hogendoorn, P.C.W., and Mertens, F. (2014). Psudomyogenic Haemangioendothelioma WHO Classification of Tumours of Soft Tissue and Bone, 2014th Lyon (France) International Agency for Research on Cancer."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1186\/s13000-015-0384-z","article-title":"Pseudomyogenic hemangioendothelioma\/epithelioid sarcoma-like hemangioendothelioma of the lower limb: Report of a rare case","volume":"10","author":"Fan","year":"2015","journal-title":"Diagn. Pathol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.cancergen.2011.01.002","article-title":"Translocation t(7;19)(q22;q13)\u2014A recurrent chromosome aberration in pseudomyogenic hemangioendothelioma?","volume":"204","author":"Trombetta","year":"2011","journal-title":"Cancer Genet."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1002\/path.4322","article-title":"A novel SERPINE1-FOSB fusion gene results in transcriptional up-regulation of FOSB in pseudomyogenic haemangioendothelioma","volume":"232","author":"Walther","year":"2014","journal-title":"J. Pathol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1038\/s41379-018-0175-7","article-title":"Diagnosis of known sarcoma fusions and novel fusion partners by targeted RNA sequencing with identification of a recurrent ACTB-FOSB fusion in pseudomyogenic hemangioendothelioma","volume":"32","author":"Zhu","year":"2019","journal-title":"Mod. Pathol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"293","DOI":"10.21873\/cgp.20134","article-title":"Fusion of the Genes WWTR1 and FOSB in Pseudomyogenic Hemangioendothelioma","volume":"16","author":"Panagopoulos","year":"2019","journal-title":"Cancer Genom. Proteom."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1186\/s13000-016-0530-2","article-title":"Diagnostic utility of FOSB immunohistochemistry in pseudomyogenic hemangioendothelioma and its histological mimics","volume":"11","author":"Sugita","year":"2016","journal-title":"Diagn. Pathol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"596","DOI":"10.1097\/PAS.0000000000000795","article-title":"FOSB is a Useful Diagnostic Marker for Pseudomyogenic Hemangioendothelioma","volume":"41","author":"Hung","year":"2017","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1097\/PAS.0000000000001552","article-title":"The 2020 WHO Classification: What\u2019s new in soft tissue tumor pathology?","volume":"45","author":"Kallen","year":"2021","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"763","DOI":"10.5858\/arpa.2017-0430-RS","article-title":"Pseudomyogenic Hemangioendothelioma","volume":"143","author":"Mansour","year":"2019","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1228","DOI":"10.1016\/j.radcr.2019.06.029","article-title":"Pseudomyogenic hemangioendothelioma-A case report and review of the literature","volume":"14","author":"Dianat","year":"2019","journal-title":"Radiol. Case Rep."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1397","DOI":"10.1016\/S1470-2045(17)30622-8","article-title":"Gemcitabine and docetaxel versus doxorubicin as first-line treatment in previously untreated advanced unresectable or metastatic soft-tissue sarcomas (GeDDiS): A randomised controlled phase 3 trial","volume":"18","author":"Seddon","year":"2017","journal-title":"Lancet Oncol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"817","DOI":"10.4103\/jcrt.JCRT_1138_20","article-title":"Multifocal primary pseudomyogenic hemangioendothelioma of bone managed with denosumab: A rare case with diagnostic and therapeutic challenge","volume":"18","author":"Pasricha","year":"2022","journal-title":"J. Cancer Res. Ther."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1093\/oxfordjournals.annonc.a058622","article-title":"Epirubicin and ifosfamide in advanced soft tissue sarcomas","volume":"4","author":"Frustaci","year":"1993","journal-title":"Ann. Oncol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1537","DOI":"10.1200\/JCO.1995.13.7.1537","article-title":"Doxorubicin versus CYVADIC versus doxorubicin plus ifosfamide in first-line treatment of advanced soft tissue sarcomas: A randomized study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group","volume":"13","author":"Santoro","year":"1995","journal-title":"J. Clin. Oncol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1381","DOI":"10.1007\/s00256-015-2168-7","article-title":"An aggressive case of pseudomyogenic haemangioendothelioma of bone with pathological fracture and rapidly progressive pulmonary metastatic disease: Case report and review of the literature","volume":"44","author":"Shah","year":"2015","journal-title":"Skelet. Radiol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1007\/s00256-014-2024-1","article-title":"Primary pseudomyogenic haemangioendothelioma of bone: Report of two cases","volume":"44","author":"Righi","year":"2015","journal-title":"Skelet. Radiol."}],"container-title":["Current Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1718-7729\/32\/4\/219\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T17:12:15Z","timestamp":1760029935000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1718-7729\/32\/4\/219"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,4,10]]},"references-count":23,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2025,4]]}},"alternative-id":["curroncol32040219"],"URL":"https:\/\/doi.org\/10.3390\/curroncol32040219","relation":{},"ISSN":["1718-7729"],"issn-type":[{"value":"1718-7729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,4,10]]}}}