{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,9,27]],"date-time":"2023-09-27T07:42:48Z","timestamp":1695800568548},"reference-count":40,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2016,4,22]],"date-time":"2016-04-22T00:00:00Z","timestamp":1461283200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["APMIS"],"published-print":{"date-parts":[[2016,6]]},"abstract":"<jats:p>In accordance with recent terminology, it is proposed that malignant mesenchymoma should be renamed \u2018composite sarcoma\u2019 and defined as \u2018a sarcoma composed of two or more cellular types each of which is sufficiently differentiated to permit clear recognition of its histogenetic type microscopically, immunohistochemically or ultrastructurally; excluding fibrosarcomatous and high\u2010grade pleomorphic undifferentiated sarcomatous component, dedifferentiated sarcoma and the combination of osteosarcoma and chondrosarcoma which is regarded as a single histogenetic type\u2019. Four cases of primary osseous composite sarcoma (<jats:styled-content style=\"fixed-case\">POCS<\/jats:styled-content>) were identified among 928 primary bone sarcomas. Their age ranged from 10 to 87 years, peak incidence in the second decade with equal sex distribution. Most presented with pain, commonest in the knee, affecting the metaphysis, appearing radiologically as expansile infiltrative osteolytic lesions with cortical erosion, periosteal reaction, variable extent of osteoblastic areas and soft tissue extension. All contained variable amounts of conventional high\u2010grade osteosarcoma with or without chondrosarcoma component; the other constituents were liposarcoma, rhabdomyosarcoma and leiomyosarcoma. In all cases, Ki67 proliferative index was over 35%, there was no <jats:styled-content style=\"fixed-case\">CDK<\/jats:styled-content>4 and <jats:styled-content style=\"fixed-case\">MDM<\/jats:styled-content>2 amplification. The absence of low\u2010grade component supported the <jats:italic>de novo<\/jats:italic> origin of <jats:styled-content style=\"fixed-case\">POCS<\/jats:styled-content> rather than derivation from divergent dedifferentiation. The two older patients with hitherto undescribed osteoleiomyosarcoma died 2 and 10 months after operation, whereas the two younger with osteorhabdomyosarcoma and osteoliposarcoma enjoyed disease\u2010free survival at 16 and 6 years after chemotherapy despite the latter showing lung metastasis at presentation. Identification of the different lines of differentiation together with their approximate amounts and histological grades is therefore mandatory for <jats:styled-content style=\"fixed-case\">POCS<\/jats:styled-content> as multi\u2010agent chemotherapy catered for each sarcoma component might offer hope for long\u2010term disease\u2010free survival.<\/jats:p>","DOI":"10.1111\/apm.12540","type":"journal-article","created":{"date-parts":[[2016,4,22]],"date-time":"2016-04-22T21:51:49Z","timestamp":1461361909000},"page":"487-499","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Critical reappraisal of primary osseous composite sarcoma (malignant mesenchymoma) \u2013 analysis of four cases and literature review"],"prefix":"10.1111","volume":"124","author":[{"given":"Louis T. 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