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'''Myxofibrosarcoma''' is a malignant soft tissue tumour, in the [[fibroblastic/myofibroblastic tumours]] group. | |||
It should '''not''' be confused with ''[[low-grade fibromyxoid sarcoma]]''.<ref name=pmid8650138>{{Cite journal | last1 = Mentzel | first1 = T. | last2 = Katenkamp | first2 = D. | last3 = Fletcher | first3 = CD. | title = [Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course]. | journal = Pathologe | volume = 17 | issue = 2 | pages = 116-21 | month = Mar | year = 1996 | doi = | PMID = 8650138 }}</ref> | |||
*[[AKA]] ''myxoid malignant fibrous histiocytoma'' or ''myxoid MFH''.<ref>{{Cite journal | last1 = Fujimura | first1 = T. | last2 = Okuyama | first2 = R. | last3 = Terui | first3 = T. | last4 = Okuno | first4 = K. | last5 = Masu | first5 = A. | last6 = Masu | first6 = T. | last7 = Chiba | first7 = S. | last8 = Kunii | first8 = T. | last9 = Tagami | first9 = H. | title = Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) showing cutaneous presentation: report of two cases. | journal = J Cutan Pathol | volume = 32 | issue = 7 | pages = 512-5 | month = Aug | year = 2005 | doi = 10.1111/j.0303-6987.2005.00368.x | PMID = 16008697 }}</ref> | |||
==General== | |||
*Malignant. | |||
*Usually older people, superficial (skin/dermis) and extremities (arm, legs).<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref><ref name=pmid17197925/> | |||
*Prognosis better than [[pleomorphic undifferentiated sarcoma]]. | |||
**Metastatic potential inversely related to component/portion that is myxoid.<ref name=pmid192434>{{Cite journal | last1 = Weiss | first1 = SW. | last2 = Enzinger | first2 = FM. | title = Myxoid variant of malignant fibrous histiocytoma. | journal = Cancer | volume = 39 | issue = 4 | pages = 1672-85 | month = Apr | year = 1977 | doi = | PMID = 192434 }}</ref> | |||
==Microscopic== | |||
Features:<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref> | |||
*Discontinuous fibrous septae. | |||
*Myxoid background. | |||
*Variable cellularity and nuclear pleomorphism. | |||
*Spindle cells or epithelioid cells.<ref name=pmid17197925/> | |||
*Curvilinear vessels.<ref name=pmid17197925>{{Cite journal | last1 = Nascimento | first1 = AF. | last2 = Bertoni | first2 = F. | last3 = Fletcher | first3 = CD. | title = Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases. | journal = Am J Surg Pathol | volume = 31 | issue = 1 | pages = 99-105 | month = Jan | year = 2007 | doi = 10.1097/01.pas.0000213379.94547.e7 | PMID = 17197925 }}</ref> | |||
DDx: | |||
*[[Liposarcoma|Myxoid liposarcoma]]. | |||
*[[Low-grade fibromyxoid sarcoma]] - alternating fibrous and myxoid areas.<ref name=pmid8650138/> | |||
*[[Myxoma]]. | |||
Image: | |||
*[http://www.sarcomaimages.com/sub.php?v=sample-case&p=myxofibrosarcoma-myxoid-mfh Myxofibrosarcoma (sarcomaimages.com)]. | |||
==IHC== | |||
*Vimentin +ve -- otherwise non-distinctive.<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref> | |||
==See also== | |||
*[[Fibroblastic/myofibroblastic tumours]]. | |||
==References== | |||
{{Reflist|1}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] |
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