Difference between revisions of "Myxofibrosarcoma"
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'''Myxofibrosarcoma''' is a malignant soft tissue tumour, in the [[fibroblastic/myofibroblastic tumours]] group. It is also known as '''myxoid [[malignant fibrous histiocytoma]]''' or '''myxoid MFH'''.<ref name=pmid16008697>{{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> | |||
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> | |||
==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]] | ||
[[Category:Soft tissue lesions]] | |||
Latest revision as of 12:26, 24 June 2016
Myxofibrosarcoma is a malignant soft tissue tumour, in the fibroblastic/myofibroblastic tumours group. It is also known as myxoid malignant fibrous histiocytoma or myxoid MFH.[1]
It should not be confused with low-grade fibromyxoid sarcoma.[2]
General
- Malignant.
- Usually older people, superficial (skin/dermis) and extremities (arm, legs).[3][4]
- Prognosis better than pleomorphic undifferentiated sarcoma.
- Metastatic potential inversely related to component/portion that is myxoid.[5]
Microscopic
Features:[3]
- Discontinuous fibrous septae.
- Myxoid background.
- Variable cellularity and nuclear pleomorphism.
- Spindle cells or epithelioid cells.[4]
- Curvilinear vessels.[4]
DDx:
- Myxoid liposarcoma.
- Low-grade fibromyxoid sarcoma - alternating fibrous and myxoid areas.[2]
- Myxoma.
Image:
IHC
- Vimentin +ve -- otherwise non-distinctive.[3]
See also
References
- ↑ Fujimura, T.; Okuyama, R.; Terui, T.; Okuno, K.; Masu, A.; Masu, T.; Chiba, S.; Kunii, T. et al. (Aug 2005). "Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) showing cutaneous presentation: report of two cases.". J Cutan Pathol 32 (7): 512-5. doi:10.1111/j.0303-6987.2005.00368.x. PMID 16008697.
- ↑ 2.0 2.1 Mentzel, T.; Katenkamp, D.; Fletcher, CD. (Mar 1996). "[Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course].". Pathologe 17 (2): 116-21. PMID 8650138.
- ↑ 3.0 3.1 3.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 612-3. ISBN 978-0781765275.
- ↑ 4.0 4.1 4.2 Nascimento, AF.; Bertoni, F.; Fletcher, CD. (Jan 2007). "Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases.". Am J Surg Pathol 31 (1): 99-105. doi:10.1097/01.pas.0000213379.94547.e7. PMID 17197925.
- ↑ Weiss, SW.; Enzinger, FM. (Apr 1977). "Myxoid variant of malignant fibrous histiocytoma.". Cancer 39 (4): 1672-85. PMID 192434.