Difference between revisions of "Myxofibrosarcoma"

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#redirect [[Fibroblastic/myofibroblastic_tumours#Myxofibrosarcoma]]
'''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]]

Revision as of 12:24, 24 June 2016

Myxofibrosarcoma is a malignant soft tissue tumour, in the fibroblastic/myofibroblastic tumours group.

It should not be confused with low-grade fibromyxoid sarcoma.[1]

  • AKA myxoid malignant fibrous histiocytoma or myxoid MFH.[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:

Image:

IHC

  • Vimentin +ve -- otherwise non-distinctive.[3]

See also

References

  1. 1.0 1.1 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.
  2. 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.
  3. 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. 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.
  5. Weiss, SW.; Enzinger, FM. (Apr 1977). "Myxoid variant of malignant fibrous histiocytoma.". Cancer 39 (4): 1672-85. PMID 192434.