Difference between revisions of "Low-grade fibromyxoid sarcoma"

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#redirect [[Soft_tissue_lesions#Low-grade_fibromyxoid_sarcoma]]
'''Low-grade fibromyxoid sarcoma''', abbreviated '''LGFMS''', is an uncommon [[malignant]] [[Fibroblastic/myofibroblastic tumours|fibroblastic/myofibroblastic tumour]].
 
It is also known as '''hyalinizing spindle cell tumour'''. It should '''not''' be confused with ''[[myxofibrosarcoma]]''.
 
==General==
*Deep soft tissue.
*Related to ''hyalinizing spindle cell tumour with giant collagen rosettes''.<ref name=pmid20864787>{{Cite journal  | last1 = Ramaswamy | first1 = AS. | last2 = Chatura | first2 = KR. | title = Hyalinizing spindle cell tumor with giant rosettes. | journal = Ann Saudi Med | volume = 31 | issue = 1 | pages = 83-6 | month =  | year =  | doi =  | PMID = 20864787 | PMC = 3101732 }}</ref>
 
==Microscopic==
Features:<ref>{{cite journal |author=Vernon SE, Bejarano PA |title=Low-grade fibromyxoid sarcoma: a brief review |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=9 |pages=1358–60 |year=2006 |month=September |pmid=16948525 |doi= |url=}}</ref>
*[[Myxoid stroma]] alternating with fibrogenic areas - '''key feature'''.
*Low cellularity.
*Spindle cells.
*+/-Rosette of collagen with central hyaline core.<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
 
Notes:
*Few/absent mitoses.
 
DDx:
*[[Leiomyoma]].
*[[Myxoid liposarcoma]].
*[[Desmoplastic fibroblastoma]].
*[[Intramuscular myxoma]].
 
===Images===
<gallery>
Image:Low-grade_fibromyxoid_sarcoma_-_low_mag.jpg | LGFMS - low mag. (WC)
Image:Low-grade_fibromyxoid_sarcoma_-_intermed_mag.jpg | LGFMS - intermed. mag. (WC)
Image:Low-grade_fibromyxoid_sarcoma_-2-_high_mag.jpg | LGFMS - high mag. (WC)
</gallery>
www:
*[http://www.springerimages.com/Images/MedicineAndPublicHealth/1-10.1007_s00428-009-0776-0-7 LGFMS (springerimages.com)].
*[http://www.uscap.org/site~/iap2006/slides18-2v.htm LGFMS (uscap.org)].
 
==IHC==
Features:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
*EMA +ve.
*CD99 +ve.
*BCL2 +ve.
 
Others:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
*SMA -ve.
*S100 -ve.
*Desmin -ve.
 
==Molecular pathology==
*t(7;16)(q33;p11).<ref>{{cite journal |author=Panagopoulos I, Storlazzi CT, Fletcher CD, ''et al.'' |title=The chimeric FUS/CREB3l2 gene is specific for low-grade fibromyxoid sarcoma |journal=Genes Chromosomes Cancer |volume=40 |issue=3 |pages=218–28 |year=2004 |month=July |pmid=15139001 |doi=10.1002/gcc.20037 |url=}}</ref>
*t(11;16).<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
 
==See also==
*[[Fibroblastic/myofibroblastic tumours]].
 
==References==
{{Reflist|2}}
 
[[Category:Diagnosis]]
[[Category:Fibroblastic/myofibroblastic tumours]]

Latest revision as of 22:40, 16 November 2014

Low-grade fibromyxoid sarcoma, abbreviated LGFMS, is an uncommon malignant fibroblastic/myofibroblastic tumour.

It is also known as hyalinizing spindle cell tumour. It should not be confused with myxofibrosarcoma.

General

  • Deep soft tissue.
  • Related to hyalinizing spindle cell tumour with giant collagen rosettes.[1]

Microscopic

Features:[2]

  • Myxoid stroma alternating with fibrogenic areas - key feature.
  • Low cellularity.
  • Spindle cells.
  • +/-Rosette of collagen with central hyaline core.[3]

Notes:

  • Few/absent mitoses.

DDx:

Images

  • LGFMS - low mag. (WC)

  • LGFMS - intermed. mag. (WC)

  • LGFMS - high mag. (WC)

www:

IHC

Features:[3]

  • EMA +ve.
  • CD99 +ve.
  • BCL2 +ve.

Others:[3]

  • SMA -ve.
  • S100 -ve.
  • Desmin -ve.

Molecular pathology

  • t(7;16)(q33;p11).[4]
  • t(11;16).[3]

See also

References

  1. ↑ Ramaswamy, AS.; Chatura, KR.. "Hyalinizing spindle cell tumor with giant rosettes.". Ann Saudi Med 31 (1): 83-6. PMC 3101732. PMID 20864787. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3101732/.
  2. ↑ Vernon SE, Bejarano PA (September 2006). "Low-grade fibromyxoid sarcoma: a brief review". Arch. Pathol. Lab. Med. 130 (9): 1358–60. PMID 16948525.
  3. ↑ 3.0 3.1 3.2 3.3 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 611. ISBN 978-0781765275.
  4. ↑ Panagopoulos I, Storlazzi CT, Fletcher CD, et al. (July 2004). "The chimeric FUS/CREB3l2 gene is specific for low-grade fibromyxoid sarcoma". Genes Chromosomes Cancer 40 (3): 218–28. doi:10.1002/gcc.20037. PMID 15139001.
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