PMID- 21399449
OWN - NLM
STAT- MEDLINE
DCOM- 20110713
LR  - 20110314
IS  - 1533-0311 (Electronic)
IS  - 0193-1091 (Linking)
VI  - 33
IP  - 2
DP  - 2011 Apr
TI  - FUS (16p11) gene rearrangement as detected by fluorescence in-situ hybridization 
      in cutaneous low-grade fibromyxoid sarcoma: a potential diagnostic tool.
PG  - 140-3
LID - 10.1097/IAE.0b013e318176de80 [doi]
AB  - Low-grade fibromyxoid sarcoma (LGFMS) is a rare, typically deep-seated soft 
      tissue neoplasm with deceptively bland cytology and metastatic potential. A 
      t(7;16)(q34;p11) translocation, yielding a FUS/CREB3L2 fusion gene, has been 
      identified in approximately 80%-90% of deep soft tissue LGFMS. Cutaneous 
      fibromyxoid neoplasms occur not infrequently; dermatopathologists rarely consider 
      LGFMS in the differential diagnosis, as this lesion is uncommon in the skin. We 
      identified a group of superficial LGFMS and a spectrum of other cutaneous 
      fibromyxoid neoplasms and performed fluorescence in situ hybridization (FISH) to 
      assess the frequency of FUS rearrangement. FISH for the chromosomal rearrangement 
      of FUS (16p11), using a dual-color, break-apart probe (Abbott Molecular/Vysis, 
      Des Plaines, IL), was performed on formalin-fixed paraffin-embedded tissue 
      sections from superficial LGFMS (n = 6), myxomas (n = 10), and 
      myxofibrosarcoma/myxoid malignant fibrous histiocytomas (myxoid MFH) (n = 5). One 
      hundred nonoverlapping tumor nuclei per case were evaluated for either fused 
      (normal) or split (translocated) signals. Of the LGFMS, 4 of 6 (67%) showed a 
      rearrangement of FUS (range: 72%-80% positive nuclei per 100 nuclei). The other 
      neoplasms within the differential diagnosis were devoid of any rearrangement 
      involving FUS (range: 0%-2% positive nuclei per 100 nuclei). Our observed 
      frequency of FUS rearrangement in superficial LGFMS is consistent with those 
      published in the literature for more deeply seated lesions. When applied to 
      suspicious superficial myxoid or fibromyxoid neoplasms, the FUS FISH probe in 
      formalin-fixed paraffin-embedded tissue can be a useful ancillary technique for 
      diagnosis of this uncommon and deceptively bland tumor.
FAU - Patel, Rajiv M
AU  - Patel RM
AD  - Department of Pathology, University of Michigan, Ann Arbor, MI 48109, USA. 
      rajivpat@med.umich.edu
FAU - Downs-Kelly, Erinn
AU  - Downs-Kelly E
FAU - Dandekar, Monisha N
AU  - Dandekar MN
FAU - Fanburg-Smith, Julie C
AU  - Fanburg-Smith JC
FAU - Billings, Steven D
AU  - Billings SD
FAU - Tubbs, Raymond R
AU  - Tubbs RR
FAU - Goldblum, John R
AU  - Goldblum JR
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Am J Dermatopathol
JT  - The American Journal of dermatopathology
JID - 7911005
RN  - 0 (RNA-Binding Protein FUS)
SB  - IM
MH  - Chromosomes, Human, Pair 16/*genetics
MH  - Fibrosarcoma/*genetics/pathology
MH  - Gene Rearrangement
MH  - Humans
MH  - RNA-Binding Protein FUS/*genetics
MH  - Skin Neoplasms/*genetics/pathology
EDAT- 2011/03/15 06:00
MHDA- 2011/07/14 06:00
CRDT- 2011/03/15 06:00
PHST- 2011/03/15 06:00 [entrez]
PHST- 2011/03/15 06:00 [pubmed]
PHST- 2011/07/14 06:00 [medline]
AID - 00000372-201104000-00005 [pii]
AID - 10.1097/IAE.0b013e318176de80 [doi]
PST - ppublish
SO  - Am J Dermatopathol. 2011 Apr;33(2):140-3. doi: 10.1097/IAE.0b013e318176de80.