PMID- 27137987
OWN - NLM
STAT- MEDLINE
DCOM- 20170822
LR  - 20181113
IS  - 1532-8392 (Electronic)
IS  - 0046-8177 (Print)
IS  - 0046-8177 (Linking)
VI  - 55
DP  - 2016 Sep
TI  - Biphenotypic sinonasal sarcoma: an expanded immunoprofile including consistent 
      nuclear beta-catenin positivity and absence of SOX10 expression.
PG  - 44-50
LID - S0046-8177(16)30049-1 [pii]
LID - 10.1016/j.humpath.2016.04.009 [doi]
AB  - Biphenotypic sinonasal sarcoma (BSNS) is a recently recognized low-grade sarcoma 
      that exhibits both neural and myogenic differentiation. This unique dual 
      phenotype stems from recurrent rearrangements in PAX3, a transcription factor 
      that promotes commitment along both lineages. While identification of PAX3 
      rearrangements by fluorescence in situ hybridization (FISH) can confirm a BSNS 
      diagnosis, this assay is not widely available. This study evaluates whether an 
      expanded immunohistochemical panel can facilitate recognition of BSNS without 
      molecular analysis. Eleven cases of BSNS were identified from the surgical 
      pathology archives of two academic medical centers. In 8 cases, the diagnosis was 
      confirmed by FISH using custom probes for PAX3. In 3 cases, FISH failed but 
      histologic and immunophenotypic findings were diagnostic for BSNS. All 11 BSNS 
      (100%) were at least focally positive for S100 as well as calponin and/or smooth 
      muscle actin. In addition, 10 (91%) of 11 expressed nuclear beta-catenin, 8 (80%) of 
      10 expressed factor XIIIa, 4 (36%) of 11 expressed desmin, and 3 (30%) of 10 
      expressed myogenin. All 11 tumors were negative for SOX10. While no single marker 
      resolves immunohistochemical overlap between BSNS and its histologic mimickers 
      such as nerve sheath tumors, an extended immunohistochemical panel that includes 
      beta-catenin and SOX10 helps to support the diagnosis of BSNS without the need for 
      gene rearrangement studies.
CI  - Copyright (c) 2016 Elsevier Inc. All rights reserved.
FAU - Rooper, Lisa M
AU  - Rooper LM
AD  - Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD 
      21231, USA.
FAU - Huang, Shih-Chiang
AU  - Huang SC
AD  - Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY 
      10065, USA.
FAU - Antonescu, Cristina R
AU  - Antonescu CR
AD  - Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY 
      10065, USA.
FAU - Westra, William H
AU  - Westra WH
AD  - Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD 
      21231, USA; Department of Otolaryngology-Head and Neck Surgery, The Johns Hopkins 
      Medical Institutions, Baltimore, MD 21231, USA; Department of Oncology, The Johns 
      Hopkins Medical Institutions, Baltimore, MD 21231, USA.
FAU - Bishop, Justin A
AU  - Bishop JA
AD  - Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD 
      21231, USA; Department of Otolaryngology-Head and Neck Surgery, The Johns Hopkins 
      Medical Institutions, Baltimore, MD 21231, USA; Department of Oncology, The Johns 
      Hopkins Medical Institutions, Baltimore, MD 21231, USA. Electronic address: 
      jbishop@jhmi.edu.
LA  - eng
GR  - P30 CA008748/CA/NCI NIH HHS/United States
GR  - P50 DE019032/DE/NIDCR NIH HHS/United States
PT  - Journal Article
PT  - Multicenter Study
PT  - Research Support, N.I.H., Extramural
DEP - 20160429
PL  - United States
TA  - Hum Pathol
JT  - Human pathology
JID - 9421547
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (CTNNB1 protein, human)
RN  - 0 (MYOG protein, human)
RN  - 0 (Myogenin)
RN  - 0 (PAX3 Transcription Factor)
RN  - 0 (PAX3 protein, human)
RN  - 0 (SOX10 protein, human)
RN  - 0 (SOXE Transcription Factors)
RN  - 0 (beta Catenin)
RN  - EC 2.3.2.13 (Factor XIIIa)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Baltimore
MH  - Biomarkers, Tumor/*analysis/genetics
MH  - Cell Nucleus/*chemistry/immunology/pathology
MH  - Factor XIIIa/analysis
MH  - Female
MH  - Gene Rearrangement
MH  - Humans
MH  - Immunohistochemistry
MH  - Immunophenotyping/methods
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Middle Aged
MH  - Myogenin/analysis
MH  - Nasal Cavity/*chemistry/immunology/pathology
MH  - Neoplasm Grading
MH  - Neoplasms, Complex and Mixed/*chemistry/genetics/immunology/pathology
MH  - New York City
MH  - PAX3 Transcription Factor/genetics
MH  - Paranasal Sinus Neoplasms/*chemistry/genetics/immunology/pathology
MH  - Phenotype
MH  - Predictive Value of Tests
MH  - SOXE Transcription Factors/*analysis
MH  - Sarcoma, Synovial/*chemistry/genetics/immunology/pathology
MH  - beta Catenin/*analysis
PMC - PMC4981530
MID - NIHMS783079
OTO - NOTNLM
OT  - Biphenotypic sinonasal sarcoma
OT  - Immunohistochemistry
OT  - SOX10
OT  - Sinonasal tract
OT  - beta-Catenin
COIS- The authors have no conflicts of interest to declare.
EDAT- 2016/05/04 06:00
MHDA- 2017/08/23 06:00
PMCR- 2017/09/01
CRDT- 2016/05/04 06:00
PHST- 2016/02/29 00:00 [received]
PHST- 2016/04/01 00:00 [revised]
PHST- 2016/04/15 00:00 [accepted]
PHST- 2016/05/04 06:00 [entrez]
PHST- 2016/05/04 06:00 [pubmed]
PHST- 2017/08/23 06:00 [medline]
PHST- 2017/09/01 00:00 [pmc-release]
AID - S0046-8177(16)30049-1 [pii]
AID - 10.1016/j.humpath.2016.04.009 [doi]
PST - ppublish
SO  - Hum Pathol. 2016 Sep;55:44-50. doi: 10.1016/j.humpath.2016.04.009. Epub 2016 Apr 
      29.