S. W. Chan, C. J. Lim, K. Guo, C. P. Ng, I. Lee et al., A role for TAZ in migration, invasion, and tumorigenesis of breast cancer cells, Cancer Res. 15 avr, vol.68, issue.8, pp.2592-2600, 2008.

V. Szablewski, A. Neuville, P. Terrier, M. Laé, R. Schaub et al., Adult sinonasal soft tissue sarcoma: Analysis of 48 cases from the French Sarcoma Group database: Adult Sinonasal Soft Tissue Sarcoma, The Laryngoscope. mars, vol.125, issue.3, pp.615-638, 2015.

W. J. Wong, A. Lauria, J. L. Hornick, X. S. Fletcher, J. A. Marino-enriquez et al.,

, Alternate PAX 3 -FOXO 1 oncogenic fusion in biphenotypic sinonasal sarcoma, BIPHENOTYPIC SINONASAL SARCOMA. Genes, Chromosomes and Cancer. janv, vol.55, issue.1, pp.25-34, 2016.

A. Kakkar, M. Rajeshwari, P. Sakthivel, M. C. Sharma, and S. C. Sharma, Biphenotypic sinonasal sarcoma: A series of six cases with evaluation of role of ?-catenin immunohistochemistry in differential diagnosis, Ann Diagn Pathol. avr, vol.33, pp.6-10, 2018.

L. M. Rooper, S. Huang, C. R. Antonescu, W. H. Westra, and J. A. Bishop, Biphenotypic sinonasal sarcoma: an expanded immunoprofile including consistent nuclear ?-catenin positivity and absence of SOX10 expression, Human Pathology. sept, vol.55, pp.44-50, 2016.

S. Andreasen, J. A. Bishop, H. Hellquist, J. Hunt, K. Kiss et al.,

, Biphenotypic sinonasal sarcoma: demographics, clinicopathological characteristics, molecular features, and prognosis of a recently described entity, Virchows Arch. 14 août, 2018.

S. R. Annavarapu, S. Cialfi, C. Dominici, G. K. Kokai, S. Uccini et al., Characterization of Wnt/b-catenin signaling in rhabdomyosarcoma, Laboratory Investigation, vol.93, p.10, 2013.

K. M. Kelly, R. B. Womer, P. H. Sorensen, Q. B. Xiong, and F. G. Barr, Common and variant gene fusions predict distinct clinical phenotypes in rhabdomyosarcoma, J Clin Oncol. mai, vol.15, issue.5, pp.1831-1837, 1997.

R. H. Edwards, J. Chatten, Q. B. Xiong, and F. G. Barr, Detection of gene fusions in rhabdomyosarcoma by reverse transcriptase-polymerase chain reaction assay of archival samples, Diagn Mol Pathol. avr, vol.6, issue.2, pp.91-98, 1997.

Y. Kang, M. Pekmezci, A. L. Folpe, A. Ersen, and A. E. Horvai, Diagnostic utility of SOX10 to distinguish malignant peripheral nerve sheath tumor from synovial sarcoma, including intraneural synovial sarcoma, Mod Pathol. janv, vol.27, issue.1, p.61, 2014.

B. Belyea, J. G. Kephart, J. Blum, D. G. Kirsch, and C. M. Linardic, Embryonic Signaling Pathways and Rhabdomyosarcoma: Contributions to Cancer Development and Opportunities for Therapeutic Targeting, 2012.

V. Avadhani, P. A. Loftus, D. Meltzer, B. Wang, and A. Tabaee, Epithelioid Hemangioendothelioma of the Maxillary Sinus, Head and Neck Pathology. juin, vol.10, issue.2, pp.229-261, 2016.

V. Y. Jo, A. Mariño-enríquez, C. Fletcher, and J. L. Hornick, , p.3

, Distinguishes Biphenotypic Sinonasal Sarcoma From Histologic Mimics, Am J Surg Pathol. 31 mai, 2018.

M. R. Tanas, J. R. Goldblum, and . Fluorescence, Situ Hybridization in the Diagnosis of Soft Tissue Neoplasms: A Review: Advances in Anatomic Pathology, vol.16, pp.383-91, 2009.

K. J. Fritchie, J. L. Wang, X. Graham, R. P. Torbenson, M. S. Lewis et al., Fusion gene profile of biphenotypic sinonasal sarcoma: an analysis of 44 cases

, Histopathology. déc, vol.69, issue.6, pp.930-936, 2016.

J. Coindre, Grading of soft tissue sarcomas: review and update, Arch Pathol Lab Med, vol.130, issue.10, pp.1448-53, 2006.

R. Cannon, R. Wiggins, B. Witt, Y. Dundar, T. Johnston et al., Imaging and Outcomes for a New Entity: Low-Grade Sinonasal Sarcoma with Neural and Myogenic Features, Journal of Neurological Surgery Reports. 15 févr, vol.78, issue.01, pp.15-24, 2017.

J. M. Coindre, Immunohistochemistry in the diagnosis of soft tissue tumours

. Histopathology, , 2003.

A. Cleven, A. Sannaa, G. A. Bruijn, I. B. Ingram, D. R. Van-de-rijn et al., Loss of H3K27 tri-methylation is a diagnostic marker for Malignant Peripheral Nerve Sheath Tumors and an indicator for an inferior survival, Mod Pathol. juin, vol.29, issue.6, p.90, 2016.

I. Schaefer, C. D. Fletcher, and J. L. Hornick, Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics, Mod Pathol. janv, vol.29, issue.1, p.13, 2016.

L. Guellec, S. Macagno, N. Velasco, V. Lamant, L. Lae et al., Loss of H3K27 trimethylation is not suitable for distinguishing malignant peripheral nerve sheath tumor from melanoma: a study of 387 cases including mimicking lesions, Mod Pathol. déc, vol.30, issue.12, pp.1677-87, 2017.

N. G. Hockstein, P. E. Dross, S. Farooqui, and I. N. Wilhelm, Low-grade sinonasal sarcoma with neural and myogenic features, Ear Nose Throat J. juin, vol.97, issue.6, pp.149-50, 2018.

M. Triki and L. Ayadi, Low-Grade Sinonasal Sarcoma With Neural and Myogenic Features, Arch Pathol Lab Med, vol.141, p.4, 2017.

K. A. Powers, L. M. Han, A. G. Chiu, and F. Z. Aly, Low-grade sinonasal sarcoma with neural and myogenic features-diagnostic challenge and pathogenic insight. Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology. mai, vol.119, pp.265-274, 2015.
DOI : 10.1016/j.oooo.2014.10.007

J. T. Lewis, D. Schembri-wismayer, E. A. Moore, J. G. Garcia, and M. L. Lonzo, Low-grade

, Sinonasal Sarcoma With Neural and Myogenic Features: A Clinicopathologic Analysis of 28 Cases, Am J Surg Pathol, vol.36, issue.4, p.9, 2012.

L. Guellec, S. Decouvelaere, A. Filleron, T. Valo, and I. ,

M. , Malignant Peripheral Nerve Sheath Tumor Is a Challenging Diagnosis: A Systematic Pathology Review, Immunohistochemistry, and Molecular Analysis in 160

, Patients From the French Sarcoma Group Database, The American Journal of Surgical Pathology. juill, vol.40, issue.7, p.908, 2016.

M. Binh, X. Sastre-garau, L. Guillou, G. De-pinieux, P. Terrier et al., MDM2 and CDK4 immunostainings are useful adjuncts in diagnosing welldifferentiated and dedifferentiated liposarcoma subtypes: a comparative analysis of 559 soft tissue neoplasms with genetic data, Am J Surg Pathol, vol.29, issue.10, pp.1340-1347, 2005.

J. Coindre,

, Bull Cancer, vol.97, pp.1337-1382, 2010.

T. Ueda, K. Aozasa, M. Tsujimoto, H. Hamada, H. Hayashi et al.,

, Multivariate analysis for clinical prognostic factors in 163 patients with soft tissue sarcoma. Cancer, vol.62, pp.1444-50, 1988.

L. Thompson and A. Franchi, New tumor entities in the 4th edition of the World Health Organization classification of head and neck tumors: Nasal cavity, paranasal sinuses and skull base, Virchows Archiv. mars, vol.472, issue.3, p.30, 2018.

J. A. Bishop, Newly Described Tumor Entities in Sinonasal Tract Pathology, Head and Neck Pathology. mars, vol.10, issue.1, p.31, 2016.

S. Huang, R. A. Ghossein, J. A. Bishop, L. Zhang, T. Chen et al., Novel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation, The American Journal of Surgical Pathology. janv, vol.40, issue.1, pp.51-60, 2016.

V. Y. Jo and C. Fletcher, Nuclear ?-Catenin Expression is Frequent in Sinonasal Hemangiopericytoma and Its Mimics, Head and Neck Pathology. juin, vol.11, issue.2, p.23, 2017.

A. H. Monsoro-burq, PAX transcription factors in neural crest development, Seminars in Cell & Developmental Biology. août, vol.44, pp.87-96, 2015.

M. Buckingham and F. Relaix, PAX3 and PAX7 as upstream regulators of myogenesis, Seminars in Cell & Developmental Biology. août, vol.44, pp.115-140, 2015.

H. Yoshida, M. Miyachi, K. Sakamoto, K. Ouchi, S. Yagyu et al.,

, PAX3-NCOA2 fusion gene has a dual role in promoting the proliferation and inhibiting the myogenic differentiation of rhabdomyosarcoma cells, Oncogene. déc, vol.33, issue.49, 2014.

D. H. Kraus, S. Dubner, L. B. Harrison, E. W. Strong, S. I. Hajdu et al.,

, Prognostic factors for recurrence and survival in head and neck soft tissue sarcomas, Cancer. 15 juill, vol.74, issue.2, p.702, 1994.

B. P. Rubin, K. Cooper, C. Fletcher, A. L. Folpe, F. H. Gannon et al.,

, Protocol for the examination of specimens from patients with tumors of soft tissue, Arch Pathol Lab Med. avr, vol.134, issue.4, pp.31-39, 2010.

B. M. Wenig, Recently Described Sinonasal Tract Lesions/Neoplasms: Considerations for the New World Health Organization Book, Head and Neck Pathology. mars, vol.8, issue.1, p.41, 2014.

H. Fudaba, Y. Momii, T. Hirano, H. Yamamoto, and M. Fujiki,

, Biphenotypic Sinonasal Sarcoma With Cerebral Hemorrhaging, J Craniofac Surg. 13 juill, 2018.

J. Sumegi, R. Streblow, R. W. Frayer, P. D. Cin, A. Rosenberg et al.,

, Recurrent (2;2) and (2;8) Translocations in Rhabdomyosarcoma without the Canonical PAX-FOXO1 fuse PAX3 to Members of the Nuclear Receptor Transcriptional Coactivator (NCOA) Family, Genes Chromosomes Cancer. mars, vol.49, issue.3, pp.224-260, 2010.

J. M. Mosquera, A. Sboner, L. Zhang, N. Kitabayashi, C. Chen et al.,

, Recurrent NCOA2 gene rearrangements in congenital/infantile spindle cell rhabdomyosarcoma, Genes, Chromosomes and Cancer. juin, vol.52, issue.6, p.50, 2013.

X. Wang, K. L. Bledsoe, R. P. Graham, Y. W. Asmann, D. S. Viswanatha et al., Recurrent PAX3-MAML3 fusion in biphenotypic sinonasal sarcoma, Nature Genetics. juill, vol.46, issue.7, pp.666-674, 2014.

J. Sumegi, R. Streblow, R. W. Frayer, P. D. Cin, A. Rosenberg et al.,

, and t(2;8) translocations in rhabdomyosarcoma without the canonical PAX-FOXO1 fuse PAX3 to members of the nuclear receptor transcriptional coactivator family, Genes, Chromosomes and Cancer, 2009.

X. Zhou and Q. Lei, Regulation of TAZ in cancer, Protein & Cell. août, vol.7, issue.8, p.61, 2016.

M. Debled, A. Ravaud, E. Stöckle, and B. B. Nguyen,

, Bull Cancer. juin, vol.84, issue.6, pp.653-63, 1997.

N. B. Bui, J. M. Coindre, J. Chauvergne, D. Maree, R. Denepoux et al.,

, J Chir (Paris). janv, vol.123, issue.1, pp.53-61, 1986.

J. K. Mito, X. Qian, L. A. Doyle, J. L. Hornick, and J. Vy, Role of Histone H3K27

, Trimethylation Loss as a Marker for Malignant Peripheral Nerve Sheath Tumor in Fine-Needle Aspiration and Small Biopsy Specimens, Am J Clin Pathol. 1 août, vol.148, issue.2, pp.179-89, 2017.

M. Kauke, A. Safi, A. Grandoch, H. Nickenig, J. Zöller et al., Sarcomas of the sinonasal tract, Head Neck. juin, vol.40, issue.6, pp.1279-86, 2018.

M. Pekmezci, A. K. Cuevas-ocampo, A. Perry, and A. E. Horvai, Significance of H3K27me3 loss in the diagnosis of malignant peripheral nerve sheath tumors, Mod Pathol. déc, vol.30, issue.12, pp.1710-1719, 2017.

A. Agaimy, F. Haller, and A. Hartmann,

, Pathologe. févr, vol.39, issue.1, pp.18-26, 2018.

P. G. Casali, N. Abecassis, S. Bauer, R. Biagini, S. Bielack et al., Soft tissue and visceral sarcomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up, Ann Oncol. 28 mai, 2018.

, Soft tissue sarcomas with complex genomic profiles. -PubMed -NCBI, vol.53

D. Sur,

, Soft tissue sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. -PubMed -NCBI, vol.54

D. Sur,

M. Johncilla and J. Vy, Soft tissue tumors of the sinonasal tract, Seminars in Diagnostic Pathology. mars, vol.33, issue.2, p.90, 2016.

M. Trojani, G. Contesso, J. M. Coindre, J. Rouesse, N. B. Bui et al.,

S. Boudjadi, B. Chatterjee, W. Sun, P. Vemu, and F. G. Barr, The expression and function of PAX3 in development and disease, Gene. août, vol.666, pp.145-57, 2018.

M. Murakami, J. Tominaga, R. Makita, Y. Uchijima, Y. Kurihara et al., Transcriptional activity of Pax3 is co-activated by TAZ, Biochem Biophys Res Commun. 13 janv, vol.339, issue.2, pp.533-542, 2006.

M. R. Gore, Treatment, outcomes, and demographics in sinonasal sarcoma: a systematic review of the literature, BMC Ear Nose Throat Disord, vol.18, p.4, 2018.

M. Wachtel and B. W. Schäfer, Unpeaceful roles of mutant PAX proteins in cancer, Seminars in Cell & Developmental Biology. août, vol.44, pp.126-160, 2015.

E. B. Stelow and J. A. Bishop, Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Tumors of the Nasal Cavity, Paranasal Sinuses and Skull Base, Head and Neck Pathology. mars, vol.11, issue.1, p.15, 2017.

D. El-demellawy, J. Mcgowan-jordan, J. De-nanassy, E. Chernetsova, and A. Nasr, Update on molecular findings in rhabdomyosarcoma, Pathology. avr, vol.49, issue.3, pp.238-284, 2017.

V. Y. Jo and C. Fletcher, WHO classification of soft tissue tumours: an update based on the, Pathology. févr, vol.46, issue.2, pp.95-104, 2013.

J. M. Coindre, F. Collin, A. V. Decouvelaere, D. Ranchère-vince, and N. Saint,

A. Aubain and . Neuville, Enseignement Post-Universitaire : Tumeurs des Tissus mous

. Paris, , 2015.

C. Fletcher, J. A. Bridge, P. Hogendoorn, and F. Mertens, WHO classification of tumours of soft tissue and bone. World Health Organization, 2013.

H. Ge, K. Liu, and T. Juan, FusionMap : detecting fusion genes from nextgeneration sequencing data at base-pair resolution, Bioinformatics, vol.27, pp.1922-1930, 2011.

M. Benelli and . Pescuccic, Discovering chimeric transcripts in paired-end RNAseq data by using EricScript, Bioinformatics, vol.28, pp.3232-3239, 2012.

I. Hostein, M. Andraud-fregeville, and L. Guillou, Rhabdomyosarcoma: value of myogenin expression analysis and molecular testing in diagnosing the alveolar subtype. An analysis of 109 paraffin-embedded specimen, Cancer, vol.101, pp.2817-2841, 2004.

C. Sun, D. Mello, V. , and M. A. , Common and distinctive functions of the Hippo effectors Taz and Yap in skeletal muscle stem cell function, Stem Cells, 2017.

N. Wwtr1, The tissue slides were deparaffinized in xylene, hydrated in alcohol, and baked in a microwave (30 min in Trisbuffer, Endogenous peroxidase was blocked

, Staining was performed on the Benchmark ultra-automated stainer (Ventana) using diamino-benzidine as chromogen

, EMA (clone, vol.29, pp.pre-diluted

. Dako,

S. Aldrich, S. Louis, and . Mo,

, Cell Marque

. Ventana, ); beta-catenin (clone 14, pre-diluted, Cell Marque

. Biosb,

, H3K27Me3 (clone C36B11, dilution 1

, Cell Signaling Technology

, For myogenin, MyoD1, SOX10, H3K27me3 beta-catenin and PAX3

, embedded 4-µm-thick sections were performed by applying custom probes using bacterial artificial chromosomes (BACs), flanking PAX3, MAML3, NCOA1 genes according to the procedure previously described (3). FISH analysis for FOXO1, NCOA2 and WWTR1 was performed with 4-µm sections of formalin-fixed paraffin-embedded tissue and the Histology FISH Accessory Kit (Agilent K5799, Fluorescence In situ Hybridization FISH analyses on interphase nuclei from paraffin

, NCOA2 (Empire genomics, EG-NCOA2BA-20-ORGR) and WWTR1(Zytovision, Zytolight spec Z-2212-50)

, RNA extraction for Real-Time RT-PCR and Paired-end RNA-sequencing

, Total RNA was extracted from formalin-fixed paraffin-embedded tissue section using Trizol reagent (Thermo Fisher Scientific, Courtaboeuf, France) according to the manufacturers' recommendations. DNA was removed using RNase-free DNase set (Qiagen) followed by a second Trizol extraction. The yield of total RNA obtained was evaluated using NanoDrop

, Reverse Transcription-Polymerase Chain Reaction analysis for PAX3-MAML3, PAX3-FOXO1, PAX3-NCOA2 and PAX3-WWTR1 fusion genes

. Real-time-rt-pcr, was performed for PAX3-MAML3 and PAX3-FOXO1 fusion genes. For PAX3-MAML3 gene fusion, the following primers and probe were used, using Taqman technology according to the technique previously described by Hostein et al (6)

A. Cca-g-, For PAX3-FOXO1 gene fusion, the following primers and probe were used: PAX3 forward primer: 5'-TTG GCA ATG GCC TCT CAC C-3' FOXO1 reverse primer: 5'

, One microgram of total RNA was reverse-transcribed in cDNA with the High Capacity cDNA Reverse Transcription Kit with RNase Inhibitor (Invitrogen, cat. No. 4374966). PCR was performed using the AmpliTaq Gold? DNA Polymerase kit (Applied Biosystems?, cat. No. 4311806) on 50 ng of cDNA with the following primers: PAX3_FWD: 5' GACCCTGTCACAGGCTAC 3' and WWTR1_REV: 5' TCTGCTGGCTCAGGGTACT 3' and for the reciprocal transcript

, The Touchdown 60°C program was used (TD 60°C; two cycles at 60°C, followed by two cycles at 59°C, two cycles at 58°C, three cycles at 57°C, three cycles at 56°C, four cycles at 55°C, four cycles at 54°C, five cycles at 53°C, and finally 10 cycles at 52°C), For PAX3-NCOA2 validation, the following primers were used: PAX3_FWD: 5' CTTTGTGCCTCCGTCGGG 3' and NCOA2_REV: 5'CTCGTGTCTGGGAAAAGCTG 3

, After purification with the Big Dye References, US77702), and sequencing reactions were performed with the Big Dye Terminator V1.1 Kit (Applied Biosystems

J. T. Lewis, A. M. Oliveira, A. G. Nascimento, D. Schembri-wismayer, and E. A. Moore,

K. D. Olsen, Low-grade sinonasal sarcoma with neural and myogenic features: a clinicopathologic analysis of 28 cases, Am J Surg Pathol, vol.36, pp.517-542, 2012.

X. Wang, K. L. Bledsoe, R. P. Graham, Y. W. Asmann, D. S. Viswanatha et al., Recurrent PAX3-MAML3 fusion in biphenotypic sinonasal sarcoma, Nat Genet, vol.46, pp.666-674, 2014.
DOI : 10.1038/ng.2989

URL : http://europepmc.org/articles/pmc4236026?pdf=render

S. Huang, R. A. Ghossein, J. A. Bishop, L. Zhang, T. Chen et al., Novel PAX3-NCOA1 fusions in biphenotypic sinonasal sarcoma with focal rhabdomyoblastic differentiation, Am J Surg Pathol, vol.40, pp.51-60, 2016.
DOI : 10.1097/pas.0000000000000492

URL : http://europepmc.org/articles/pmc4679641?pdf=render

W. J. Wong, A. Lauria, J. L. Hornick, X. S. Fletcher, J. A. Marino-enriquez et al.,

, Alternate PAX3-FOXO1 oncogenic fusion in biphenotypic sinonasal sarcoma, Genes Chromosomes Cancer, vol.55, pp.25-34, 2016.

K. J. Fritchie, J. L. Wang, X. Graham, R. P. Torbenson, M. S. Lewis et al., Fusion gene profile of biphenotypic sinonasal sarcoma: an analysis of 44 cases, Histopathology, vol.69, pp.930-936, 2016.

I. Hostein, M. Andraud-fregeville, and L. Guillou, Rhabdomyosarcoma: value of myogenin expression analysis and molecular testing in diagnosing the alveolar subtype. An analysis of 109 paraffin-embedded specimen, Cancer, vol.101, pp.2817-2841, 2004.

H. Ge, K. Liu, and T. Juan, FusionMap: detecting fusion genes from nextgeneration sequencing data at base-pair resolution, Bioinformatics, vol.27, pp.1922-1930, 2011.

C. Trapnell, A. Roberts, and L. Goff, Differential gene and transcript expression analysis of RNA-seq experiments with TopHat and Cufflinks, Nature protocols, vol.7, pp.562-578, 2012.

S. Lagstad, S. Zhao, and A. M. Hoff, chimeraviz: a tool for visualizing chimeric RNA, Bioinformatics, vol.33, pp.2954-2960, 2017.

D. Nicorici, . Sm, H. Edgren, S. Kangaspeska, A. Murumagi et al., FusionCatcher -a tool for finding somatic fusion genes in paired-end RNAsequencing data. bioRxiv, 2014.

M. Benelli, C. Pescucci, and G. Marseglia, Discovering chimeric transcripts in pairedend RNA-seq data by using EricScript, Bioinformatics, vol.28, pp.3232-3239, 2012.

L. M. Rooper, S. Huang, C. R. Antonescu, W. H. Westra, and J. A. Bishop,

, Biphenotypic sinonasal sarcoma: an expanded immunoprofile including consistent nuclear ?-catenin positivity and absence of SOX10 expression, Hum Pathol, vol.55, pp.44-50, 2016.

V. Y. Jo, A. Mariño-enriquez, C. Fletcher, and J. L. Hornick, Expression of PAX3 distinguishes biphenotypic sinonasal sarcoma from histologic mimics, Am J Surg Pathol, vol.42, pp.1275-85, 2018.

J. R. Misra and K. D. Irvine, The Hippo signaling network and its biological functions, Annu Rev Genet, 2018.

C. Errani, L. Zhang, and Y. S. Sung, A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites, Gens Chromosomes Cancer, vol.50, pp.644-53, 2011.

C. Sun, D. Mello, V. , and M. A. , Common and distinctive functions of the Hippo effectors Taz and Yap in skeletal muscle stem cell function, Stem Cells, vol.35, pp.1958-72, 2017.