Adult sinonasal soft tissue sarcoma: Analysis of 48 cases from the French Sarcoma Group database

Szablewski, Vanessa; Neuville, Agnès; Terrier, Philippe; Laé, Marick; Schaub, Roxane; Garrel, R.; Coindre, Jean‐Michel; Costes, Valérie

doi:10.1002/lary.24910

Cited by 41 publications

(44 citation statements)

References 34 publications

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“…1,2 Sinonasal tract sarcomas comprise less than 10% of head and neck sarcomas and tend to be more aggressive due to their proximity to the eyes and brain. 3,4 The histologic classification is an important prognostic factor, with rhabdomyosarcoma (RMS) conferring the most dismal outcome, with 30% survival rate at 5 years. 3,4 Given the rarity and wide morphologic spectrum of sinonasal sarcomas, the histologic diagnosis is often challenging, especially on limited and fragmented biopsies.…”

Section: Introductionmentioning

confidence: 99%

“…1,4 Recently, Lewis and colleagues described a new pathologic entity using the terminology: ‘low grade sinonasal sarcoma with neural and myogenic features (LGSSNMF)’, since it histologically resembled adult fibrosarcoma or monophasic synovial sarcoma, but expressed a dual neural and myogenic immunophenotype. 5 LGSSNMF was subsequently renamed by the same group as biphenotypic sinonasal sarcoma (BSNS), being characterized by a recurrent t(2;4)(q35;q31.1) translocation, resulting in a PAX3-MAML3 fusion.…”

Section: Introductionmentioning

confidence: 99%

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Novel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation

Huang

Ghossein

Bishop

et al. 2016

American Journal of Surgical Pathology

124

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Sarcomas arising in the sinonasal region are uncommon and encompass a wide variety of tumor types, including the newly described biphenotypic sinonasal sarcoma (BSNS), which is characterized by a monomorphic spindle cell proliferation with dual neural and myogenic phenotypes. Most BSNSs harbor a pathognomonic PAX3-MAML3 fusion driven by t(2;4)(q35;q31.1), while the alternative fusion partner gene remains unidentified in a subset of PAX3-rearranged cases. As NCOA1 on 2p23 is a known partner in PAX3-related fusions in other tumor types (i.e. alveolar rhabdomyosarcoma), we investigated its status by FISH and RT-PCR assays in 2 BSNS cases showing only PAX3 gene rearrangements. Novel PAX3-NCOA1 fusions were identified in these two index cases showing an inv(2)(q35p23) by FISH and confirmed by RT-PCR. Five additional BSNS cases with typical morphology were studied by FISH, revealing a PAX3-MAML3 fusion in 4 cases and only PAX3 rearrangement in the remaining case without abnormalities in MAML3 or NCOA1 gene. Except for one case with surface ulceration, all other tumors lacked increased mitotic activity or necrosis, and all cases immunohistochemically co-expressed S100 protein and actin, but lacked SOX10 reactivity. Interestingly, the two PAX3-NCOA1-positive cases showed desmin reactivity and displayed a small component of rhabdomyoblastic cells, which were not seen in the more common PAX3-MAML3 fusion cases. In conclusion, we report a novel PAX3-NCOA1 fusion in BSNS, which appears to be associated with focal rhabdomyoblastic differentiation and should be distinguished from PAX3-NCOA1-positive alveolar rhabdomyosarcoma or malignant Triton tumor. SOX10 immunohistochemistry is a useful marker in distinguishing BSNS from peripheral nerve sheath tumors.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Novel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation

Huang

Ghossein

Bishop

et al. 2016

American Journal of Surgical Pathology

124

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“…MPNST of the nasopharynx and sinonasal cavity is rare, with few case reports and series in the literature (31,32). Most MPNSTs arise in deep-seated locations, and a subset arises in the setting of neurofibromatosis type 1 (NF1).…”

Section: Malignant Peripheral Nerve Sheath Tumor (And Neurofibroma)mentioning

confidence: 98%

Soft tissue tumors of the sinonasal tract

Johncilla

2016

Seminars in Diagnostic Pathology

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“…[26][27][28] Clinical. Rhabdomyosarcoma, although uncommon, is still the most common sinonasal sarcoma, [29][30][31] with a slight female to male predilection (1.2:1). 32 In adults, alveolar rhabdomyosarcoma is the most common subtype in the sinonasal tract.…”

Section: Mesenchymal Chondrosarcomamentioning

confidence: 99%

“…Rhabdomyosarcoma is considered a systemic disease, managed with multimodality therapies including surgery, chemotherapy and radiation, 40 frequently associated with adverse late sequela of treatment. 41 There is an overall poor prognosis of sinonasal tract alveolar rhabdomyosarcoma (5-year survival 30-40%), 29,35,[41][42][43][44] with patients frequently showing regional and/or distant metastases, although young patients (5-year survival 62.5%) tend to have a better prognosis. 28,32,43 Sinonasal Undifferentiated Carcinoma (SNUC) SNUC is a rare tumor, lacking glandular or squamous features, and is not otherwise classifiable.…”

Section: Mesenchymal Chondrosarcomamentioning

confidence: 99%

Small round blue cell tumors of the sinonasal tract: a differential diagnosis approach

Thompson

2017

Modern Pathology

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One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. Biopsies are usually small and limited, resulting in considerable diagnostic difficulty for practicing surgical pathologists. These tumors share several overlapping histologic and immunophenotypic findings while also showing considerable variation within and between cases. Specific tumor site of origin, imaging findings, and clinical findings must be combined with the histology and pertinent ancillary studies if the correct diagnosis is to be reached. Discrimination between neoplasms is critical as there are significant differences in therapy and overall outcome. It is important to have a well developed differential diagnosis for this category of tumors, where each of the diagnoses is considered, evaluated, and either confirmed or excluded from further consideration. In an undifferentiated tumor, showing a small round blue cell morphology, using the mnemonic 'MR SLEEP' helps to highlight tumors to consider: melanoma, mesenchymal chondrosarcoma, rhabdomyosarcoma, sinonasal undifferentiated carcinoma, squamous cell carcinoma (including NUT carcinoma), small cell osteosarcoma, lymphoma, esthesioneuroblastoma (olfactory neuroblastoma), Ewing sarcoma/primitive neuroectodermal tumor, pituitary adenoma, and plasmacytoma. A panel of pertinent immunohistochemistry studies, histochemistries and/or molecular tests should aid in reaching a diagnosis, especially when taking the pattern and intensity of reactions into consideration.

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Adult sinonasal soft tissue sarcoma: Analysis of 48 cases from the French Sarcoma Group database

Abstract: These results suggest that sinonasal tract should be considered as an unfavorable site for rhabdomyosarcoma. Moreover, surgery should always be considered in treatment.

Cited by 41 publications

References 34 publications

Novel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation

Novel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation

Soft tissue tumors of the sinonasal tract

Small round blue cell tumors of the sinonasal tract: a differential diagnosis approach

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