Biphenotypic sinonasal sarcoma: an expanded immunoprofile including consistent nuclear ß-catenin positivity and absence of SOX10 expression.
Hum Pathol
; 55: 44-50, 2016 09.
Article
em En
| MEDLINE
| ID: mdl-27137987
ABSTRACT
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 ß-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 ß-catenin and SOX10 helps to support the diagnosis of BSNS without the need for gene rearrangement studies.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias dos Seios Paranasais
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Biomarcadores Tumorais
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Núcleo Celular
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Neoplasias Complexas Mistas
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Sarcoma Sinovial
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Beta Catenina
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Fatores de Transcrição SOXE
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Cavidade Nasal
Tipo de estudo:
Clinical_trials
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Prognostic_studies
Limite:
Aged80
País/Região como assunto:
America do norte
Idioma:
En
Revista:
Hum Pathol
Ano de publicação:
2016
Tipo de documento:
Article