Round cell tumor with a myxoid matrix harboring a PHF1-TFE3 fusion: Myoepithelial neoplasm or ossifying fibromyxoid tumor?
Pathol Res Pract
; 225: 153578, 2021 Sep.
Article
em En
| MEDLINE
| ID: mdl-34391181
ABSTRACT
Myoepithelial tumors arising in soft tissue are uncommon and mostly manifest a benign clinical course, although a malignant form does exist. An EWSR1 gene rearrangement is a common event in these tumors. Ossifying fibromyxoid tumor, a rare soft tissue neoplasm of uncertain differentiation, may have overlapping histologic and immunophenotypic features with myoepithelial tumors, but frequently harbors a PHF1 gene rearrangement. Interestingly, a PHF1-TFE3 fusion has been recently reported in both entities. Here we report a case of a malignant soft tissue tumor demonstrating myoepithelial differentiation and harboring a PHF1-TFE3 fusion. Despite being slow-growing and lacking significant cytologic atypia at initial presentation, the patient deteriorated rapidly with local recurrence and distant metastases. A discussion of the potential clinicopathologic implications of a PHF1-TFE3 fusion in these entities is also developed.
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Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias de Tecidos Moles
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Fibroma Ossificante
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Mioepitelioma
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Proteínas de Ligação a DNA
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Fatores de Transcrição de Zíper de Leucina e Hélice-Alça-Hélix Básicos
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Fusão Oncogênica
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Proteínas do Grupo Polycomb
Limite:
Female
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Humans
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Middle aged
Idioma:
En
Revista:
Pathol Res Pract
Ano de publicação:
2021
Tipo de documento:
Article