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Primitive myxoid mesenchymal tumor of infancy presenting with an abdominal and thoracic mass misdiagnosed as neuroblastoma: A case report.
Yener, Sevim; Tosun, Ilkay; Yildiz, Zeliha A; Zemheri, Itir Ebru; Ilçe, Zekeriya.
Afiliación
  • Yener S; Department of Pediatric Urology, Health Sciences University Umraniye Training and Research Hospital, Umraniye, Istanbul, Turkey.
  • Tosun I; Department of Pathology, Health Sciences University Umraniye Training and Research Hospital, Umraniye, Istanbul, Turkey.
  • Yildiz ZA; Department of Pediatric Surgery, Health Sciences University Umraniye Training and Research Hospital, Umraniye, Istanbul, Turkey.
  • Zemheri IE; Department of Pathology, Health Sciences University Umraniye Training and Research Hospital, Umraniye, Istanbul, Turkey.
  • Ilçe Z; Department of Pediatric Surgery, Health Sciences University Umraniye Training and Research Hospital, Umraniye, Istanbul, Turkey.
Article en En | MEDLINE | ID: mdl-39011617
ABSTRACT
ABSTRACT Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a rare soft tissue tumor that has been recently described in only a few cases. A 10-month-old boy was admitted with a mass filling the abdomen and right hemothorax. Gross examination revealed a fragile, and occasionally gelatinous and rubbery mass. On microscopic examination, there were fibrillary off-white appearance with occasionally myxoid microcystic areas. Immunohistochemical examination results were as follows BCOR (+), and SATB2 (+) with diffuse nuclear expression, Desmin (-), CD34 (-), S-100 (-), MyoD1 (-), Myogenin (-). The next-generation sequencing showed internal tandem duplication of the BCOR exon 15. In conclusion, PMMTI is a distinctive entity with unique characteristics. However, it can be clinically confused with other childhood malignancies such as neuroblastoma. Recurrence can be seen despite chemotherapy in patients with microscopic residue.

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Indian J Pathol Microbiol Año: 2024 Tipo del documento: Article País de afiliación: Turquía Pais de publicación: India

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Indian J Pathol Microbiol Año: 2024 Tipo del documento: Article País de afiliación: Turquía Pais de publicación: India