Extraosseous Ewing sarcoma with foci of neuroblastoma-like differentiation associated with EWSR1(Ewing sarcoma breakpoint region 1)/FLI1 translocation without prior chemotherapy.
Hum Pathol
; 43(10): 1772-6, 2012 Oct.
Article
en En
| MEDLINE
| ID: mdl-22703924
Peripheral primitive neuroectodermal tumor/Ewing sarcoma and neuroblastoma are distinct malignant tumors belonging to the group of undifferentiated solid pediatric tumors. We report a case of a 14-year-old adolescent girl who presented with a right lower quadrant mass. At surgery, a mobile retroperitoneal mass was entirely removed. Histologic evaluation revealed 2 distinct components; the first, consisting of sheets of undifferentiated cells, was CD99+ and CD56-, whereas the second, consisting of multiple foci of neuropil and maturing neuroblasts, was CD99- and CD56+. Fluorescence in situ hybridization analysis revealed the presence of EWSR1/FLI1 translocation in both histologic distinct components. MYCN (myelocytomatosis viral related oncogene, neuroblastoma derived) was not amplified. The tests for t(11;22) and t(21;22) performed by reverse transcription-polymerase chain reaction were negative. The final diagnosis corresponds to an extraosseous Ewing sarcoma with foci of neuroblastoma-like differentiation. This is the first case, documented by molecular studies, in which neuroblastoma-like differentiation has been noted in primitive neuroectodermal tumor/Ewing sarcoma without prior chemotherapy.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Neoplasias Retroperitoneales
/
Sarcoma de Ewing
/
Proteínas de Unión a Calmodulina
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Proteínas de Unión al ARN
/
Proteína Proto-Oncogénica c-fli-1
Tipo de estudio:
Risk_factors_studies
Límite:
Adolescent
/
Female
/
Humans
Idioma:
En
Revista:
Hum Pathol
Asunto de la revista:
PATOLOGIA
Año:
2012
Tipo del documento:
Article
País de afiliación:
Canadá
Pais de publicación:
Estados Unidos