关键词: Ewing's sarcoma R1 rearrangement Ewing's sarcoma/primitive neuroectodermal tumor NKX2.2 ileum

Mesh : 12E7 Antigen / metabolism Adolescent Adult Child Female Homeobox Protein Nkx-2.2 Homeodomain Proteins / metabolism Humans Immunochemistry In Situ Hybridization, Fluorescence Male Middle Aged Neuroectodermal Tumors, Primitive / diagnosis pathology surgery Nuclear Proteins Proto-Oncogene Protein c-fli-1 / metabolism RNA-Binding Protein EWS / genetics Sarcoma, Ewing / diagnosis pathology surgery Soft Tissue Neoplasms / diagnosis pathology surgery Transcription Factors / metabolism Young Adult Zebrafish Proteins

来  源:   DOI:10.4103/IJPM.IJPM_546_17

Abstract:
Rare cases of Ewing\'s sarcoma/primitive neuroectodermal tumors (EWS/PNETs) arising from mesenteric tissue have been reported. This report describes an EWS/PNET in a 25-year-old woman who presented with abdominal pain lasting 3 days. Radiologic evaluation revealed a 9 cm × 6 cm homogeneous mass in the lower abdomen with homogeneous enhancement and invasion of the ileum. Surgical resection was completed during exploratory laparotomy. Immunohistochemically, the tumor cells revealed CD99, friend leukemia virus integration-1 and NKX2.2 (NK2 Homeobox 2, a protein coding gene) and subsequently showed EWSR1 rearrangement. The histological feature, immunohistochemical results and genetic fluorescence in situ hybridization analysis of this case were confirming the diagnosis of EWS/PNET. Adjuvant chemotherapy was suggested, but the patient was lost to follow-up.
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