关键词: Mesenchymal chondrosarcoma NKX3.1

Mesh : 12E7 Antigen / metabolism Adult Aged Biomarkers, Tumor / metabolism Biopsy / methods Bone Neoplasms / pathology Cell Differentiation Cell Nucleus / metabolism pathology Child Chondrosarcoma, Mesenchymal / diagnosis metabolism Desmoplastic Small Round Cell Tumor / diagnosis Diagnosis, Differential Female Homeobox Protein Nkx-2.2 Homeodomain Proteins / metabolism Humans Hyaline Cartilage / pathology Immunohistochemistry / methods Male Middle Aged NFATC Transcription Factors / metabolism Nuclear Proteins RNA-Binding Protein EWS / metabolism Rhabdomyosarcoma / diagnosis S100 Proteins / metabolism SOX9 Transcription Factor / metabolism Sarcoma, Ewing / diagnosis Sarcoma, Small Cell / diagnosis Transcription Factors / metabolism

来  源:   DOI:10.1016/j.anndiagpath.2020.151660   PDF(Sci-hub)

Abstract:
BACKGROUND: Mesenchymal chondrosarcoma is a rare subtype of chondrosarcoma. The tumor has a characteristic bimorphic pattern with areas of poorly differentiated small round cell component and interspersed islands of well differentiated hyaline cartilage. Histological diagnosis of mesenchymal chondrosarcoma is very challenging especially in small biopsies when tumor presents with little cartilaginous component. In such cases, it is very difficult to distinguish mesenchymal chondrosarcoma from other round blue cell tumors like Ewing\'s sarcoma, rhabdomyosarcoma, small cell osteosarcoma and desmoplastic round blue cell tumor. Immunohistochemically, mesenchymal chondrosarcoma stains positive for NKX2.2, CD99, S100 and SOX9. This immunoprofile is non-specific and overlaps with other round blue cell tumors. Till recently, there was no reliable immunohistochemical marker to differentiate mesenchymal chondrosarcoma from other round blue cell tumors. NKX3.1, though widely used as a diagnostic biomarker for prostatic adenocarcinoma, has been recently proposed by Yoshida et al. (2020) as a unique marker of mesenchymal chondrosarcoma and EWSR1-NFATC2 sarcoma.
OBJECTIVE: The aim of our study was to further explore utility of NKX3.1 as a diagnostic marker of mesenchymal chondrosarcoma.
METHODS: We applied NKX3.1 immunohistochemistry to 21 cases of mesenchymal chondrosarcoma and 32 cases of other round blue cell tumors.
RESULTS: 14 out of 21 cases (66.7%) of mesenchymal chondrosarcoma stained positive for NKX3.1 with nuclear expression in small round component. Cartilaginous component was predominantly negative. All other round blue cell tumors showed negative results.
CONCLUSIONS: Based on our study results we suggest that NKX3.1 is a useful immunohistochemical marker in differentiating mesenchymal chondrosarcoma from its histological mimics.
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