Fibrillary to dense stroma with sharp borders although some fragments of matrix have long delicate spicules that blend with the background
sulphated glycosaminoglycan’s stain, without staining to hyaluronic acid
Alcian Blue PH 1
S100 Protein: Positive
EMA and cytokeratin’s (AE1AE3, CAM5.2, CEA): Negative
Neuroendocrine markers: Positive
Rudimentary cell junctions
Cytoplasm with: well developed endoplasmic reticulum, Golgi and many mitochondria.
RER distended and filled with parallel microtubules of 24-nm
t (9; 22) (q22-31; 11-12) - EWS/TEC gene fusion-(75%)
t(9;17)- described in some variants
Muscle differentiation (immunohistochemical and electron microscopy)
Lipoblasts with scalloped nuclei clustered around branching capillaries
Also has metachromatic myxoid stroma
Also has tumour cells single or arranged in cords
Presence of physaliphorous cells
Epithelial markers : Positive
Extra renal rhabdoid tumour
Rarely a myxoid lesion
Prominent eosinophilic nucleoli
Eosinophilic para nuclear cytoplasmic inclusions.
Lung metastasis are frequent at presentation, in high grade tumours
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