Definition
Primary tumours of the uterus with histological and immunohistochemical features of neuroectodermal differentiation. The lack of EWSR1 gene rearrangement indicates that they have a closer affinity to central neuroectodermal tumours (medulloblastoma and olfactory neuroblastoma) than to peripheral PNETs.
These tumours occur across a wide age range in adult women, most commonly over the age of 50 years.
Presentation is with vaginal bleeding, uterine enlargement, pelvic mass or pain.
The cells form sheets with variable degrees of nesting or trabecular architecture. Features of neuroectodermal differentiation include rosettes, a fibrillary background, ganglion cells and astrocyte-like cells.
There may be co-existing endometrial hyperplasia, endometrioid adenocarcinoma, malignant mixed Mullerian tumour or adenosarcoma.
Cytokeratin cocktail of AE1/AE3, Cam5.2, MNF116, keratin8&18 |
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Neu-N |
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0/1 |
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2/4 |
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0/4 |
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Molecular studies
None of 11 cases examined showed EWSR1 gene rearrangement.
These are aggressive tumours.
©SMUHT/PW Bishop