Neuroectodermal tumour of the uterus of central type

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.

Epidemiology

These tumours occur across a wide age range in adult women, most commonly over the age of 50 years.

Clinical features

Presentation is with vaginal bleeding, uterine enlargement, pelvic mass or pain.

Histopathology

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.

Immunohistochemistry

 

Cytokeratin cocktail of AE1/AE3, Cam5.2, MNF116, keratin8&18

2/16

 

Synaptophysin

15/16

Neurofilament

10/10

CD99

6/8

Chromogranin

3/6

CD56

3/3

NSE

2/2

Neu-N

2/2

EMA

0/1

S100

2/4

GFAP

0/4

   

Ultrastructure

Molecular studies

None of 11 cases examined showed EWSR1 gene rearrangement.

Differential diagnosis

Prognosis

These are aggressive tumours.

References

1 Euscher ED, Deavers MT, Lopez-Terrada D, et al. Uterine tumors with neuroectodermal differentiation: a series of 17 cases and review of the literature. Am J Surg Pathol 2008; 32:219-28

This page last revised 22.3.2008

©SMUHT/PW Bishop