Endometrial endometrioid adenocarcinoma associated with primitive neuroectodermal tumour of the uterus: a poor prognostic subtype of uterine tumours

• Type: Article in International Scientific Journal
• Date: 2011

Authors

Carla Bartosch (Author) Other; Joana Vieira (Author) Other; Manuel R Teixeira (Author) ICBAS; Jose Manuel Lopes (Author) FMUP

Journal

Title: Medical Oncology

Vol. 28

Pages: 1488-1494

ISSN: 1357-0560

Publisher: Springer Nature

Indexing

ISI Web of Knowledge - 6 Citations

Scopus - 7 Citations

Scientific classification

FOS: Medical and Health sciences > Clinical medicine

Other information

Authenticus ID: P-002-JAR

DOI: 10.1007/s12032-010-9579-z

Abstract (EN): Uterine primitive neuroectodermal tumours are extremely rare tumours. They can occur in pure form or combined with another component including endometrioid adenocarcinoma. We aimed to review the clinical impact of neuroectodermal phenotype in uterine tumours, after we recently diagnosed one such case. A 58-year-old female presented with irregular vaginal bleeding. Ultrasonography and CT showed the presence of a large uterine mass with irregular contours. At laparotomy it was found to extend to the right ureter, sigmoid colon and some small intestinal loops. Microscopic examination revealed that the tumour consisted of an endometrioid adenocarcinoma component merging with an extensive neuroectodermal component. No EWSR1 or FUS rearrangement was found in the two tumour components. The patient received two courses of chemotherapy but died 11 months after the initial diagnosis. We reviewed the morphological and molecular criteria for the diagnosis of uterine primitive neuroectodermal tumours published in the literature. We conclude that regardless of the detection of an EWSR1 rearrangement, the presence of a neuroectodermal differentiation component in these rare uterine tumours is a marker of aggressive behaviour, and its presence should be highlighted in the diagnosis.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 7