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

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Abstract

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.

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Authors and Affiliations

  1. Department of Pathology, Hospital de S. João, E.P.E., Alameda Professor Hernâni Monteiro, 4202-451, Porto, Portugal

    Carla Bartosch & José Manuel Lopes

  2. Department of Genetics, Portuguese Oncology Institute, Porto, Portugal

    Joana Vieira & Manuel R. Teixeira

  3. Institute of Biomedical Sciences (ICBAS), University of Porto, Porto, Portugal

    Manuel R. Teixeira

  4. Department of Pathology, Medical Faculty, University of Porto, Porto, Portugal

    José Manuel Lopes

  5. IPATIMUP (Institute of Molecular Pathology and Immunology University of Porto), Porto, Portugal

    José Manuel Lopes

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  1. Carla Bartosch
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  2. Joana Vieira
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  3. Manuel R. Teixeira
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  4. José Manuel Lopes
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Correspondence to José Manuel Lopes.

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Bartosch, C., Vieira, J., Teixeira, M.R. et al. Endometrial endometrioid adenocarcinoma associated with primitive neuroectodermal tumour of the uterus: a poor prognostic subtype of uterine tumours. Med Oncol 28, 1488–1494 (2011). https://doi.org/10.1007/s12032-010-9579-z

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  • DOI: https://doi.org/10.1007/s12032-010-9579-z

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