Pathology & Oncology Research

, Volume 16, Issue 4, pp 601–608 | Cite as

Uterine Tumors with Neuroectodermal Differentiation. A Report of 4 Cases

  • Pavel Dundr
  • Daniela Fischerová
  • Ctibor Povýšil
  • Adéla Berková
  • Lenka Bauerová
  • David Cibula
Article

Abstract

We report four cases of uterine tumors with neuroectodermal differentiation. One tumor had neuroectodermal component only; in the three other tumors, the neuroectodermal component was admixed with another component, namely rhabdomyosarcoma (1 case), and endometrioid adenocarcinoma (2 cases). Histologically, the neuroectodermal component consisted of small to medium sized cells arranged in diffuse sheets. The tumor cells had round nuclei with stippled to coarsely granular chromatin, mostly with non-prominent nucleoli, and scant eosinophilic or amphophilic cytoplasm. Immunohistochemically, 4/4 tumors showed expression of vimentin, synaptophysin and CD56; 3/4 tumors were CD99 and NSE positive; 2/4 tumors showed focal expression of S-100 protein; and 1/4 tumors had focal dot-like cytoplasmic positivity for cytokeratin AE1/AE3. FLI-1 was negative in all cases. FISH examination was performed and none of the tumors showed rearrangement of EWSR1 gene. Uterine tumors with neuroectodermal differentiation are rare; to the best of our knowledge only 44 cases have been reported in the literature to date, referred to as Ewing sarcoma, peripheral PNET (pPNET), PNET (not otherwise specified) and uterine tumors with neuroendocrine differentiation.

Keywords

Uterine tumors PNET Ewing sarcoma EWSR1 gene Neuroectodermal 

Abbreviations

EWSR1

Ewing sarcoma breakpoint region 1

NOS

not otherwise specified

PNET

primitive neuroectodermal tumor

Notes

Acknowledgement

This work was presented on The 17th Congress of the Czech and Slovak Pathologists, September 23–25, 2009, Mikulov, Czech Republic.

This work was supported by Ministry of Education Research Project MSM0021620808.

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Copyright information

© Arányi Lajos Foundation 2010

Authors and Affiliations

  • Pavel Dundr
    • 1
  • Daniela Fischerová
    • 2
  • Ctibor Povýšil
    • 1
  • Adéla Berková
    • 3
  • Lenka Bauerová
    • 1
  • David Cibula
    • 2
  1. 1.Department of Pathology, First Faculty of MedicineCharles University in Prague and General University Hospital in PraguePragueCzech Republic
  2. 2.Oncogynecological Centre, Department of Obstetrics and Gynecology, First Faculty of MedicineCharles University in Prague and General University Hospital in PraguePrague 2Czech Republic
  3. 3.1st Department of Medicine—Department of Haematology, First Faculty of MedicineCharles University in Prague and General University Hospital in PraguePrague 2Czech Republic

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