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Primary epithelioid sarcoma of the oesophagus

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Abstract

Epithelioid sarcoma is a rare soft tissue tumour presenting two main variants: the ‘classical’ distal type and the more recently described proximal type. The latter is distinguished from the former by occurrence in elderly patients, more axial and deep location, prominent atypical and pleomorphic appearance and eventually aggressive clinical behaviour with poor outcome. To date, only few perivisceral epithelioid sarcomas have been described, and they are mainly related to the colon and bladder in pelvic and perineal sites. We report a hitherto undescribed epithelioid sarcoma of the distal oesophagus and discuss the relevance of molecular cytogenetics.

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References

  1. Debiec-Rychter M, Sciot R, Hagemeijer A (2000) Common chromosomal aberrations in the proximal type of epithelioid sarcoma. Cancer Genet Cytogenet 123:133–136

    Article  PubMed  CAS  Google Scholar 

  2. Enzinger FM (1970) Epithelioid sarcoma. Cancer 26:1029–1041

    Article  PubMed  CAS  Google Scholar 

  3. Guillou L, Wadden C, Coindre J, Krausz T, Fletcher CDM (1997) “Proximal type” epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Am J Surg Pathol 21:130–146

    Article  PubMed  CAS  Google Scholar 

  4. Hasegawa T, Matsuno Y, Shimoda T, Umeda T, Yokoyama R, Hirohashi S (2001) Proximal-type epithelioid sarcoma: a clinicopathologic study of 20 cases. Mod Path 14:655–663

    Article  CAS  Google Scholar 

  5. Hoot A, Russo P, Judkins A, Perlman E, Biegel J (2004) Immunoistochemical analysis of hSNF5/INI1 distinguishes renal and extra-renal malignant rhabdoid tumors from others pediatric soft tissue tumors. Am J Surg Pathol 28:1485–1491

    Article  PubMed  Google Scholar 

  6. Isaacson PG (1999) Mucosa-associated lymphoid tissue lymphoma. Semin Hematol 36:39–147

    Google Scholar 

  7. Lualdi E, Modena P, Debiec-Rychter M, Pedentour F, Teixeira M, Facchinetti F, Dagrada G, Pilotti S, Sozzi G (2004) Molecular cytogenetic characterization of proximal-type epithelioid sarcoma. Genes Chromosomes Cancer 41:283–290

    Article  PubMed  CAS  Google Scholar 

  8. Modena P, Lualdi E, Facchinetti F, Galli L, Teixeira M, Pilotti S, Sozzi (2005) SMARCB1/INI1 tumor suppressor gene is frequently inactivated in epithelioid sarcomas. Cancer Res 65:4012–4019

    Article  PubMed  CAS  Google Scholar 

  9. Oda Y, Tsuneyoshi M (2006) Extrarenal rhabdoid tumor of soft tissue: clinicopathological and molecular genetic review and distribution from other soft-tissue sarcoma with rhabdoid features. Pathfind Int 56:287–295

    Article  Google Scholar 

  10. Spillane A, Thomas M, Fisher C (2000) Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma. Ann Surg Oncol 7:218–225

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Medicina Sperimentale, Università “La Sapienza” Roma, Rome, Italy

    Francesca Maggiani

  2. Department of Morphology and Molecular Pathology, University Hospital Sint Rafael, Catholic University of Leuven, Minderbroederstraat 12, Leuven, Belgium

    Francesca Maggiani, Nadine Ectors & Raf Sciot

  3. Department of Human Genetics, University Hospital Gasthuisberg, Catholic University of Leuven, Leuven, Belgium

    Maria Debiec-Rychter

  4. Department of Thoracic Surgery, University Hospital Gasthuisberg, Catholic University of Leuven, Leuven, Belgium

    Antoon Lerut

Authors
  1. Francesca Maggiani
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  2. Maria Debiec-Rychter
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  3. Nadine Ectors
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  4. Antoon Lerut
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  5. Raf Sciot
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Corresponding author

Correspondence to Raf Sciot.

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Maggiani, F., Debiec-Rychter, M., Ectors, N. et al. Primary epithelioid sarcoma of the oesophagus. Virchows Arch 451, 835–838 (2007). https://doi.org/10.1007/s00428-007-0440-5

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  • DOI: https://doi.org/10.1007/s00428-007-0440-5

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