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Primary clear cell sarcoma of the ileum: an uncommon and misleading site

Taminelli, Lorenzo ; Zaman, Khalil ; Gengler, Carole ; Peloponissios, Nicolas ; Bouzourene, Hanifa ; Coindre, Jean-Michel ; Hostein, Isabelle ; Guillou, Louis

In: Virchows Archiv, 2005, vol. 447, no. 4, p. 772-777

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    Titre
    • Primary clear cell sarcoma of the ileum: an uncommon and misleading site
    Auteur
    • Taminelli, Lorenzo. Department of Pathology, University Hospital, Lausanne, Switzerland
    • Zaman, Khalil. Department of Oncology, University Hospital, Lausanne, Switzerland
    • Gengler, Carole. Department of Pathology, University Hospital, Lausanne, Switzerland
    • Peloponissios, Nicolas. Department of Surgery, University Hospital, Lausanne, Switzerland
    • Bouzourene, Hanifa. Department of Pathology, University Hospital, Lausanne, Switzerland
    • Coindre, Jean-Michel. Department of Pathology, Bergonié Institute, Bordeaux, France
    • Hostein, Isabelle. Department of Pathology, Bergonié Institute, Bordeaux, France
    • Guillou, Louis. Department of Pathology, University Hospital, Lausanne, Switzerland
    Type de document
    • Postprint
    Identifiant OAI-PMH
    • oai:doc.rero.ch:318771
    Summary
    A clear cell sarcoma, arising primarily in the ileum of a 35-year-old man, is reported. Histologically, the neoplasm infiltrated the full thickness of the intestinal wall. It consisted of strands and sheets of round to spindle-shaped cells with clear to eosinophilic cytoplasm, vesicular nuclei and prominent nucleoli. Vascular invasion was present at diagnosis. Tumour cells expressed S-100 protein, melan-A and tyrosinase. They were negative for HMB45, CD117, cytokeratins, epithelial membrane antigen, smooth muscle actin, desmin, CD31, CD34, chromogranin and synaptophysin. Reverse transcription-polymerase chain reaction analysis performed on paraffin-embedded tissue showed EWS-ATF1 fusion transcripts representative of the t(12;22) (q13;q12) clear cell sarcoma reciprocal translocation. The patient, who developed liver metastases 2 months after diagnosis, died of disease at 15 months. This case demonstrates that the gastrointestinal tract is a potential site for primary clear cell sarcoma of soft tissues, and, furthermore, that cytogenetics and/or molecular techniques play a central role in the diagnosis