A 33-year-old man underwent resection of a benign intramural schwannoma of the oesophagus. Similar clinicopathological and immunohistological features prompted a review of an earlier case initially diagnosed as a S-100 negative leiomyosarcoma. This stained positive using a new S-100 immunohistochemical antibody (Dako, Trappes, France). The frequency of these extremely rare tumours may be higher than initially thought. Pitfalls for the surgeon are highlighted and attention is brought to a recent consensus.
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