Leiomyosarcoma of the small intestine is a malignant mesenchymal tumour composed of smooth muscle cells from the muscular coat or, rarely, from muscularis mucosae, which in the majority of cases is characterised by a clinically silent development and by an unfavorable prognosis. The considerable difficulty of obtaining a histological definition of the degree of malignancy of the leiomyosarcoma is also typical. The paper reports a clinical case and summarises its main clinical and anatomopathological aspects. In particular, the difficulties of clinical and histological diagnosis of the degree of malignancy are underlined, together with the fact that these have a considerable influence on the possibilities and results of surgical therapy and survival rates.

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