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WHO Classification of Tumours
Leiomyosarcoma, NOS
Kidney


Definition

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma , myxoid leiomyosarcoma , granular cell and inflammatory leiomyosarcoma
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Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Primary renal leiomyosarcoma is rare, but it is the most common renal sarcoma (50-60% of cases). It occurs chiefly in adults; men and women are equally affected. Tumours are typically large, solid, and focally necrotic. Renal leiomyosarcoma metastasizes to lung, liver, and bone. The prognosis is poor; most patients die within 1 year of diagnosis. Complete surgical excision is the only curative therapy. Small tumour size (<5cm), low histological grade, and limitation to the kidney are associated with a more favourable outcome

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Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



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