title: [Uterine leiomyosarcoma: epidemiology, pathology, biology, diagnosis, prognosis and treatment].
abstract: Uterine leiomyosarcoma is a rare disease with a poor prognosis. The rarity of this tumor needs a specialized management in tertiary reference centers in order to provide patients with optimal diagnostic, prognostic and therapeutic care. The pathological diagnosis relies on the presence of three characteristics in proliferating smooth muscle cells: necrosis, cytologic atypia and mitosis. Despite progress in the knowledge of the biology of these tumors, no oncogenic driver has been found. Prognosis depends mainly on the age of the patient, race, FIGO stage, mitotic index and hormonal receptor expression in the tumor. Surgery is one of the cornerstones of management and cytotoxic chemotherapy is the mainstay of treatment in metastatic disease with a potential role in the adjuvant setting. In locally advanced or metastatic disease, prognosis is poor with a median overall survival of about 12 to 14 months despite a 30% response rate to polychemotherapy regimens. Anti-angiogenics and hormonal therapy have a role to play in the setting of metastatic disease. It is mandatory to include such patients in clinical trials aiming to improve the therapeutic management of these patients. Multimodal therapy can improve the prognosis of selected patients too.
authors.affiliation: Institut Gustave-Roussy, département de médecine, service d'oncologie gynécologique, 114, rue Édouard-Vaillant, 94805 Villejuif, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Hadoux
authors.forename: Philippe
authors.initials: P
authors.lastname: Morice
authors.forename: Catherine
authors.initials: C
authors.lastname: Lhommé
authors.forename: Pierre
authors.initials: P
authors.lastname: Duvillard
authors.forename: Corinne
authors.initials: C
authors.lastname: Balleyguier
authors.forename: Christine
authors.initials: C
authors.lastname: Haie-Meder
authors.forename: Sébastien
authors.initials: S
authors.lastname: Gouy
authors.forename: Catherine
authors.initials: C
authors.lastname: Uzan
authors.forename: Renaud
authors.initials: R
authors.lastname: Mazeron
authors.forename: Youssef
authors.initials: Y
authors.lastname: Tazi
authors.forename: Alexandra
authors.initials: A
authors.lastname: Leary
authors.forename: Florence
authors.initials: F
authors.lastname: Duffaud
authors.forename: Patricia
authors.initials: P
authors.lastname: Pautier
mesh_terms: D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D005260:Female; D006801:Humans; D007890:Leiomyosarcoma / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D035583:Rare Diseases / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000628:therapy; D014594:Uterine Neoplasms / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000628:therapy
chemical_list: D020533:Angiogenesis Inhibitors; D018931:Antineoplastic Agents, Hormonal
keywords: chemotherapy; multidisciplinarity; rare disease; uterine leiomyosarcoma