TY - JOUR
T1 - Léiomyosarcomes utérins
T2 - épidémiologie, histologie, biologie, diagnostic, pronostic et traitement
AU - Hadoux, Julien
AU - Morice, Philippe
AU - Lhommé, Catherine
AU - Duvillard, Pierre
AU - Balleyguier, Corinne
AU - Haie-Meder, Christine
AU - Gouy, Sébastien
AU - Uzan, Catherine
AU - Mazeron, Renaud
AU - Tazi, Youssef
AU - Leary, Alexandra
AU - Duffaud, Florence
AU - Pautier, Patricia
PY - 2013/1/1
Y1 - 2013/1/1
N2 - 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. Antiangiogenics 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.
AB - 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. Antiangiogenics 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.
KW - Chemotherapy
KW - Multidisciplinarity
KW - Rare disease
KW - Uterine leiomyosarcoma
UR - http://www.scopus.com/inward/record.url?scp=84884767570&partnerID=8YFLogxK
U2 - 10.1684/bdc.2013.1801
DO - 10.1684/bdc.2013.1801
M3 - Article
AN - SCOPUS:84884767570
SN - 0007-4551
VL - 100
SP - 903
EP - 915
JO - Bulletin du Cancer
JF - Bulletin du Cancer
IS - 9
ER -