The biology of uterine sarcomas: A review and update

Kobayashi,Hiroshi; Uekuri,Chiaki; Akasaka,Juria; Ito,Fuminori; Shigemitsu,Aiko; Koike,Natsuki; Shigetomi,Hiroshi

doi:10.3892/mco.2013.124

The biology of uterine sarcomas: A review and update

Authors:
- Hiroshi Kobayashi
- Chiaki Uekuri
- Juria Akasaka
- Fuminori Ito
- Aiko Shigemitsu
- Natsuki Koike
- Hiroshi Shigetomi
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Affiliations: Department of Obstetrics and Gynecology, Nara Medical University, Nara 634-8522, Japan
Published online on: May 20, 2013 https://doi.org/10.3892/mco.2013.124
Pages: 599-609

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Abstract

Uterine sarcoma is a rare neoplasm, accounting for only 5% of uterine malignancies. The pathogenesis of uterine sarcoma remains largely unknown, although recent basic science and pre-clinical animal models have provided a better understanding of tumor biology. The aim of this study was to review the clinical features, imaging characteristics, genetic aberrations and therapeutic approaches in uterine sarcoma. This study reviewed the English-language literature on clinical and basic studies on uterine sarcoma. The common variants of uterine sarcoma are carcinosarcoma, leiomyosarcoma and endometrial stromal sarcoma (ESS). Genetic profiling efforts have identified amplification, overexpression and mutation, while the molecular mechanisms of tumorigenesis driven by these genomic and genetic aberrations have yet to be fully elucidated yet. Recent genome-wide studies have also identified complex chromosomal rearrangements as oncogenic mechanisms. The cell cycle regulators, p16 and p53, are frequently overexpressed and appear to be involved in key modifications of sarcomagenesis. Molecular‑targeted therapy has now been evaluated in clinical trials for certain subtypes. In conclusion, aberrations of cell cycle control would be a critical step in the development of uterine sarcoma. This review has provided new areas of study targeting molecular and genetic pathways.

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