Uterine sarcoma Part II—Uterine endometrial stromal sarcoma: The TAG systematic review

Huann Cheng Horng, Kuo Chang Wen, Peng Hui Wang*, Yi Jen Chen, Ming Shyen Yen, Heung Tat Ng, Yen Hou Chang, Yi Chang, Hsiang Tai Chao, Kuan Chong Chao, Chi Mu Chuang, Chi Hong Ho, Chen Yu Huang, Zhi Chen Hung, Ling Yu Jiang, Hei Yu Lau, Hsin Yang Li, Chi Yao Lin, Chia Hao Liu, Pi Lin SunNae Fang Twu, Hua Hsi Wu, Hann Chin Yu, Fong Yuan Ju, Chih Ping Tsai, Wen Hsun Chang, Yen Mei Hsu, Na Rong Lee, Chih Yao Chen, Ting Chen Chang, Wen Chun Chang, Chii Hou Chen, Ruey Jian Chen, Song Nan Chow, Yih Ron Lien, Bor Ching Sheu, Pao Ling Torng, Men Luh Yen, Wen Ling Lee, Kuan Chin Wang, Chih Long Chang, Chih Ping Chen, Jen Ruei Chen, Tze Chien Chen, Jian Pei Huang, Ming Chao Huang, Yeou Lih Wang, Cheng Chang Chang, Jah Yao Liu, Her Young Su, Yu Chi Wang, Mu Hsien Yu, Lee Wen Huang, Kok Min Seow, Tsung Hsuan Lai, Fa Kung Lee, Ching Hui Chen, Ben Shian Huang, Sheng Mou Hsiao, Hsu Dong Sun, Wen Yih Wu, Sen Wen Teng, Kuo Hu Chen, Jeng Hsiu Hung, Hung Cheng Lai, Chiou Chung Yuan, Ching Hung Hsieh, Chin Jung Wang, Shing Jyh Chang, Man Jung Hung, Shih Tien Hsu, Yu Min Ke, Chien Hsing Lu, Lou Sun, Wei Chun Chang, Yao Ching Hung, Wu Chou Lin, Po Hui Wang, Tze Ho Chen, Yiu Tai Li, Meng Hsing Wu, Kuo Feng Huang, Hung Chun Fu, San Nung Chen, An Jen Chiang, Ju Yueh Li, Li Te Lin, Hsiao Wen Tsai, Kuan Hao Tsui

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

41 Scopus citations

Abstract

Endometrial stromal tumors are rare uterine tumors (<1%). Four main categories include endometrial stromal nodule, low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and uterine undifferentiated sarcoma (UUS). This review is a series of articles discussing the uterine sarcomas. LG-ESS, a hormone-dependent tumor harboring chromosomal rearrangement, is an indolent tumor with a favorable prognosis, but characterized by late recurrences even in patients with Stage I disease, suggesting the requirement of a long-term follow-up. Patients with HG-ESS, based on the identification of YWHAE-NUTM2A/B (YWHAE-FAM22A/B) gene fusion, typically present with advanced stage diseases and frequently have recurrences, usually within a few years after initial surgery. UUS is, a high-grade sarcoma, extremely rare, lacking a specific line of differentiation, which is a diagnosis of exclusion (the wastebasket category, which fails to fulfill the morphological and immunohistochemical criteria of translocation-positive ESS). Surgery is the main strategy in the management of uterine sarcoma. Due to rarity, complex biological characteristics, and unknown etiology and risk factors of uterine sarcomas, the role of adjuvant therapy is not clear. Only LG-ESS might respond to progestins or aromatase inhibitors.

Original languageEnglish
Pages (from-to)472-479
Number of pages8
JournalTaiwanese Journal of Obstetrics and Gynecology
Volume55
Issue number4
DOIs
StatePublished - 01 08 2016

Bibliographical note

Publisher Copyright:
© 2016

Keywords

  • diagnosis
  • endometrial stromal sarcoma
  • treatment
  • undifferentiated sarcoma
  • uterine sarcoma
  • uterus

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