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

• Main Authors: 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 Sun, Nae-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
• Format: Article
• Language: English
• Published: Elsevier 2016-08-01
• Series: Taiwanese Journal of Obstetrics & Gynecology
• Subjects: diagnosis; endometrial stromal sarcoma; treatment; undifferentiated sarcoma; uterine sarcoma; uterus
• Online Access: http://www.sciencedirect.com/science/article/pii/S1028455916300596

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.