Gynecologic cancer intergroup (GCIG) consensus review for high-grade undifferentiated sarcomas of the uterus

Patricia Pautier, Eun Ji Nam, Diane M. Provencher, Anne L. Hamilton, Giorgia Mangili, Nadeem Ahmad Siddiqui, Anneke M. Westermann, Nicholas Simon Reed, Philipp Harter, Isabelle Ray-Coquard

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    40 Citations (Scopus)

    Abstract

    High-grade undifferentiated sarcomas (HGUSs) are rare uterine malignancies arising from the endometrial stroma. They are poorly differentiated sarcomas composed of cells that do not resemble proliferative-phase endometrial stroma. High-grade undifferentiated sarcomas are characterized by aggressive behavior and poor prognosis. Cyclin D1 has been reported as a diagnostic immunomarker for high-grade endometrial stromal sarcoma with an YWHAE-FAM22 rearrangement. YWHAE-FAM22 endometrial stromal sarcomas (ESS) represent a clinically aggressive subtype ofESS classified as high-grade endometrial sarcomas, and its distinction from the usual low-grade ESS with JAZF1 rearrangement and from HGUS with no identifiable molecular aberration may be important in guiding clinical management. Median age of the patients is between 55 and 60 years. The most common symptoms are vaginal bleeding, abdominal pain, and increasing abdominal girth. Disease is usually advanced with approximately 70%of the patients staged III to IVaccording to the International Federation of Gynecology and Obstetrics classification. Preferential metastatic locations include peritoneum, lungs, intra-Abdominal lymph nodes, and bone. Median progression-free survival ranged from 7 to 10months, andmedian overall survival ranged from 11 to 23 months. There is no clear prognostic factor identified for HGUS, not even stage. The standard management for HGUS consists of total hysterectomy and bilateral salpingooophorectomy. Systematic lymphadenectomy is not recommended. Adjuvant therapies, such as chemotherapy and radiotherapy, have to be discussed in multidisciplinary staff meetings. 2014 by IGCS and ESGO.

    Original languageEnglish
    Pages (from-to)S73-S77
    JournalInternational Journal of Gynecological Cancer
    Volume24
    Issue number9
    DOIs
    Publication statusPublished - 1 Nov 2014

    Keywords

    • High-grade undifferentiated sarcoma
    • Uterine sarcoma

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