NCCN Guidelines® Insights: Uterine Neoplasms, Version 3.2021

Featured Updates to the NCCN Guidelines

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  • 1 Memorial Sloan Kettering Cancer Center;
  • | 2 UC San Diego Moores Cancer Center;
  • | 3 University of Wisconsin Carbone Cancer Center;
  • | 4 Dana-Farber/Brigham and Women’s Cancer Center;
  • | 5 Duke Cancer Institute;
  • | 6 Moffitt Cancer Center;
  • | 7 Fox Chase Cancer Center;
  • | 8 The Ohio State University Comprehensive Cancer Center-James Cancer Hospital and Solove Research Institute;
  • | 9 Vanderbilt-Ingram Cancer Center;
  • | 10 Yale Cancer Center/Smilow Cancer Hospital;
  • | 11 Stanford Cancer Institute;
  • | 12 University of Colorado Cancer Center;
  • | 13 Roswell Park Comprehensive Cancer Center;
  • | 14 Huntsman Cancer Institute at the University of Utah;
  • | 15 Abramson Cancer Center at the University of Pennsylvania;
  • | 16 City of Hope National Medical Center;
  • | 17 O'Neal Comprehensive Cancer Center at UAB;
  • | 18 UT Southwestern Simmons Comprehensive Cancer Center;
  • | 19 Mayo Clinic Cancer Center;
  • | 20 Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine;
  • | 21 Fred & Pamela Buffett Cancer Center;
  • | 22 University of Michigan Rogel Cancer Center;
  • | 23 UCLA Jonsson Comprehensive Cancer Center;
  • | 24 Massachusetts General Hospital Cancer Center;
  • | 25 The University of Texas MD Anderson Cancer Center;
  • | 26 Robert H. Lurie Comprehensive Cancer Center of Northwestern University;
  • | 27 UCSF Helen Diller Family Comprehensive Cancer Center;
  • | 28 Fred Hutchinson Cancer Research CenterSeattle Cancer Care Alliance;
  • | 29 The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins;
  • | 30 Patient Advocate;
  • | 31 Case Comprehensive Cancer CenterUniversity Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute; and
  • | 32 National Comprehensive Cancer Network.
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The NCCN Guidelines for Uterine Neoplasms provide recommendations for diagnostic workup, clinical staging, and treatment options for patients with endometrial cancer or uterine sarcoma. These NCCN Guidelines Insights focus on the recent addition of molecular profiling information to aid in accurate diagnosis, classification, and treatment of uterine sarcomas.

  • 1.

    Siegel RL, Miller KD, Fuchs HE, et al. Cancer statistics, 2021. CA Cancer J Clin 2021;71:733.

  • 2.

    D’Angelo E, Prat J. Uterine sarcomas: a review. Gynecol Oncol 2010;116:131139.

  • 3.

    WHO Classification of Tumours Editorial Board. WHO Classification of Tumours, 5th ed. Female Genital Tumours. Vol. 4. Lyon, France. International Agency for Research on Cancer; 2020.

    • Search Google Scholar
    • Export Citation
  • 4.

    Tropé CG, Abeler VM, Kristensen GB. Diagnosis and treatment of sarcoma of the uterus. A review. Acta Oncol 2012;51:694705.

  • 5.

    Amant F, Coosemans A, Debiec-Rychter M, et al. Clinical management of uterine sarcomas. Lancet Oncol 2009;10:11881198.

  • 6.

    Parra-Herran C, Howitt BE. Uterine mesenchymal tumors: update on classification, staging, and molecular features. Surg Pathol Clin 2019;12:363396.

  • 7.

    Novetsky AP, Powell MA. Management of sarcomas of the uterus. Curr Opin Oncol 2013;25:546552.

  • 8.

    Lax SF. Molecular genetic changes in epithelial, stromal and mixed neoplasms of the endometrium. Pathology 2007;39:4654.

  • 9.

    Huang HY, Ladanyi M, Soslow RA. Molecular detection of JAZF1-JJAZ1 gene fusion in endometrial stromal neoplasms with classic and variant histology: evidence for genetic heterogeneity. Am J Surg Pathol 2004;28:224232.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10.

    Leath CA III, Huh WK, Hyde J Jr, et al. A multi-institutional review of outcomes of endometrial stromal sarcoma. Gynecol Oncol 2007;105:630634.

  • 11.

    Koontz JI, Soreng AL, Nucci M, et al. Frequent fusion of the JAZF1 and JJAZ1 genes in endometrial stromal tumors. Proc Natl Acad Sci USA 2001;98:63486353.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 12.

    Zou Y, Turashvili G, Soslow RA, et al. High-grade transformation of low-grade endometrial stromal sarcomas lacking YWHAE and BCOR genetic abnormalities. Mod Pathol 2020;33:18611870.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13.

    Lee CH, Mariño-Enriquez A, Ou W, et al. The clinicopathologic features of YWHAE-FAM22 endometrial stromal sarcomas: a histologically high-grade and clinically aggressive tumor. Am J Surg Pathol 2012;36:641653.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14.

    Hoang LN, Aneja A, Conlon N, et al. Novel high-grade endometrial stromal sarcoma: a morphologic mimicker of myxoid leiomyosarcoma. Am J Surg Pathol 2017;41:1224.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15.

    Lewis N, Soslow RA, Delair DF, et al. ZC3H7B-BCOR high-grade endometrial stromal sarcomas: a report of 17 cases of a newly defined entity. Mod Pathol 2018;31:674684.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16.

    Mariño-Enriquez A, Lauria A, Przybyl J, et al. BCOR internal tandem duplication in high-grade uterine sarcomas. Am J Surg Pathol 2018;42:335341.

  • 17.

    Kurman RJ, Carcangiu ML, Herrington CS, Young RH, eds. WHO Classification of Tumours of Female Reproductive Organs, 4th ed. Vol. 6. Lyon, France: International Agency for Research on Cancer; 2014.

    • Search Google Scholar
    • Export Citation
  • 18.

    Binzer-Panchal A, Hardell E, Viklund B, et al. Integrated molecular analysis of undifferentiated uterine sarcomas reveals clinically relevant molecular subtypes. Clin Cancer Res 2019;25:21552165.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19.

    Cotzia P, Benayed R, Mullaney K, et al. Undifferentiated uterine sarcomas represent under-recognized high-grade endometrial stromal sarcomas. Am J Surg Pathol 2019;43:662669.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20.

    Kolin DL, Dong F, Baltay M, et al. SMARCA4-deficient undifferentiated uterine sarcoma (malignant rhabdoid tumor of the uterus): a clinicopathologic entity distinct from undifferentiated carcinoma. Mod Pathol 2018;31:14421456.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 21.

    Kolin DL, Quick CM, Dong F, et al. SMARCA4-deficient uterine sarcoma and undifferentiated endometrial carcinoma are distinct clinicopathologic entities. Am J Surg Pathol 2020;44:263270.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 22.

    Köbel M, Hoang LN, Tessier-Cloutier B, et al. Undifferentiated endometrial carcinomas show frequent loss of core switch/sucrose nonfermentable complex proteins. Am J Surg Pathol 2018;42:7683.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 23.

    Tessier-Cloutier B, Coatham M, Carey M, et al. SWI/SNF-deficiency defines highly aggressive undifferentiated endometrial carcinoma. J Pathol Clin Res 2021;7:144153.

  • 24.

    Arias-Stella JA III, Benayed R, Oliva E, et al. Novel PLAG1 gene rearrangement distinguishes a subset of uterine myxoid leiomyosarcoma from other uterine myxoid mesenchymal tumors. Am J Surg Pathol 2019;43:382388.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25.

    Yoon JY, Mariño-Enriquez A, Stickle N, et al. Myxoid smooth muscle neoplasia of the uterus: comprehensive analysis by next-generation sequencing and nucleic acid hybridization. Mod Pathol 2019;32:16881697.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 26.

    Chiang S, Samore W, Zhang L, et al. PGR gene fusions identify a molecular subset of uterine epithelioid leiomyosarcoma with rhabdoid features. Am J Surg Pathol 2019;43:810818.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 27.

    Selenica P, Conlon N, Gonzalez C, et al. Genomic profiling aids classification of diagnostically challenging uterine mesenchymal tumors with myomelanocytic differentiation. Am J Surg Pathol 2021;45:7792.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 28.

    Goebel EA, Hernandez Bonilla S, Dong F, et al. Uterine tumor resembling ovarian sex cord tumor (UTROSCT): a morphologic and molecular study of 26 cases confirms recurrent NCOA1-3 rearrangement. Am J Surg Pathol 2020;44:3042.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 29.

    Dickson BC, Childs TJ, Colgan TJ, et al. Uterine tumor resembling ovarian sex cord tumor: a distinct entity characterized by recurrent NCOA2/3 gene fusions. Am J Surg Pathol 2019;43:178186.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 30.

    Lee CH, Kao YC, Lee WR, et al. Clinicopathologic characterization of GREB1-rearranged uterine sarcomas with variable sex-cord differentiation. Am J Surg Pathol 2019;43:928942.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 31.

    Moore M, McCluggage WG. Uterine tumour resembling ovarian sex cord tumour: first report of a large series with follow-up. Histopathology 2017;71:751759.

  • 32.

    Leiner J, Le Loarer F. The current landscape of rhabdomyosarcomas: an update. Virchows Arch 2020;476:97108.

  • 33.

    Pinto A, Kahn RM, Rosenberg AE, et al. Uterine rhabdomyosarcoma in adults. Hum Pathol 2018;74:122128.

  • 34.

    Li RF, Gupta M, McCluggage WG, et al. Embryonal rhabdomyosarcoma (botryoid type) of the uterine corpus and cervix in adult women: report of a case series and review of the literature. Am J Surg Pathol 2013;37:344355.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 35.

    de Kock L, Yoon JY, Apellaniz-Ruiz M, et al. Significantly greater prevalence of DICER1 alterations in uterine embryonal rhabdomyosarcoma compared to adenosarcoma. Mod Pathol 2020;33:12071219.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 36.

    Bennett JA, Ordulu Z, Young RH, et al. Embryonal rhabdomyosarcoma of the uterine corpus: a clinicopathological and molecular analysis of 21 cases highlighting a frequent association with DICER1 mutations [published online May 20, 2021]. Mod Pathol, doi: 10.1038/s41379-021-00821-x

    • PubMed
    • Search Google Scholar
    • Export Citation
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