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Vaginal Undifferentiated Sarcoma

MedGen UID:
311449
•Concept ID:
C1519935
•
Neoplastic Process

Definition

A high grade infiltrating sarcoma that arises from the vagina. It is characterized by the presence of malignant small spindle cells with scant cytoplasm. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVaginal Undifferentiated Sarcoma

Professional guidelines

PubMed

Sarcomas of uterine cervix: clinicopathological features, treatment, and outcome.
Khosla D, Gupta R, Srinivasan R, Patel FD, Rajwanshi A
Int J Gynecol Cancer 2012 Jul;22(6):1026-30. doi: 10.1097/IGC.0b013e31825a97f6. PMID: 22740005
Myogenin and desmin immunohistochemistry in the assessment of post-chemotherapy genitourinary embryonal rhabdomyosarcoma: prognostic and management implications.
Godbole P, Outram A, Wilcox DT, Duffy PG, Sebire NJ
J Urol 2006 Oct;176(4 Pt 2):1751-4. doi: 10.1016/j.juro.2006.03.117. PMID: 16945640
Clinical staging and treatment results in rhabdomyosarcoma of the female genital tract among children and adolescents.
Hays DM, Shimada H, Raney RB Jr, Tefft M, Newton W, Crist WM, Lawrence W Jr, Ragab A, Beltangady M, Maurer HM
Cancer 1988 May 1;61(9):1893-903. doi: 10.1002/1097-0142(19880501)61:9<1893::aid-cncr2820610929>3.0.co;2-9. PMID: 3281745

Recent clinical studies

Etiology

High-Grade Sarcomas with Myogenic Differentiation Harboring Hotspot PDGFRB Mutations.
Dermawan JK, Chiang S, Hensley ML, Tap WD, Antonescu CR
Mod Pathol 2023 May;36(5):100104. Epub 2023 Jan 23 doi: 10.1016/j.modpat.2023.100104. PMID: 36788091Free PMC Article
Uterine sarcomas and rare uterine mesenchymal tumors with malignant potential. Diagnostic guidelines of the French Sarcoma Group and the Rare Gynecological Tumors Group.
Croce S, Devouassoux-Shisheboran M, Pautier P, Ray-Coquard I, Treilleux I, Neuville A, Arnould L, Just PA, Belda MALF, Averous G, Leroux A, Mery E, Loussouarn D, Weinbreck N, Le Guellec S, Mishellany F, Morice P, Guyon F, Genestie C
Gynecol Oncol 2022 Nov;167(2):373-389. Epub 2022 Sep 14 doi: 10.1016/j.ygyno.2022.07.031. PMID: 36114030
Pleomorphic Undifferentiated Uterine Sarcoma in a Young Patient Presenting With Elevated Beta-hCG and Rare Variants of Benign Leiomyoma: A Case Report and Review of the Literature.
Krishnan V, Sauthier P, Provencher D, Rahimi K
Int J Gynecol Pathol 2020 Jul;39(4):362-366. doi: 10.1097/PGP.0000000000000606. PMID: 31033798
Imaging in gynecological disease (15): clinical and ultrasound characteristics of uterine sarcoma.
Ludovisi M, Moro F, Pasciuto T, Di Noi S, Giunchi S, Savelli L, Pascual MA, Sladkevicius P, Alcazar JL, Franchi D, Mancari R, Moruzzi MC, Jurkovic D, Chiappa V, Guerriero S, Exacoustos C, Epstein E, Frühauf F, Fischerova D, Fruscio R, Ciccarone F, Zannoni GF, Scambia G, Valentin L, Testa AC
Ultrasound Obstet Gynecol 2019 Nov;54(5):676-687. Epub 2019 Oct 7 doi: 10.1002/uog.20270. PMID: 30908820
Primary vaginal sarcoma: Experience of a regional cancer center in China.
Wang Y, Huang YW, Li YF
J Obstet Gynaecol Res 2015 Sep;41(9):1463-8. Epub 2015 Jun 26 doi: 10.1111/jog.12746. PMID: 26111799

Diagnosis

Uterine sarcomas and rare uterine mesenchymal tumors with malignant potential. Diagnostic guidelines of the French Sarcoma Group and the Rare Gynecological Tumors Group.
Croce S, Devouassoux-Shisheboran M, Pautier P, Ray-Coquard I, Treilleux I, Neuville A, Arnould L, Just PA, Belda MALF, Averous G, Leroux A, Mery E, Loussouarn D, Weinbreck N, Le Guellec S, Mishellany F, Morice P, Guyon F, Genestie C
Gynecol Oncol 2022 Nov;167(2):373-389. Epub 2022 Sep 14 doi: 10.1016/j.ygyno.2022.07.031. PMID: 36114030
Pleomorphic Undifferentiated Uterine Sarcoma in a Young Patient Presenting With Elevated Beta-hCG and Rare Variants of Benign Leiomyoma: A Case Report and Review of the Literature.
Krishnan V, Sauthier P, Provencher D, Rahimi K
Int J Gynecol Pathol 2020 Jul;39(4):362-366. doi: 10.1097/PGP.0000000000000606. PMID: 31033798
Gynecologic Cancer InterGroup (GCIG) consensus review for high-grade undifferentiated sarcomas of the uterus.
Pautier P, Nam EJ, Provencher DM, Hamilton AL, Mangili G, Siddiqui NA, Westermann AM, Reed NS, Harter P, Ray-Coquard I
Int J Gynecol Cancer 2014 Nov;24(9 Suppl 3):S73-7. doi: 10.1097/IGC.0000000000000281. PMID: 25341584
Mullerian adenosarcoma of the female genital tract.
McCluggage WG
Adv Anat Pathol 2010 Mar;17(2):122-9. doi: 10.1097/PAP.0b013e3181cfe732. PMID: 20179434
Extracutaneous malignant melanomas.
Hussein MR
Cancer Invest 2008 Jun;26(5):516-34. doi: 10.1080/07357900701781762. PMID: 18568775

Therapy

Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatric Oncology MMT95 study.
Oberlin O, Rey A, Sanchez de Toledo J, Martelli H, Jenney ME, Scopinaro M, Bergeron C, Merks JH, Bouvet N, Ellershaw C, Kelsey A, Spooner D, Stevens MC
J Clin Oncol 2012 Jul 10;30(20):2457-65. Epub 2012 Jun 4 doi: 10.1200/JCO.2011.40.3287. PMID: 22665534
Primary vulvar and vaginal extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor: diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction.
Vang R, Taubenberger JK, Mannion CM, Bijwaard K, Malpica A, Ordonez NG, Tavassoli FA, Silver SA
Int J Gynecol Pathol 2000 Apr;19(2):103-9. doi: 10.1097/00004347-200004000-00002. PMID: 10782405
Conservative treatment for lower gynecological tract malignancies in children and adolescents: the Institut Gustave-Roussy experience.
Gerbaulet AP, Esche BA, Haie CM, Castaigne D, Flamant F, Chassagne D
Int J Radiat Oncol Biol Phys 1989 Sep;17(3):655-8. doi: 10.1016/0360-3016(89)90119-3. PMID: 2777654
Clinical staging and treatment results in rhabdomyosarcoma of the female genital tract among children and adolescents.
Hays DM, Shimada H, Raney RB Jr, Tefft M, Newton W, Crist WM, Lawrence W Jr, Ragab A, Beltangady M, Maurer HM
Cancer 1988 May 1;61(9):1893-903. doi: 10.1002/1097-0142(19880501)61:9<1893::aid-cncr2820610929>3.0.co;2-9. PMID: 3281745
Infants younger than 1 year of age with rhabdomyosarcoma.
Ragab AH, Heyn R, Tefft M, Hays DN, Newton WA Jr, Beltangady M
Cancer 1986 Dec 15;58(12):2606-10. doi: 10.1002/1097-0142(19861215)58:12<2606::aid-cncr2820581209>3.0.co;2-t. PMID: 3779610

Prognosis

Pleomorphic Undifferentiated Uterine Sarcoma in a Young Patient Presenting With Elevated Beta-hCG and Rare Variants of Benign Leiomyoma: A Case Report and Review of the Literature.
Krishnan V, Sauthier P, Provencher D, Rahimi K
Int J Gynecol Pathol 2020 Jul;39(4):362-366. doi: 10.1097/PGP.0000000000000606. PMID: 31033798
Endometrial cancer with congenital uterine anomalies: 3 case reports and a literature review.
Gao J, Zhang J, Tian W, Teng F, Zhang H, Zhang X, Wang Y, Xue F
Cancer Biol Ther 2017 Mar 4;18(3):123-131. Epub 2017 Jan 24 doi: 10.1080/15384047.2017.1281495. PMID: 28118070Free PMC Article
Gynecologic Cancer InterGroup (GCIG) consensus review for high-grade undifferentiated sarcomas of the uterus.
Pautier P, Nam EJ, Provencher DM, Hamilton AL, Mangili G, Siddiqui NA, Westermann AM, Reed NS, Harter P, Ray-Coquard I
Int J Gynecol Cancer 2014 Nov;24(9 Suppl 3):S73-7. doi: 10.1097/IGC.0000000000000281. PMID: 25341584
Mullerian adenosarcoma of the female genital tract.
McCluggage WG
Adv Anat Pathol 2010 Mar;17(2):122-9. doi: 10.1097/PAP.0b013e3181cfe732. PMID: 20179434
Extracutaneous malignant melanomas.
Hussein MR
Cancer Invest 2008 Jun;26(5):516-34. doi: 10.1080/07357900701781762. PMID: 18568775

Clinical prediction guides

Uterine sarcomas and rare uterine mesenchymal tumors with malignant potential. Diagnostic guidelines of the French Sarcoma Group and the Rare Gynecological Tumors Group.
Croce S, Devouassoux-Shisheboran M, Pautier P, Ray-Coquard I, Treilleux I, Neuville A, Arnould L, Just PA, Belda MALF, Averous G, Leroux A, Mery E, Loussouarn D, Weinbreck N, Le Guellec S, Mishellany F, Morice P, Guyon F, Genestie C
Gynecol Oncol 2022 Nov;167(2):373-389. Epub 2022 Sep 14 doi: 10.1016/j.ygyno.2022.07.031. PMID: 36114030
Imaging in gynecological disease (15): clinical and ultrasound characteristics of uterine sarcoma.
Ludovisi M, Moro F, Pasciuto T, Di Noi S, Giunchi S, Savelli L, Pascual MA, Sladkevicius P, Alcazar JL, Franchi D, Mancari R, Moruzzi MC, Jurkovic D, Chiappa V, Guerriero S, Exacoustos C, Epstein E, Frühauf F, Fischerova D, Fruscio R, Ciccarone F, Zannoni GF, Scambia G, Valentin L, Testa AC
Ultrasound Obstet Gynecol 2019 Nov;54(5):676-687. Epub 2019 Oct 7 doi: 10.1002/uog.20270. PMID: 30908820
Rare case of undifferentiated uterine sarcoma with neuroectodermal differentiation and osteoclast-like giant cells.
Cheng CH, Su B, Ding DC
Taiwan J Obstet Gynecol 2018 Jun;57(3):442-446. doi: 10.1016/j.tjog.2018.04.020. PMID: 29880181
Primary sarcoma of the adult vagina: a clinicopathologic study.
Peters WA 3rd, Kumar NB, Andersen WA, Morley GW
Obstet Gynecol 1985 May;65(5):699-704. PMID: 2984620
Müllerian adenosarcoma of the uterine cervix with heterologous elements: a light and electron microscopic study.
Roth LM, Pride GL, Sharma HM
Cancer 1976 Apr;37(4):1725-36. doi: 10.1002/1097-0142(197604)37:4<1725::aid-cncr2820370418>3.0.co;2-1. PMID: 177174

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