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Endometrioid stromal sarcoma of the vagina

MedGen UID:
770969
•Concept ID:
C3642329
•
Neoplastic Process
Synonyms: Endometrioid Stromal Sarcoma of the Vagina; endometrioid stromal sarcoma of the vagina; endometrioid stromal sarcoma of vagina; vagina endometrioid stromal sarcoma; vaginal endometrial stromal sarcoma
 
Monarch Initiative: MONDO:0003313

Definition

A rare sarcoma that arises from the vagina. This category includes low grade endometrioid stromal sarcoma and undifferentiated vaginal sarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEndometrioid stromal sarcoma of the vagina

Recent clinical studies

Etiology

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
Clinical Characteristics and Prognosis of Unexpected Uterine Sarcoma After Hysterectomy for Presumed Myoma With and Without Transvaginal Scalpel Morcellation.
Zhang J, Li T, Zhang J, Zhu L, Lang J, Leng J
Int J Gynecol Cancer 2016 Mar;26(3):456-63. doi: 10.1097/IGC.0000000000000638. PMID: 26807642
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
Vaginal treatment of endometrial cancer: role in the elderly.
Moscarini M, Ricciardi E, Quarto A, Maniglio P, Caserta D
World J Surg Oncol 2011 Jul 13;9:74. doi: 10.1186/1477-7819-9-74. PMID: 21752282Free PMC Article
Exfoliative cytology of neuroendocrine small cell carcinoma of the endometrium. A report of two cases.
Proca D, Keyhani-Rofagha S, Copeland LJ, Hameed A
Acta Cytol 1998 Jul-Aug;42(4):978-82. doi: 10.1159/000331980. PMID: 9684589

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
Gynecological Sarcomas: Molecular Characteristics, Behavior, and Histology-Driven Therapy.
Libertini M, Hallin M, Thway K, Noujaim J, Benson C, van der Graaf W, Jones RL
Int J Surg Pathol 2021 Feb;29(1):4-20. Epub 2020 Sep 10 doi: 10.1177/1066896920958120. PMID: 32909482
Vaginal Low-Grade Endometrial Stromal Sarcoma: An Extremely Rare Case Report and Review of the Literature.
Tang Y, Chen Y, Tian L, Chen J, Yang P, Zhang D, Cui Q, Zhao L, Li L
Int J Gynecol Pathol 2020 Sep;39(5):447-451. doi: 10.1097/PGP.0000000000000626. PMID: 31569185
Endometrioid stromal sarcoma: a clinicopathologic study of 63 cases.
Masand RP, Euscher ED, Deavers MT, Malpica A
Am J Surg Pathol 2013 Nov;37(11):1635-47. doi: 10.1097/PAS.0000000000000083. PMID: 24121169
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

Therapy

Clinical Characteristics and Prognosis of Unexpected Uterine Sarcoma After Hysterectomy for Presumed Myoma With and Without Transvaginal Scalpel Morcellation.
Zhang J, Li T, Zhang J, Zhu L, Lang J, Leng J
Int J Gynecol Cancer 2016 Mar;26(3):456-63. doi: 10.1097/IGC.0000000000000638. PMID: 26807642
Endometrial stromal sarcoma arising in vagina.
Liu Z, Ding J, Li X, Yu K
Int J Clin Exp Pathol 2013;6(12):2997-3002. Epub 2013 Nov 15 PMID: 24294390Free PMC Article

Prognosis

Gynecological Sarcomas: Molecular Characteristics, Behavior, and Histology-Driven Therapy.
Libertini M, Hallin M, Thway K, Noujaim J, Benson C, van der Graaf W, Jones RL
Int J Surg Pathol 2021 Feb;29(1):4-20. Epub 2020 Sep 10 doi: 10.1177/1066896920958120. PMID: 32909482
Clinical Characteristics and Prognosis of Unexpected Uterine Sarcoma After Hysterectomy for Presumed Myoma With and Without Transvaginal Scalpel Morcellation.
Zhang J, Li T, Zhang J, Zhu L, Lang J, Leng J
Int J Gynecol Cancer 2016 Mar;26(3):456-63. doi: 10.1097/IGC.0000000000000638. PMID: 26807642
Endometrial stromal sarcoma arising in vagina.
Liu Z, Ding J, Li X, Yu K
Int J Clin Exp Pathol 2013;6(12):2997-3002. Epub 2013 Nov 15 PMID: 24294390Free PMC Article
Endometrioid stromal sarcoma: a clinicopathologic study of 63 cases.
Masand RP, Euscher ED, Deavers MT, Malpica A
Am J Surg Pathol 2013 Nov;37(11):1635-47. doi: 10.1097/PAS.0000000000000083. PMID: 24121169
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

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
Gynecological Sarcomas: Molecular Characteristics, Behavior, and Histology-Driven Therapy.
Libertini M, Hallin M, Thway K, Noujaim J, Benson C, van der Graaf W, Jones RL
Int J Surg Pathol 2021 Feb;29(1):4-20. Epub 2020 Sep 10 doi: 10.1177/1066896920958120. PMID: 32909482
Low-grade Endometrial Stromal Sarcoma Primarily Arising in the Vagina: A Case Report.
Rivard C, Carter JS, Downs LS Jr
J Reprod Med 2015 Sep-Oct;60(9-10):433-5. PMID: 26592071
Endometrioid stromal sarcoma: a clinicopathologic study of 63 cases.
Masand RP, Euscher ED, Deavers MT, Malpica A
Am J Surg Pathol 2013 Nov;37(11):1635-47. doi: 10.1097/PAS.0000000000000083. PMID: 24121169
Cytology of vaginal and uterine sarcomas.
Ito E, Saito T, Suzuki T, Fujii M, Kudo R
Acta Cytol 2004 Sep-Oct;48(5):601-7. doi: 10.1159/000326429. PMID: 15471250

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