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Endometrioid stromal sarcoma of the cervix

MedGen UID:
770966
•Concept ID:
C3642326
•
Neoplastic Process
Synonyms: cervical endometrial stromal sarcoma; endometrial stromal sarcoma of the cervix; Endometrioid Stromal Sarcoma of the Cervix; endometrioid stromal sarcoma of the cervix; endometrioid stromal sarcoma of uterine cervix; uterine cervix endometrioid stromal sarcoma
 
Monarch Initiative: MONDO:0003030

Definition

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

Term Hierarchy

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

Professional guidelines

PubMed

Ewing Sarcoma of the Female Genital Tract: Clinicopathologic Analysis of 21 Cases With an Emphasis on the Differential Diagnosis of Gynecologic Round Cell, Spindle, and Epithelioid Neoplasms.
Sharma AE, Wepy CB, Chapel DB, Maccio L, Irshaid L, Al-Ibraheemi A, Dickson BC, Nucci MR, Crum CP, Fletcher CDM, Kolin DL
Am J Surg Pathol 2024 Aug 1;48(8):972-984. Epub 2024 May 2 doi: 10.1097/PAS.0000000000002232. PMID: 38708674
Primary sarcoma of the cervix: an analysis of patient and tumor characteristics, treatment patterns, and outcomes.
Albert A, Lee A, Allbright R, Vijayakumar S
J Gynecol Oncol 2020 May;31(3):e25. Epub 2019 Oct 23 doi: 10.3802/jgo.2020.31.e25. PMID: 31912680Free PMC Article

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
A multi-institutional review of outcomes of endometrial stromal sarcoma.
Leath CA 3rd, Huh WK, Hyde J Jr, Cohn DE, Resnick KE, Taylor NP, Powell MA, Mutch DG, Bradley WH, Geller MA, Argenta PA, Gold MA
Gynecol Oncol 2007 Jun;105(3):630-4. Epub 2007 Feb 23 doi: 10.1016/j.ygyno.2007.01.031. PMID: 17320937
Malignant mullerian mixed tumors of the uterine cervix: a report of nine cases of a neoplasm with morphology often different from its counterpart in the corpus.
Clement PB, Zubovits JT, Young RH, Scully RE
Int J Gynecol Pathol 1998 Jul;17(3):211-22. PMID: 9656116
Malignant lesions of the female genital tract and peritoneum that may be underdiagnosed.
Young RH, Clement PB
Semin Diagn Pathol 1995 Feb;12(1):14-29. PMID: 7770671
Stromal endometriosis of the uterine cervix. A variant of endometriosis that may simulate a sarcoma.
Clement PB, Young RH, Scully RE
Am J Surg Pathol 1990 May;14(5):449-55. doi: 10.1097/00000478-199005000-00004. PMID: 2327550

Diagnosis

Sarcoma of the uterine cervix: experience of a single center.
Yuan H, Li L, Li N, Yao H
World J Surg Oncol 2024 Apr 18;22(1):104. doi: 10.1186/s12957-024-03376-8. PMID: 38637834Free PMC Article
Pan-TRK immunohistochemistry in gynaecological mesenchymal tumours: diagnostic implications and pitfalls.
Moura MS, Costa J, Velasco V, Kommoss F, Oliva E, Le Loarer F, McCluggage WG, Razack R, Treilleux I, Mills A, Longacre T, Devouassoux-Shisheboran M, Hostein I, Azmani R, Blanchard L, Hartog C, Soubeyran I, Khalifa E, Croce S
Histopathology 2024 Feb;84(3):451-462. Epub 2023 Nov 21 doi: 10.1111/his.15082. PMID: 37988282
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
Primary Angiosarcoma of the Cervix: Case Report of a Rare Lesion.
Shah VI, Rowlands GL, Thompson IW, Sumathi VP, McCluggage WG
Int J Gynecol Pathol 2020 Jan;39(1):97-102. doi: 10.1097/PGP.0000000000000567. PMID: 31815894
Problematic issues in the staging of endometrial, cervical and vulval carcinomas.
Hirschowitz L, Nucci M, Zaino RJ
Histopathology 2013 Jan;62(1):176-202. doi: 10.1111/his.12058. PMID: 23240675

Therapy

Is fertility-preservation safe for adult non-metastatic gynecologic rhabdomyosarcoma patients? Systematic review and pooled survival analysis of 137 patients.
Elsebaie MA, Elsayed Z
Arch Gynecol Obstet 2018 Mar;297(3):559-572. Epub 2017 Nov 20 doi: 10.1007/s00404-017-4591-6. PMID: 29159540
Different types and different prognosis-study of 310 uterine sarcomas.
Nickie-Psikuta M, Gawrychowski K
Eur J Gynaecol Oncol 1993;14 Suppl:105-13. PMID: 8200360
Gynecologic Oncology Group studies with ifosfamide.
Sutton GP, Blessing JA, Manetta A, Homesley H, McGuire W
Semin Oncol 1992 Dec;19(6 Suppl 12):31-4. PMID: 1485172

Prognosis

Sarcoma of the uterine cervix: experience of a single center.
Yuan H, Li L, Li N, Yao H
World J Surg Oncol 2024 Apr 18;22(1):104. doi: 10.1186/s12957-024-03376-8. PMID: 38637834Free PMC Article
Primary sarcoma of the cervix: an analysis of patient and tumor characteristics, treatment patterns, and outcomes.
Albert A, Lee A, Allbright R, Vijayakumar S
J Gynecol Oncol 2020 May;31(3):e25. Epub 2019 Oct 23 doi: 10.3802/jgo.2020.31.e25. PMID: 31912680Free PMC Article
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
Malignant mesonephric neoplasms of the uterine cervix. A report of eight cases, including four with a malignant spindle cell component.
Clement PB, Young RH, Keh P, Ostör AG, Scully RE
Am J Surg Pathol 1995 Oct;19(10):1158-71. doi: 10.1097/00000478-199510000-00006. PMID: 7573674
Stromal endometriosis of the uterine cervix. A variant of endometriosis that may simulate a sarcoma.
Clement PB, Young RH, Scully RE
Am J Surg Pathol 1990 May;14(5):449-55. doi: 10.1097/00000478-199005000-00004. PMID: 2327550

Clinical prediction guides

Ewing Sarcoma of the Female Genital Tract: Clinicopathologic Analysis of 21 Cases With an Emphasis on the Differential Diagnosis of Gynecologic Round Cell, Spindle, and Epithelioid Neoplasms.
Sharma AE, Wepy CB, Chapel DB, Maccio L, Irshaid L, Al-Ibraheemi A, Dickson BC, Nucci MR, Crum CP, Fletcher CDM, Kolin DL
Am J Surg Pathol 2024 Aug 1;48(8):972-984. Epub 2024 May 2 doi: 10.1097/PAS.0000000000002232. PMID: 38708674
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
Adult NTRK-rearranged spindle cell neoplasms of the viscera: with an emphasis on rare locations and heterologous elements.
Tsai JW, Lee JC, Hsieh TH, Huang SC, Lee PH, Liu TT, Kao YC, Chang CD, Weng TF, Li CF, Lin JC, Liang CW, Su YL, Chang IY, Wang YT, Chang NY, Yu SC, Wang JC, Huang HY
Mod Pathol 2022 Jul;35(7):911-921. Epub 2022 Feb 11 doi: 10.1038/s41379-021-01005-3. PMID: 35149769
Malignant mesonephric neoplasms of the uterine cervix. A report of eight cases, including four with a malignant spindle cell component.
Clement PB, Young RH, Keh P, Ostör AG, Scully RE
Am J Surg Pathol 1995 Oct;19(10):1158-71. doi: 10.1097/00000478-199510000-00006. PMID: 7573674
Gynecologic Oncology Group studies with ifosfamide.
Sutton GP, Blessing JA, Manetta A, Homesley H, McGuire W
Semin Oncol 1992 Dec;19(6 Suppl 12):31-4. PMID: 1485172

Recent systematic reviews

Is fertility-preservation safe for adult non-metastatic gynecologic rhabdomyosarcoma patients? Systematic review and pooled survival analysis of 137 patients.
Elsebaie MA, Elsayed Z
Arch Gynecol Obstet 2018 Mar;297(3):559-572. Epub 2017 Nov 20 doi: 10.1007/s00404-017-4591-6. PMID: 29159540

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