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Myxoid leiomyosarcoma

MedGen UID:
104725
•Concept ID:
C0205816
•
Neoplastic Process
Synonyms: Leiomyosarcoma, Myxoid; Leiomyosarcomas, Myxoid; Myxoid Leiomyosarcoma; Myxoid Leiomyosarcomas
SNOMED CT: Myxoid leiomyosarcoma (16090008)
 
Monarch Initiative: MONDO:0003359

Definition

A morphologic variant of leiomyosarcoma characterized by the presence of cellular pleomorphism, malignant cells with large nuclei, and a myxoid stroma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyxoid leiomyosarcoma

Professional guidelines

PubMed

Myxoid Mesenchymal Tumors of the Uterus: An Update on Classification, Definitions, and Differential Diagnosis.
Busca A, Parra-Herran C
Adv Anat Pathol 2017 Nov;24(6):354-361. doi: 10.1097/PAP.0000000000000164. PMID: 28787279
The value of fine-needle aspiration biopsy in the differential diagnosis of adult myxoid sarcoma.
Kilpatrick SE, Ward WG, Bos GD
Cancer 2000 Jun 25;90(3):167-77. doi: 10.1002/1097-0142(20000625)90:3<167::aid-cncr5>3.0.co;2-z. PMID: 10896330
Diffuse, perinodular, and other patterns of hydropic degeneration within and adjacent to uterine leiomyomas. Problems in differential diagnosis.
Clement PB, Young RH, Scully RE
Am J Surg Pathol 1992 Jan;16(1):26-32. doi: 10.1097/00000478-199201000-00004. PMID: 1309411

Recent clinical studies

Etiology

PLAG1-rearrangment in a uterine leiomyosarcoma with myxoid stroma and heterologous differentiation.
Thiryayi SA, Turashvili G, Latta EK, Swanson D, Zhang L, Antonescu CR, Dickson BC
Genes Chromosomes Cancer 2021 Oct;60(10):713-717. Epub 2021 Jul 8 doi: 10.1002/gcc.22980. PMID: 34184333Free PMC Article
Inflammatory Myofibroblastic Tumor in Female Genital Tract.
Shukla PS, Mittal K
Arch Pathol Lab Med 2019 Jan;143(1):122-129. Epub 2018 Jul 2 doi: 10.5858/arpa.2017-0575-RA. PMID: 29965784
Myxoid leiomyosarcoma of the uterus.
Burch DM, Tavassoli FA
Histopathology 2011 Dec;59(6):1144-55. doi: 10.1111/j.1365-2559.2011.04053.x. PMID: 22175894
Endometrial stromal tumors: an update on a group of tumors with a protean phenotype.
Oliva E, Clement PB, Young RH
Adv Anat Pathol 2000 Sep;7(5):257-81. doi: 10.1097/00125480-200007050-00001. PMID: 10976906
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

Diagnosis

PLAG1-rearrangment in a uterine leiomyosarcoma with myxoid stroma and heterologous differentiation.
Thiryayi SA, Turashvili G, Latta EK, Swanson D, Zhang L, Antonescu CR, Dickson BC
Genes Chromosomes Cancer 2021 Oct;60(10):713-717. Epub 2021 Jul 8 doi: 10.1002/gcc.22980. PMID: 34184333Free PMC Article
Inflammatory Myofibroblastic Tumor in Female Genital Tract.
Shukla PS, Mittal K
Arch Pathol Lab Med 2019 Jan;143(1):122-129. Epub 2018 Jul 2 doi: 10.5858/arpa.2017-0575-RA. PMID: 29965784
Endometrial stromal sarcomas and related neoplasms: new developments and diagnostic considerations.
Hoang L, Chiang S, Lee CH
Pathology 2018 Feb;50(2):162-177. Epub 2017 Dec 22 doi: 10.1016/j.pathol.2017.11.086. PMID: 29275929
Myxoid Mesenchymal Tumors of the Uterus: An Update on Classification, Definitions, and Differential Diagnosis.
Busca A, Parra-Herran C
Adv Anat Pathol 2017 Nov;24(6):354-361. doi: 10.1097/PAP.0000000000000164. PMID: 28787279
Myxoid leiomyosarcoma.
Salm R, Evans DJ
Histopathology 1985 Feb;9(2):159-69. doi: 10.1111/j.1365-2559.1985.tb02432.x. PMID: 3988243

Therapy

Gastrointestinal stromal tumors with BRAF gene fusions. A report of two cases showing low or absent KIT expression resulting in diagnostic pitfalls.
Torrence D, Xie Z, Zhang L, Chi P, Antonescu CR
Genes Chromosomes Cancer 2021 Dec;60(12):789-795. Epub 2021 Aug 25 doi: 10.1002/gcc.22991. PMID: 34398495Free PMC Article
Myxoid leiomyosarcoma of the uterus in a patient receiving tamoxifen therapy: a case report.
Botsis D, Koliopoulos C, Kondi-Pafitis A, Creatsas G
Int J Gynecol Pathol 2006 Apr;25(2):173-5. doi: 10.1097/01.pgp.0000185407.93308.ce. PMID: 16633068
Uterine myxoid leiomyosarcoma and CYVADIC-etoposide therapy.
Fukunishi H, Yukimura N, Takeuchi S, Kitazawa S
Int J Gynaecol Obstet 1994 Sep;46(3):321-4. doi: 10.1016/0020-7292(94)90413-8. PMID: 7806003
Myxoid leiomyosarcoma of the uterus.
Kunzel KE, Mills NZ, Muderspach LI, d'Ablaing G 3rd
Gynecol Oncol 1993 Feb;48(2):277-80. doi: 10.1006/gyno.1993.1048. PMID: 8428703

Prognosis

Myxoid Mesenchymal Tumors of the Uterus: An Update on Classification, Definitions, and Differential Diagnosis.
Busca A, Parra-Herran C
Adv Anat Pathol 2017 Nov;24(6):354-361. doi: 10.1097/PAP.0000000000000164. PMID: 28787279
Myxoid leiomyosarcoma of the uterus: a clinicopathological and immunohistochemical study of 10 cases.
Lu B, Shi H, Zhang X
Hum Pathol 2017 Jan;59:139-146. Epub 2016 Oct 4 doi: 10.1016/j.humpath.2016.09.014. PMID: 27717885
Myxoid leiomyosarcoma of the uterus: a case report and review of the literature.
Zhang L, Li Y, Qu Q, Wen K
Eur J Gynaecol Oncol 2016;37(6):870-872. PMID: 29943940
Myxoid Leiomyosarcoma of the Uterus: A Clinicopathologic Analysis of 30 Cases and Review of the Literature With Reappraisal of Its Distinction From Other Uterine Myxoid Mesenchymal Neoplasms.
Parra-Herran C, Schoolmeester JK, Yuan L, Dal Cin P, Fletcher CD, Quade BJ, Nucci MR
Am J Surg Pathol 2016 Mar;40(3):285-301. doi: 10.1097/PAS.0000000000000593. PMID: 26866354
Myxoid leiomyosarcoma.
Salm R, Evans DJ
Histopathology 1985 Feb;9(2):159-69. doi: 10.1111/j.1365-2559.1985.tb02432.x. PMID: 3988243

Clinical prediction guides

Uterine myxoid leiomyosarcoma - a rare malignant tumor: the role of complex morphopathological assay. Review and case presentation.
Istrate-Ofiţeru AM, Zorilă GL, Ruican D, Petrescu AM, Berbecaru EIA, Roşu GC, Căpitănescu RG, Nagy RD, Cercelaru L, Edu A, Iliescu DG, Drăguşin RC
Rom J Morphol Embryol 2021 Oct-Dec;62(4):883-896. doi: 10.47162/RJME.62.4.01. PMID: 35673808Free PMC Article
Myxoid Mesenchymal Tumors of the Uterus: An Update on Classification, Definitions, and Differential Diagnosis.
Busca A, Parra-Herran C
Adv Anat Pathol 2017 Nov;24(6):354-361. doi: 10.1097/PAP.0000000000000164. PMID: 28787279
Abnormal p53 and p16 staining patterns distinguish uterine leiomyosarcoma from inflammatory myofibroblastic tumour.
Schaefer IM, Hornick JL, Sholl LM, Quade BJ, Nucci MR, Parra-Herran C
Histopathology 2017 Jun;70(7):1138-1146. Epub 2017 Mar 20 doi: 10.1111/his.13176. PMID: 28130839
Myxoid leiomyosarcoma of the uterus: a clinicopathological and immunohistochemical study of 10 cases.
Lu B, Shi H, Zhang X
Hum Pathol 2017 Jan;59:139-146. Epub 2016 Oct 4 doi: 10.1016/j.humpath.2016.09.014. PMID: 27717885
Myxoid Leiomyosarcoma of the Uterus: A Clinicopathologic Analysis of 30 Cases and Review of the Literature With Reappraisal of Its Distinction From Other Uterine Myxoid Mesenchymal Neoplasms.
Parra-Herran C, Schoolmeester JK, Yuan L, Dal Cin P, Fletcher CD, Quade BJ, Nucci MR
Am J Surg Pathol 2016 Mar;40(3):285-301. doi: 10.1097/PAS.0000000000000593. PMID: 26866354

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