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Uterine corpus rhabdomyosarcoma

MedGen UID:
925579
•Concept ID:
C4288047
•
Neoplastic Process
Synonyms: body of uterus rhabdomyosarcoma; body of uterus rhabdomyosarcoma (disease); rhabdomyosarcoma (disease) of body of uterus; rhabdomyosarcoma of the corpus uteri; Rhabdomyosarcoma of the corpus uteri; Uterine Corpus Rhabdomyosarcoma; uterine corpus rhabdomyosarcoma
 
Monarch Initiative: MONDO:0016260
Orphanet: ORPHA213615

Definition

Rhabdomyosarcoma of the corpus uteri is an extremely rare, highly malignant soft tissue sarcoma located in the uterine body and arising from primitive mesenchymal cells displaying variable degrees of skeletal muscle differentiation. It most often presents with abnormal vaginal discharge or dysfunctional uterine bleeding, abdominal pain and lower abdominal mass. Association with DICER1 syndrome has been reported. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUterine corpus rhabdomyosarcoma

Professional guidelines

PubMed

Local treatment of rhabdomyosarcoma of the female genital tract: Expert consensus from the Children's Oncology Group, the European Soft-Tissue Sarcoma Group, and the Cooperative Weichteilsarkom Studiengruppe.
Lautz TB, Martelli H, Fuchs J, Chargari C, Smeulders N, Granberg CF, Wolden SL, Sparber-Sauer M, Hawkins DS, Bisogno G, Koscielniak E, Rodeberg DA, Seitz G; INSTRuCT group
Pediatr Blood Cancer 2023 May;70(5):e28601. Epub 2020 Aug 6 doi: 10.1002/pbc.28601. PMID: 32762004
DICER1-associated embryonal rhabdomyosarcoma and adenosarcoma of the gynecologic tract: Pathology, molecular genetics, and indications for molecular testing.
Apellaniz-Ruiz M, McCluggage WG, Foulkes WD
Genes Chromosomes Cancer 2021 Mar;60(3):217-233. Epub 2020 Nov 19 doi: 10.1002/gcc.22913. PMID: 33135284

Recent clinical studies

Etiology

A rare adult case of primary uterine rhabdomyosarcoma with mixed pattern: a clinicopathological & immunohistochemical study with literature review.
Kamel NKH, Hasby EA
Diagn Pathol 2024 Jul 17;19(1):98. doi: 10.1186/s13000-024-01518-w. PMID: 39020398Free PMC Article
Embryonal rhabdomyosarcoma of the uterine corpus: a clinicopathological and molecular analysis of 21 cases highlighting a frequent association with DICER1 mutations.
Bennett JA, Ordulu Z, Young RH, Pinto A, Van de Vijver K, Burandt E, Wanjari P, Shah R, de Kock L, Foulkes WD, McCluggage WG, Ritterhouse LL, Oliva E
Mod Pathol 2021 Sep;34(9):1750-1762. Epub 2021 May 20 doi: 10.1038/s41379-021-00821-x. PMID: 34017064
DICER1-Related Embryonal Rhabdomyosarcoma of the Uterine Corpus in a Prepubertal Girl.
Dural O, Kebudi R, Yavuz E, Yilmaz I, Buyukkapu Bay S, Schultz KAP, Hill DA
J Pediatr Adolesc Gynecol 2020 Apr;33(2):173-176. Epub 2019 Dec 12 doi: 10.1016/j.jpag.2019.12.002. PMID: 31838154Free PMC Article
Localized vaginal/uterine rhabdomyosarcoma-results of a pooled analysis from four international cooperative groups.
Minard-Colin V, Walterhouse D, Bisogno G, Martelli H, Anderson J, Rodeberg DA, Ferrari A, Jenney M, Wolden S, De Salvo G, Arndt C, Merks JHM, Gallego S, Schwob D, Haie-Meder C, Bergeron C, Stevens MCG, Oberlin O, Hawkins D; International Society of Pediatric Oncology Sarcoma Committee, the Children's Oncology Group, the Italian Cooperative Soft Tissue Sarcoma Group, and the European pediatric Soft tissue sarcoma Study Group
Pediatr Blood Cancer 2018 Sep;65(9):e27096. Epub 2018 May 21 doi: 10.1002/pbc.27096. PMID: 29781567Free PMC Article
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

Diagnosis

A rare adult case of primary uterine rhabdomyosarcoma with mixed pattern: a clinicopathological & immunohistochemical study with literature review.
Kamel NKH, Hasby EA
Diagn Pathol 2024 Jul 17;19(1):98. doi: 10.1186/s13000-024-01518-w. PMID: 39020398Free PMC Article
Embryonal rhabdomyosarcoma of the uterine corpus: a clinicopathological and molecular analysis of 21 cases highlighting a frequent association with DICER1 mutations.
Bennett JA, Ordulu Z, Young RH, Pinto A, Van de Vijver K, Burandt E, Wanjari P, Shah R, de Kock L, Foulkes WD, McCluggage WG, Ritterhouse LL, Oliva E
Mod Pathol 2021 Sep;34(9):1750-1762. Epub 2021 May 20 doi: 10.1038/s41379-021-00821-x. PMID: 34017064
DICER1-associated embryonal rhabdomyosarcoma and adenosarcoma of the gynecologic tract: Pathology, molecular genetics, and indications for molecular testing.
Apellaniz-Ruiz M, McCluggage WG, Foulkes WD
Genes Chromosomes Cancer 2021 Mar;60(3):217-233. Epub 2020 Nov 19 doi: 10.1002/gcc.22913. PMID: 33135284
Embryonal rhabdomyosarcoma (botryoid type) of the uterine corpus and cervix in adult women: report of a case series and review of the literature.
Li RF, Gupta M, McCluggage WG, Ronnett BM
Am J Surg Pathol 2013 Mar;37(3):344-55. doi: 10.1097/PAS.0b013e31826e0271. PMID: 23348207
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

Therapy

Local treatment of rhabdomyosarcoma of the female genital tract: Expert consensus from the Children's Oncology Group, the European Soft-Tissue Sarcoma Group, and the Cooperative Weichteilsarkom Studiengruppe.
Lautz TB, Martelli H, Fuchs J, Chargari C, Smeulders N, Granberg CF, Wolden SL, Sparber-Sauer M, Hawkins DS, Bisogno G, Koscielniak E, Rodeberg DA, Seitz G; INSTRuCT group
Pediatr Blood Cancer 2023 May;70(5):e28601. Epub 2020 Aug 6 doi: 10.1002/pbc.28601. PMID: 32762004
Localized vaginal/uterine rhabdomyosarcoma-results of a pooled analysis from four international cooperative groups.
Minard-Colin V, Walterhouse D, Bisogno G, Martelli H, Anderson J, Rodeberg DA, Ferrari A, Jenney M, Wolden S, De Salvo G, Arndt C, Merks JHM, Gallego S, Schwob D, Haie-Meder C, Bergeron C, Stevens MCG, Oberlin O, Hawkins D; International Society of Pediatric Oncology Sarcoma Committee, the Children's Oncology Group, the Italian Cooperative Soft Tissue Sarcoma Group, and the European pediatric Soft tissue sarcoma Study Group
Pediatr Blood Cancer 2018 Sep;65(9):e27096. Epub 2018 May 21 doi: 10.1002/pbc.27096. PMID: 29781567Free PMC Article
Embryonal rhabdomyosarcoma of the uterine corpus.
Garrett LA, Harmon DC, Schorge JO
J Clin Oncol 2013 Feb 1;31(4):e48-50. Epub 2012 Dec 17 doi: 10.1200/JCO.2012.43.1841. PMID: 23248250
Primary chemotherapy with or without radiation therapy and/or surgery for children with localized sarcoma of the bladder, prostate, vagina, uterus, and cervix. A comparison of the results in Intergroup Rhabdomyosarcoma Studies I and II.
Raney RB Jr, Gehan EA, Hays DM, Tefft M, Newton WA Jr, Haeberlen V, Maurer HM
Cancer 1990 Nov 15;66(10):2072-81. doi: 10.1002/1097-0142(19901115)66:10<2072::aid-cncr2820661006>3.0.co;2-9. PMID: 2224761
Embryonal rhabdomyosarcoma of the uterine corpus and cervix.
Montag TW, D'ablaing G, Schlaerth JB, Gaddis O Jr, Morrow CP
Gynecol Oncol 1986 Oct;25(2):171-94. doi: 10.1016/0090-8258(86)90098-3. PMID: 3758828

Prognosis

A rare adult case of primary uterine rhabdomyosarcoma with mixed pattern: a clinicopathological & immunohistochemical study with literature review.
Kamel NKH, Hasby EA
Diagn Pathol 2024 Jul 17;19(1):98. doi: 10.1186/s13000-024-01518-w. PMID: 39020398Free PMC Article
Rhabdomyosarcoma of the uterus in an adult patient with osteopetrosis: a case report.
Aminimoghaddam S, Rahbari A, Pourali R
J Med Case Rep 2021 Nov 28;15(1):570. doi: 10.1186/s13256-021-03172-y. PMID: 34838127Free PMC Article
Localized vaginal/uterine rhabdomyosarcoma-results of a pooled analysis from four international cooperative groups.
Minard-Colin V, Walterhouse D, Bisogno G, Martelli H, Anderson J, Rodeberg DA, Ferrari A, Jenney M, Wolden S, De Salvo G, Arndt C, Merks JHM, Gallego S, Schwob D, Haie-Meder C, Bergeron C, Stevens MCG, Oberlin O, Hawkins D; International Society of Pediatric Oncology Sarcoma Committee, the Children's Oncology Group, the Italian Cooperative Soft Tissue Sarcoma Group, and the European pediatric Soft tissue sarcoma Study Group
Pediatr Blood Cancer 2018 Sep;65(9):e27096. Epub 2018 May 21 doi: 10.1002/pbc.27096. PMID: 29781567Free PMC Article
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
Pleomorphic rhabdomyosarcoma of the uterine corpus: a clinicopathologic study of 4 cases and a review of the literature.
Fadare O, Bonvicino A, Martel M, Renshaw IL, Azodi M, Parkash V
Int J Gynecol Pathol 2010 Mar;29(2):122-34. doi: 10.1097/PGP.0b013e3181bc98c0. PMID: 20173498

Clinical prediction guides

Clinicopathologic and molecular analysis of embryonal rhabdomyosarcoma of the genitourinary tract: evidence for a distinct DICER1-associated subgroup.
Kommoss FKF, Stichel D, Mora J, Esteller M, Jones DTW, Pfister SM, Brack E, Wachtel M, Bode PK, Sinn HP, Schmidt D, Mentzel T, Kommoss F, Sahm F, von Deimling A, Koelsche C
Mod Pathol 2021 Aug;34(8):1558-1569. Epub 2021 Apr 12 doi: 10.1038/s41379-021-00804-y. PMID: 33846547Free PMC Article
Embryonal rhabdomyosarcoma arising from the uterine corpus in a postmenopausal female: a surgical case challenging the genuine diagnosis on a cytology specimen.
Yamada S, Harada Y, Noguchi H, Satoh N, Kimura S, Nakayama T, Tanimoto A
Diagn Pathol 2016 Jan 12;11:3. doi: 10.1186/s13000-016-0451-0. PMID: 26755204Free PMC Article
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

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