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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

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
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

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
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
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
Sangoi AR, Kshirsagar M, Horvai AE, Roma AA
Int J Gynecol Pathol 2017 Mar;36(2):140-145. doi: 10.1097/PGP.0000000000000301. PMID: 27294605
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

Diagnosis

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
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
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
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
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

Therapy

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
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
Chmaj-Wierzchowska K, Wierzchowski M, Szymanowski K, Czerniak T, Mróz M, Sobiak S, Opala T
Ginekol Pol 2010 Jul;81(7):541-3. PMID: 20825058
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
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

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
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
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
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
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

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
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
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

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