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Ovary rhabdomyosarcoma

MedGen UID:
233277
•Concept ID:
C1335176
•
Neoplastic Process
Synonyms: Ovarian Rhabdomyosarcoma; ovarian rhabdomyosarcoma; ovary rhabdomyosarcoma; ovary rhabdomyosarcoma (disease); rhabdomyosarcoma (disease) of ovary; Rhabdomyosarcoma of Ovary; rhabdomyosarcoma of ovary; Rhabdomyosarcoma of the Ovary; rhabdomyosarcoma of the ovary
 
Monarch Initiative: MONDO:0002858

Definition

A malignant mesenchymal tumor with skeletal muscle differentiation affecting the ovaries. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvary rhabdomyosarcoma

Professional guidelines

PubMed

Ovarian conservation in management of pediatric gynecology malignancies.
Stambough K, Childress KJ
Curr Opin Obstet Gynecol 2018 Oct;30(5):316-325. doi: 10.1097/GCO.0000000000000484. PMID: 30113330
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):168-77. Epub 2015 Mar 26 doi: 10.1177/0394632015576029. PMID: 25816394
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393

Recent clinical studies

Etiology

Li-Fraumeni Syndrome: Imaging Features and Guidelines.
Gosangi B, Dixe de Oliveira Santo I, Keraliya A, Wang Y, Irugu D, Thomas R, Khandelwal A, Rubinowitz AN, Bader AS
Radiographics 2024 Aug;44(8):e230202. doi: 10.1148/rg.230202. PMID: 39024172
Gynecologic Manifestations of the DICER1 Syndrome.
Stewart CJ, Charles A, Foulkes WD
Surg Pathol Clin 2016 Jun;9(2):227-41. Epub 2016 Apr 9 doi: 10.1016/j.path.2016.01.002. PMID: 27241106
Vaginal Bleeding in Prepubertal Girls: Etiology and Clinical Management.
Söderström HF, Carlsson A, Börjesson A, Elfving M
J Pediatr Adolesc Gynecol 2016 Jun;29(3):280-5. Epub 2015 Oct 24 doi: 10.1016/j.jpag.2015.10.017. PMID: 26506030
DICER1 pleuropulmonary blastoma familial tumour predisposition syndrome: What the paediatric urologist needs to know.
Faure A, Atkinson J, Bouty A, O'Brien M, Levard G, Hutson J, Heloury Y
J Pediatr Urol 2016 Feb;12(1):5-10. Epub 2015 Sep 26 doi: 10.1016/j.jpurol.2015.08.012. PMID: 26454454
Ovarian transposition in prepubescent and adolescent girls with cancer.
Irtan S, Orbach D, Helfre S, Sarnacki S
Lancet Oncol 2013 Dec;14(13):e601-8. doi: 10.1016/S1470-2045(13)70288-2. PMID: 24275133

Diagnosis

DICER1-mutated rhabdomyosarcoma of the ovary with teratoid features.
Lethongsavarn V, Vieille P, Kikweta Makhama J, Azmani R, Lafrance W, Khneisser P, Truffaut N, Alame M, Genestie C, Gaspar N, Diedhiou A, Croce S, Le Loarer F
Genes Chromosomes Cancer 2023 Dec;62(12):746-754. Epub 2023 Sep 14 doi: 10.1002/gcc.23201. PMID: 37706559
Gynecologic Manifestations of the DICER1 Syndrome.
Stewart CJ, Charles A, Foulkes WD
Surg Pathol Clin 2016 Jun;9(2):227-41. Epub 2016 Apr 9 doi: 10.1016/j.path.2016.01.002. PMID: 27241106
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):168-77. Epub 2015 Mar 26 doi: 10.1177/0394632015576029. PMID: 25816394
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393
Ovarian transposition in prepubescent and adolescent girls with cancer.
Irtan S, Orbach D, Helfre S, Sarnacki S
Lancet Oncol 2013 Dec;14(13):e601-8. doi: 10.1016/S1470-2045(13)70288-2. PMID: 24275133

Therapy

Ovarian conservation in management of pediatric gynecology malignancies.
Stambough K, Childress KJ
Curr Opin Obstet Gynecol 2018 Oct;30(5):316-325. doi: 10.1097/GCO.0000000000000484. PMID: 30113330
Ovarian transposition in prepubescent and adolescent girls with cancer.
Irtan S, Orbach D, Helfre S, Sarnacki S
Lancet Oncol 2013 Dec;14(13):e601-8. doi: 10.1016/S1470-2045(13)70288-2. PMID: 24275133
Preclinical anticancer activity of the specific endothelin A receptor antagonist ZD4054.
Growcott JW
Anticancer Drugs 2009 Feb;20(2):83-8. doi: 10.1097/CAD.0b013e328320791c. PMID: 19065106
Conservative surgery plus adjuvant therapy for vulvovaginal rhabdomyosarcoma, diethylstilbestrol clear cell adenocarcinoma of the vagina, and unilateral germ cell tumors of the ovary.
Hicks ML, Piver MS
Obstet Gynecol Clin North Am 1992 Mar;19(1):219-33. PMID: 1316595
The systemic administration of intravenous melphalan.
Sarosy G, Leyland-Jones B, Soochan P, Cheson BD
J Clin Oncol 1988 Nov;6(11):1768-82. doi: 10.1200/JCO.1988.6.11.1768. PMID: 3054005

Prognosis

Gynecologic Manifestations of the DICER1 Syndrome.
Stewart CJ, Charles A, Foulkes WD
Surg Pathol Clin 2016 Jun;9(2):227-41. Epub 2016 Apr 9 doi: 10.1016/j.path.2016.01.002. PMID: 27241106
DICER1 pleuropulmonary blastoma familial tumour predisposition syndrome: What the paediatric urologist needs to know.
Faure A, Atkinson J, Bouty A, O'Brien M, Levard G, Hutson J, Heloury Y
J Pediatr Urol 2016 Feb;12(1):5-10. Epub 2015 Sep 26 doi: 10.1016/j.jpurol.2015.08.012. PMID: 26454454
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):168-77. Epub 2015 Mar 26 doi: 10.1177/0394632015576029. PMID: 25816394
Ovarian transposition in prepubescent and adolescent girls with cancer.
Irtan S, Orbach D, Helfre S, Sarnacki S
Lancet Oncol 2013 Dec;14(13):e601-8. doi: 10.1016/S1470-2045(13)70288-2. PMID: 24275133
The systemic administration of intravenous melphalan.
Sarosy G, Leyland-Jones B, Soochan P, Cheson BD
J Clin Oncol 1988 Nov;6(11):1768-82. doi: 10.1200/JCO.1988.6.11.1768. PMID: 3054005

Clinical prediction guides

New concept in urologic surgery: The total extended genital sparing radical cystectomy in women.
Cisternino A, Capone L, Rosati A, Latiano C, Sebastio N, Colella A, Cretì G
Arch Ital Urol Androl 2023 May 22;95(2):11058. doi: 10.4081/aiua.2023.11058. PMID: 37212881
Uncommon hereditary gynaecological tumour syndromes: pathological features in tumours that may predict risk for a germline mutation.
Garg K, Karnezis AN, Rabban JT
Pathology 2018 Feb;50(2):238-256. Epub 2018 Jan 17 doi: 10.1016/j.pathol.2017.10.009. PMID: 29373116
Rhabdomyosarcoma arising in the hand or foot: a clinicopathologic analysis.
Miller DV, Coffin CM, Zhou H
Pediatr Dev Pathol 2004 Jul-Aug;7(4):361-9. Epub 2004 Jun 17 doi: 10.1007/s10024-003-6071-1. PMID: 15383931
Management of stage I cervical sarcoma botryoides in childhood and adolescence.
Gruessner SE, Omwandho CO, Dreyer T, Blütters-Sawatzki R, Reiter A, Tinneberg HR, Bohle RM
Eur J Pediatr 2004 Aug;163(8):452-6. Epub 2004 Jun 2 doi: 10.1007/s00431-004-1469-y. PMID: 15173941
The systemic administration of intravenous melphalan.
Sarosy G, Leyland-Jones B, Soochan P, Cheson BD
J Clin Oncol 1988 Nov;6(11):1768-82. doi: 10.1200/JCO.1988.6.11.1768. PMID: 3054005

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