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Rhabdomyosarcoma of the cervix uteri

MedGen UID:
927341
•Concept ID:
C4289809
•
Neoplastic Process
Synonyms: CERMS; Cervical Rhabdomyosarcoma; Cervical rhabdomyosarcoma; cervical rhabdomyosarcoma; rhabdomyosarcoma (disease) of uterine cervix; Rhabdomyosarcoma of cervix uteri; rhabdomyosarcoma of the cervix uteri; uterine cervix rhabdomyosarcoma; uterine cervix rhabdomyosarcoma (disease)
SNOMED CT: Rhabdomyosarcoma of cervix uteri (763408003); Cervical rhabdomyosarcoma (763408003)
 
Monarch Initiative: MONDO:0016282
Orphanet: ORPHA213802

Definition

A rare highly malignant soft tissue sarcoma located in the uterine cervix and arising from primitive mesenchymal cells displaying skeletal muscle differentiation. It most often presents with abnormal vaginal discharge or dysfunctional uterine bleeding, abdominal pain and/or a cervical mass protruding into the vagina. Association with DICER1 syndrome has been reported. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRhabdomyosarcoma of the cervix uteri

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
Embryonal Rhabdomyosarcoma of the Uterine Cervix: A Clinicopathologic Study of 94 Cases Emphasizing Issues in Differential Diagnosis Staging, and Prognostic Factors.
Devins KM, Young RH, Ghioni M, Burandt E, Bennett JA, Oliva E
Am J Surg Pathol 2022 Nov 1;46(11):1477-1489. Epub 2022 Aug 9 doi: 10.1097/PAS.0000000000001933. PMID: 35941719
Sarcomas of uterine cervix: clinicopathological features, treatment, and outcome.
Khosla D, Gupta R, Srinivasan R, Patel FD, Rajwanshi A
Int J Gynecol Cancer 2012 Jul;22(6):1026-30. doi: 10.1097/IGC.0b013e31825a97f6. PMID: 22740005

Recent clinical studies

Etiology

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
HPV infection and p16(INK4A) and TP53 expression in rare cancers of the uterine cervix.
Missaoui N, Mestiri S, Bdioui A, Zahmoul T, Hamchi H, Mokni M, Hmissa S
Pathol Res Pract 2018 Apr;214(4):498-506. Epub 2018 Mar 6 doi: 10.1016/j.prp.2018.03.004. PMID: 29572122
Sarcomas of uterine cervix: clinicopathological features, treatment, and outcome.
Khosla D, Gupta R, Srinivasan R, Patel FD, Rajwanshi A
Int J Gynecol Cancer 2012 Jul;22(6):1026-30. doi: 10.1097/IGC.0b013e31825a97f6. PMID: 22740005
Alveolar rhabdomyosarcoma of the cervix.
Ng TY, Loo KT, Leung TW, Lo SH, Yuen KK
Gynecol Oncol 2003 Dec;91(3):623-6. doi: 10.1016/s0090-8258(03)00252-x. PMID: 14675688
Rhabdomyosarcoma of the uterus in a postmenopausal patient.
Podczaski E, Sees J, Kaminski P, Sorosky J, Larson JE, DeGeest K, Zaino RJ, Mortel R
Gynecol Oncol 1990 Jun;37(3):439-42. doi: 10.1016/0090-8258(90)90384-w. PMID: 2190875

Diagnosis

Embryonal Rhabdomyosarcoma of the Uterine Cervix: A Clinicopathologic Study of 94 Cases Emphasizing Issues in Differential Diagnosis Staging, and Prognostic Factors.
Devins KM, Young RH, Ghioni M, Burandt E, Bennett JA, Oliva E
Am J Surg Pathol 2022 Nov 1;46(11):1477-1489. Epub 2022 Aug 9 doi: 10.1097/PAS.0000000000001933. PMID: 35941719
Rhabdomyosarcoma of the Cervix in a Post-Menopausal Woman-An Unparalleled Phenomenon.
Pawlik J, Pawlik W, Branecka-Woźniak D, Kotrych K, Cymbaluk-Płoska A
Int J Environ Res Public Health 2021 Jul 24;18(15) doi: 10.3390/ijerph18157851. PMID: 34360144Free PMC Article
Embryonal rhabdomyosarcoma of the uterine cervix in adults: a case report and literature review.
Ditto A, Martinelli F, Carcangiu M, Solima E, de Carrillo KJ, Sanfilippo R, Haeusler E, Raspagliesi F
J Low Genit Tract Dis 2013 Oct;17(4):e12-7. doi: 10.1097/LGT.0b013e31827a8b8c. PMID: 23903199
Embryonal rhabdomyosarcoma of the uterine cervix in a 41-year-old woman: a deceptively benign entity.
Jain S, Jain K, Chopra P
Int J Gynecol Pathol 2013 Jul;32(4):421-5. doi: 10.1097/PGP.0b013e31826a646a. PMID: 23722516
Embryonal rhabdomyosarcoma of the cervix and appendiceal carcinoid tumor.
Adams BN, Brandt JS, Loukeris K, Holcomb K
Obstet Gynecol 2011 Feb;117(2 Pt 2):482-484. doi: 10.1097/AOG.0b013e3182051dd0. PMID: 21252797

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
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
Embryonal rhabdomyosarcoma of the uterine cervix in adults: a case report and literature review.
Ditto A, Martinelli F, Carcangiu M, Solima E, de Carrillo KJ, Sanfilippo R, Haeusler E, Raspagliesi F
J Low Genit Tract Dis 2013 Oct;17(4):e12-7. doi: 10.1097/LGT.0b013e31827a8b8c. PMID: 23903199
Embryonal rhabdomyosarcoma of the cervix and appendiceal carcinoid tumor.
Adams BN, Brandt JS, Loukeris K, Holcomb K
Obstet Gynecol 2011 Feb;117(2 Pt 2):482-484. doi: 10.1097/AOG.0b013e3182051dd0. PMID: 21252797
Case report of a sarcoma botryoides of the uterine cervix in fertile age and literature review.
Scaravilli G, Simeone S, Dell'Aversana Orabona G, Capuano S, Serao M, Rossi R, Orabona P, Balbi C
Arch Gynecol Obstet 2009 Nov;280(5):863-6. Epub 2009 Mar 18 doi: 10.1007/s00404-009-1022-3. PMID: 19294398

Prognosis

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
Embryonal Rhabdomyosarcoma of the Uterine Cervix: A Clinicopathologic Study of 94 Cases Emphasizing Issues in Differential Diagnosis Staging, and Prognostic Factors.
Devins KM, Young RH, Ghioni M, Burandt E, Bennett JA, Oliva E
Am J Surg Pathol 2022 Nov 1;46(11):1477-1489. Epub 2022 Aug 9 doi: 10.1097/PAS.0000000000001933. PMID: 35941719
Rhabdomyosarcoma of the Cervix in a Post-Menopausal Woman-An Unparalleled Phenomenon.
Pawlik J, Pawlik W, Branecka-Woźniak D, Kotrych K, Cymbaluk-Płoska A
Int J Environ Res Public Health 2021 Jul 24;18(15) doi: 10.3390/ijerph18157851. PMID: 34360144Free PMC Article
HPV infection and p16(INK4A) and TP53 expression in rare cancers of the uterine cervix.
Missaoui N, Mestiri S, Bdioui A, Zahmoul T, Hamchi H, Mokni M, Hmissa S
Pathol Res Pract 2018 Apr;214(4):498-506. Epub 2018 Mar 6 doi: 10.1016/j.prp.2018.03.004. PMID: 29572122
Adenosarcoma of the uterine cervix with heterologous elements: a case report and review of literature.
Bagga R, Keepanasseril A, Srinivasan R, Dey P, Gainder S, Saha SC, Dhaliwal LK, Patel F
Arch Gynecol Obstet 2010 Apr;281(4):669-75. Epub 2009 Aug 15 doi: 10.1007/s00404-009-1200-3. PMID: 19685064

Clinical prediction guides

Embryonal Rhabdomyosarcoma of the Uterine Cervix: A Clinicopathologic Study of 94 Cases Emphasizing Issues in Differential Diagnosis Staging, and Prognostic Factors.
Devins KM, Young RH, Ghioni M, Burandt E, Bennett JA, Oliva E
Am J Surg Pathol 2022 Nov 1;46(11):1477-1489. Epub 2022 Aug 9 doi: 10.1097/PAS.0000000000001933. PMID: 35941719
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

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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