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Vaginal rhabdomyosarcoma

MedGen UID:
925567
•Concept ID:
C4288035
•
Neoplastic Process
Synonyms: rhabdomyosarcoma (disease) of vagina; vagina rhabdomyosarcoma; vagina rhabdomyosarcoma (disease); Vaginal Rhabdomyosarcoma; vaginal rhabdomyosarcoma; vulvovaginal rhabdomyosarcoma; Vulvovaginal rhabdomyosarcoma
 
Monarch Initiative: MONDO:0016095
Orphanet: ORPHA206492

Definition

Vulvovaginal rhabdomyosarcoma is a rare vulvovaginal tumour, a highly malignant soft tissue sarcoma composed of cells with round to oval or spindle-shaped nuclei and eosinophilic cytoplasm that may show differentiation towards striated muscle cells. It usually affects children and presents with a vulvar or vaginal mass that may be polypoid or grape-like (embryonal subtype) and associated with bleeding and ulceration. [from ORDO]

Term Hierarchy

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

Professional guidelines

PubMed

ESTRO/ESGO/SIOPe Guidelines for the management of patients with vaginal cancer.
Nout RA, Calaminus G, Planchamp F, Chargari C, Lax S, Martelli H, McCluggage WG, Morice P, Pakiz M, Schmid MP, Stunt J, Timmermann B, Vokuhl C, Orbach D, Fotopoulou C
Int J Gynecol Cancer 2023 Aug 7;33(8):1185-1202. doi: 10.1136/ijgc-2023-004695. PMID: 37336757
ESTRO/ESGO/SIOPe guidelines for the management of patients with vaginal cancer.
Nout R, Calaminus G, Planchamp F, Chargari C, Lax SF, Martelli H, McCluggage WG, Morice P, Pakiz M, Schmid MP, Stunt J, Timmermann B, Vokuhl C, Orbach D, Fotopoulou C
Radiother Oncol 2023 Sep;186:109662. Epub 2023 May 25 doi: 10.1016/j.radonc.2023.109662. PMID: 37244358
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

Recent clinical studies

Etiology

ESTRO/ESGO/SIOPe Guidelines for the management of patients with vaginal cancer.
Nout RA, Calaminus G, Planchamp F, Chargari C, Lax S, Martelli H, McCluggage WG, Morice P, Pakiz M, Schmid MP, Stunt J, Timmermann B, Vokuhl C, Orbach D, Fotopoulou C
Int J Gynecol Cancer 2023 Aug 7;33(8):1185-1202. doi: 10.1136/ijgc-2023-004695. PMID: 37336757
Vaginal bleeding in children: A retrospective audit at a tertiary paediatric gynaecology service.
Drever N, Peek S, Moussaoui D, Dkeidek AI, Grover SR
J Paediatr Child Health 2023 Apr;59(4):653-659. Epub 2023 Feb 7 doi: 10.1111/jpc.16366. PMID: 36748561
Vaginal tumours in childhood: a descriptive analysis from a large paediatric medical centre.
Meng Z, Lin D, Liu C, Wang G, Sun N
Pediatr Surg Int 2022 Jun;38(6):927-934. Epub 2022 Mar 24 doi: 10.1007/s00383-022-05085-3. PMID: 35325284
Foley Catheter as a Vaginal Stent in a Toddler with Vaginal Rhabdomyosarcoma.
Fowler KG, Mohindra P, Kim A, Gomez-Lobo V
J Pediatr Adolesc Gynecol 2018 Jun;31(3):315-317. Epub 2017 Dec 9 doi: 10.1016/j.jpag.2017.12.003. PMID: 29233732
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

Diagnosis

ESTRO/ESGO/SIOPe Guidelines for the management of patients with vaginal cancer.
Nout RA, Calaminus G, Planchamp F, Chargari C, Lax S, Martelli H, McCluggage WG, Morice P, Pakiz M, Schmid MP, Stunt J, Timmermann B, Vokuhl C, Orbach D, Fotopoulou C
Int J Gynecol Cancer 2023 Aug 7;33(8):1185-1202. doi: 10.1136/ijgc-2023-004695. PMID: 37336757
Vaginal tumours in childhood: a descriptive analysis from a large paediatric medical centre.
Meng Z, Lin D, Liu C, Wang G, Sun N
Pediatr Surg Int 2022 Jun;38(6):927-934. Epub 2022 Mar 24 doi: 10.1007/s00383-022-05085-3. PMID: 35325284
Foley Catheter as a Vaginal Stent in a Toddler with Vaginal Rhabdomyosarcoma.
Fowler KG, Mohindra P, Kim A, Gomez-Lobo V
J Pediatr Adolesc Gynecol 2018 Jun;31(3):315-317. Epub 2017 Dec 9 doi: 10.1016/j.jpag.2017.12.003. PMID: 29233732
Literature Review of Benign Müllerian Papilloma Contrasted With Vaginal Rhabdomyosarcoma.
McQuillan SK, Grover SR, Pyman J, Jayasinghe YL
J Pediatr Adolesc Gynecol 2016 Aug;29(4):333-7. Epub 2015 Mar 11 doi: 10.1016/j.jpag.2015.02.114. PMID: 26948653
Vaginal rhabdomyosarcoma.
Pal DK, Jha S
Indian Pediatr 2009 May;46(5):431. PMID: 19478359

Therapy

Foley Catheter as a Vaginal Stent in a Toddler with Vaginal Rhabdomyosarcoma.
Fowler KG, Mohindra P, Kim A, Gomez-Lobo V
J Pediatr Adolesc Gynecol 2018 Jun;31(3):315-317. Epub 2017 Dec 9 doi: 10.1016/j.jpag.2017.12.003. PMID: 29233732
Vaginectomy and Buccal Mucosa Vaginoplasty as Local Therapy for Pediatric Vaginal Rhabdomyosarcoma.
Romao RL, Lorenzo AJ
Urology 2017 Apr;102:222-224. Epub 2017 Feb 21 doi: 10.1016/j.urology.2017.02.010. PMID: 28232175
Uterine/female genital sarcomas.
Hensley ML
Curr Treat Options Oncol 2000 Jun;1(2):161-8. doi: 10.1007/s11864-000-0061-6. PMID: 12057054
Progress in the surgical management of vaginal rhabdomyosarcoma: a 25-year review from the Intergroup Rhabdomyosarcoma Study Group.
Andrassy RJ, Wiener ES, Raney RB, Hays DM, Arndt CA, Lobe TE, Lawrence W, Anderson JR, Qualman SJ, Crist WM
J Pediatr Surg 1999 May;34(5):731-4; discussion 734-5. doi: 10.1016/s0022-3468(99)90365-2. PMID: 10359173
Long-term sequelae of conservative treatment by surgery, brachytherapy, and chemotherapy for vulval and vaginal rhabdomyosarcoma in children.
Flamant F, Gerbaulet A, Nihoul-Fekete C, Valteau-Couanet D, Chassagne D, Lemerle J
J Clin Oncol 1990 Nov;8(11):1847-53. doi: 10.1200/JCO.1990.8.11.1847. PMID: 2230871

Prognosis

Vaginal tumours in childhood: a descriptive analysis from a large paediatric medical centre.
Meng Z, Lin D, Liu C, Wang G, Sun N
Pediatr Surg Int 2022 Jun;38(6):927-934. Epub 2022 Mar 24 doi: 10.1007/s00383-022-05085-3. PMID: 35325284
Local control for vaginal botryoid rhabdomyosarcoma with pre-rectal transperineal surgical resection and autologous buccal graft vaginal replacement: A novel, minimally invasive, radiation-sparing approach.
Michlitsch JG, Romao RLP, Gleason JM, Braga LH, Allen L, Gupta A, Lorenzo AJ
J Pediatr Surg 2018 Jul;53(7):1374-1380. Epub 2017 Nov 21 doi: 10.1016/j.jpedsurg.2017.11.044. PMID: 29258699
Vulval and vaginal rhabdomyosarcoma in children: update and reappraisal of Institut Gustave Roussy brachytherapy experience.
Magné N, Oberlin O, Martelli H, Gerbaulet A, Chassagne D, Haie-Meder C
Int J Radiat Oncol Biol Phys 2008 Nov 1;72(3):878-83. Epub 2008 Mar 20 doi: 10.1016/j.ijrobp.2008.01.034. PMID: 18355981
Uterine/female genital sarcomas.
Hensley ML
Curr Treat Options Oncol 2000 Jun;1(2):161-8. doi: 10.1007/s11864-000-0061-6. PMID: 12057054
Long-term sequelae of conservative treatment by surgery, brachytherapy, and chemotherapy for vulval and vaginal rhabdomyosarcoma in children.
Flamant F, Gerbaulet A, Nihoul-Fekete C, Valteau-Couanet D, Chassagne D, Lemerle J
J Clin Oncol 1990 Nov;8(11):1847-53. doi: 10.1200/JCO.1990.8.11.1847. PMID: 2230871

Clinical prediction guides

Vaginal tumours in childhood: a descriptive analysis from a large paediatric medical centre.
Meng Z, Lin D, Liu C, Wang G, Sun N
Pediatr Surg Int 2022 Jun;38(6):927-934. Epub 2022 Mar 24 doi: 10.1007/s00383-022-05085-3. PMID: 35325284
Congenital urethral polyps in girls: as a differential diagnosis of interlabial masses.
Akbarzadeh A, Khorramirouz R, Saadat S, Hiradfar M, Kajbafzadeh AM
J Pediatr Adolesc Gynecol 2014 Dec;27(6):330-4. Epub 2014 Sep 23 doi: 10.1016/j.jpag.2014.01.001. PMID: 25256883

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