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__NOTOC__
__NOTOC__
{{SI}}                                                                 
{{SI}}                                                                 
{{CMG}} {{AE}} {{MV}}
{{CMG}} {{AE}} {{RAK}} {{MV}}
   
   
{{SK}} Vaginal sarcoma; Embryonal vaginal rhabdomyosarcoma; Botryoid tumor  
{{SK}} Vaginal sarcoma; Embryonal vaginal rhabdomyosarcoma; Botryoid tumor  
   
   
==Overview==
==Overview==
'''Sarcoma botryoides''' is a rare subtype of embryonal [[rhabdomyosarcoma]]. The most commonly affected sites is the vagina, and it occurs more frequently among young girls. The most common clinical finding is [[vaginal bleeding]]. Sarcoma botryoides was first discovered by Alexander Brunschwig, an American physician in 1946. There is no classification system for sarcoma botryoides. Genes involved in the development of sarcoma botryoides, include [[K-ras gene]] and [[P53]] oncogene.<ref name="sarcoma">Sarcoma Botryoides. https://en.wikipedia.org/wiki/Sarcoma_botryoides Accessed on May 11, 2016 </ref> There are no established causes for sarcoma botryoides.<ref name="sarcoma">Sarcoma Botryoides. https://en.wikipedia.org/wiki/Sarcoma_botryoides Accessed on May 11, 2016 </ref> Sarcoma botryoides is more commonly observed among girls aged 3 to 8 years old.<ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref> The median age at diagnosis is 3 years (approximately 38.3 months).  If left untreated, patients with sarcoma botryoides may progress to develop direct extension to pelvic soft tissue structures. The mainstay of therapy for sarcoma botryoides, include: vincristine, cyclophosphamide, and actinomycin D. Surgery is the mainstay of therapy for sarcoma botryoides.<ref name="sarcoma">Sarcoma Botryoides. https://en.wikipedia.org/wiki/Sarcoma_botryoides Accessed on May 11, 2016 </ref><ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref>
'''Sarcoma botryoides''' is a rare subtype of [[Embryo|embryonal]] [[rhabdomyosarcoma]]. The botryoides subtype of [[Embryo|embryonal]] [[rhabdomyosarcoma]] accounts for about 10% of all [[rhabdomyosarcoma]] cases and arises from the sub-[[Mucous membrane|mucosal surface]] of [[Body orifice|body orifices]] such as the [[vagina]], [[bladder]] and [[cervix]]. The most common clinical finding is [[vaginal bleeding]]. Sarcoma botryoides was first described by Guersant in 1854. [[Genes]] involved in the development of sarcoma botryoides include [[KRAS|K-RAS gene]] and [[P53]] [[oncogene]]. It is more commonly observed among girls aged 3 to 8 years old. If left untreated, patients with sarcoma botryoides may progress to develop direct extension to [[pelvic]] [[soft tissue]] structures. The mainstay of therapy for sarcoma botryoides is [[surgery]] and [[Adjuvant treatment|adjuvant]] multi-agent [[chemotherapy]].


==Historical Perspective==
==Historical Perspective==
*Sarcoma botryoides was first discovered by Alexander Brunschwig, an American physician in 1946.<ref name="lxas">Alexander Brunschwing. Online Library Wiley. http://onlinelibrary.wiley.com/store/10.3322/canjclin.24.6.361/asset/361_ftp.pdf;jsessionid=F1E07B3CD830111D029BB265921D62D9.f02t02?v=1&t=io38ry91&s=7188f41feb9d1914c70a711514ec4efd6145aa84&systemMessage=Wiley+Online+Library+will+be+unavailable+on+Saturday+14th+May+11%3A00-14%3A00+BST+%2F+06%3A00-09%3A00+EDT+%2F+18%3A00-21%3A00+SGT+for+essential+maintenance.Apologies+for+the+inconvenience. Accessed on May 11, 2016 </ref>
*Sarcoma botryoides was first described by Guersant in 1854.<ref name="RaoAithala1983">{{cite journal|last1=Rao|first1=P. L. N. G.|last2=Aithala|first2=G.|last3=Warrier|first3=P. K. R.|title=Sarcoma botryoides of the vagina|journal=The Indian Journal of Pediatrics|volume=50|issue=4|year=1983|pages=445–448|issn=0019-5456|doi=10.1007/BF02753388}}</ref>
**The patient, a 13-month-old infant, had a [[polyp]] of the [[Vaginal|vagina]] that grew quite rapidly and resulted in death 2 months after it was first noted.
**Granicher is said to have been the first to observe sarcoma botryoides at [[birth]].
 
* Pfannenstiel in 1892 actually was the first to call [[attention]] to the "grapelike" appearance of the [[tumor]].<ref name="Hellriegel1985">{{cite journal|last1=Hellriegel|first1=W.|title=Primäre Sarkome der Weichteile|volume=19 / 6|year=1985|pages=25–109|issn=0085-1396|doi=10.1007/978-3-642-82387-9_2}}</ref>  
** The term “sarcoma botryoides” based on the tumor's gross [[appearance]], has persisted in literature.


==Classification==
==Classification==
*There is no classification system for sarcoma botryoides.
*There is no established system for the [[Staging (pathology)|staging]] of sarcoma botryoides.
*Sarcoma botryoides is a rare subtype of [[Embryo|embryonal]] [[rhabdomyosarcoma]].


==Pathophysiology==
==Pathophysiology==
*The pathogenesis of sarcoma botryoides is not fully understood.
*The [[pathogenesis]] of sarcoma botryoides is not fully understood.
*Genes involved in the development of sarcoma botryoides, include:
*[[Genes]] involved in the development of sarcoma botryoides, include:
:*[[KRAS|K-ras]] gene  
:*[[KRAS|K-ras]] gene  
:*[[P53|P53 oncogene]]
:*[[P53|P53 oncogene]]
*On gross pathology, characteristic findings of sarcoma botryoides, include:<ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref>
*On [[gross pathology]], characteristic findings of sarcoma botryoides, include:<ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref>
:*Grape-like morphology
:*Grape-like morphology
:*Fleshy nodular polypoid projections  
:*Fleshy nodular polypoid projections  
*On microscopic histopathological analysis, characteristic findings of sarcoma botryoides, include:<ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref>
*On [[microscopic]] [[histopathological]] analysis, characteristic findings of sarcoma botryoides include:<ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref><ref name="pmid8438559">{{cite journal| author=Palazzo JP, Gibas Z, Dunton CJ, Talerman A| title=Cytogenetic study of botryoid rhabdomyosarcoma of the uterine cervix. | journal=Virchows Arch A Pathol Anat Histopathol | year= 1993 | volume= 422 | issue= 1 | pages= 87-91 | pmid=8438559 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8438559  }}</ref>
:*Rhabdomyoblasts that may contain cross-striations
:*Rhabdomyoblasts
:*Tumor cells are crowded in a distinct layer beneath the vaginal epithelium (cambium layer)
:**May include cross-striations
:*Spindle-shaped tumor cells that are desmin positive
:*Myxoid [[stroma]]
:*Randomly arranged small cells
:*Cambium layer
:*Myxoid matrix
:**Formed by [[Tumor cell|tumor cells]] beneath the [[vaginal]] [[epithelium]]
:*Spindle-shaped [[Tumor cell|tumor cells]]
:*Randomly arranged small [[Cell (biology)|cells]]
:*Tadpole-like morphology
:*Tadpole-like morphology
:*Positive for Desmin, MyoD1
:*[[Desmin]] and MyoD1 postivie


==Causes==
==Causes==
Line 35: Line 43:
   
   
==Differentiating Sarcoma Botryoides from Other Diseases==
==Differentiating Sarcoma Botryoides from Other Diseases==
*Sarcoma botryoides must be differentiated from other tumors that cause vaginal bleeding, such as:
*Sarcoma botryoides must be differentiated from other [[tumors]] that cause [[vaginal bleeding]], such as:<ref name="pmid10408845">{{cite journal| author=Zanetta G, Rota SM, Lissoni A, Chiari S, Bratina G, Mangioni C| title=Conservative treatment followed by chemotherapy with doxorubicin and ifosfamide for cervical sarcoma botryoides in young females. | journal=Br J Cancer | year= 1999 | volume= 80 | issue= 3-4 | pages= 403-6 | pmid=10408845 | doi=10.1038/sj.bjc.6690370 | pmc=2362332 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10408845  }}</ref>
:*Squamous cell vaginal carcinoma
**Vaginal [[adenocarcinoma]]
:*Vaginal [[adenocarcinoma]]
***Distinguished [[Histology|histologically]] from sarcoma botryoides
:*Melanoma metastasis
***Shows [[fibrous]] stroma and leaf-like pattern of [[Gland|glands]].
 
**Squamous cell [[vaginal carcinoma]]
**[[Edema|Edematous]] [[cervical]] [[Mesoderm|mesodermal]] [[polyps]]
***Also known by pseudosarcoma botryoides
**[[Rhabdomyoma]]
**[[Melanoma]] [[metastasis]]
==Epidemiology and Demographics==
==Epidemiology and Demographics==


=== Incidence ===
=== Incidence ===
* Sarcoma botryoides is very rare.  
* Sarcoma botryoides is very rare.  
* The prevalence of sarcoma botryoides remains unknown.<ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref>
* The [[prevalence]] of sarcoma botryoides remains unknown.<ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref>
* Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and adolescence. It is a malignant tumour that arises from embryonic muscle cells and accounts for approximately 5% of all malignancies in children.<ref name="pmid17511807">{{cite journal| author=Maharaj NR, Nimako D, Hadley GP| title=Multimodal therapy for the initial management of genital embryonal rhabdomyosarcoma in childhood. | journal=Int J Gynecol Cancer | year= 2008 | volume= 18 | issue= 1 | pages= 190-2 | pmid=17511807 | doi=10.1111/j.1525-1438.2007.00985.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17511807  }}</ref>
===Age===
===Age===
*The median age at diagnosis is 3 years (approximately 38.3 months).
*The [[incidence]] of sarcoma botryoides decreases with age; the median age at diagnosis is 3 years.<ref name="pmid1090863">{{cite journal| author=Hilgers RD| title=Pelvic exenteration for vaginal embryonal rhabdomyosarcoma: a review. | journal=Obstet Gynecol | year= 1975 | volume= 45 | issue= 2 | pages= 175-80 | pmid=1090863 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1090863  }}</ref>
*Sarcoma botryoides is more commonly observed among patients aged 3 to 8 years old.
*Sarcoma botryoides is more commonly observed among patients aged 3 to 8 years old.
*Sarcoma botryoides is more commonly observed among young children.  
*Sarcoma botryoides is more commonly observed among young children.  


===Gender===
===Gender===
*Sarcoma botryoides affects females more frequently than males.   
*Sarcoma botryoides affects [[Female|females]] more frequently than [[Male|males]].   
===Race===
===Race===
*There is no racial predilection for sarcoma botryoides.
*There is no racial predilection to sarcoma botryoides.


==Risk Factors==
==Risk Factors==
*There are no risk factors associated with the development of sarcoma botryoides.<ref>Comprehensive treatment for sarcoma botryoides of uterine cervix. Chinese Journal of Cancer Research
*There are no [[risk factors]] associated with the development of sarcoma botryoides.<ref>Comprehensive treatment for sarcoma botryoides of uterine cervix. Chinese Journal of Cancer Research
June 1991, Volume 3, Issue 2, pp 68-70 &lt;nowiki&gt;</ref>&lt;/nowiki&gt;
June 1991, Volume 3, Issue 2, pp 68-70 &lt;nowiki&gt;</ref>


== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with sarcoma botryoides remain asymptomatic.  
*The majority of [[patients]] with sarcoma botryoides remain [[asymptomatic]].  
*Classic textbook presentation of ‘grape-like’ vaginal tissue loss.<ref name="pmid25519859">{{cite journal| author=van Sambeeck SJ, Mavinkurve-Groothuis AM, Flucke U, Dors N| title=Sarcoma botryoides in an infant. | journal=BMJ Case Rep | year= 2014 | volume= 2014 | issue=  | pages=  | pmid=25519859 | doi=10.1136/bcr-2013-202080 | pmc=4275735 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25519859  }}</ref>  
*If left untreated, patients with sarcoma botryoides may progress to develop direct extension to [[pelvic]] [[soft tissue]] structures.
*Early clinical features include vaginal discharge, and vaginal bleeding.<ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref>
*If a patient presents with the classical presentation of a grape-like [[Vaginal|vaginal tissue]], every [[Physician|doctor]] should be alarmed and commence rapid [[diagnosis]] followed by [[therapy]].<ref name="pmid25519859">{{cite journal| author=van Sambeeck SJ, Mavinkurve-Groothuis AM, Flucke U, Dors N| title=Sarcoma botryoides in an infant. | journal=BMJ Case Rep | year= 2014 | volume= 2014 | issue=  | pages=  | pmid=25519859 | doi=10.1136/bcr-2013-202080 | pmc=4275735 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25519859  }}</ref>  
*If left untreated, patients with sarcoma botryoides may progress to develop direct extension to pelvic soft tissue structures.
*Common complications of sarcoma botryoides, include:
*Common complications of sarcoma botryoides, include:
:*[[Bleeding]]
:*[[Bleeding]]
:*[[Infertility]]  
:*[[Infertility]]  
*Prognosis is generally poor, and the median survival rate of patients with sarcoma botryoides is approximately 10 to 35%.
:*Death
*Favorable [[prognostic]] parameters:<ref name="pmid195314572" />
**Disease without deep [[Myometrium|myometrial]] invasion
**Single [[polyp]]
**Embryonal [[histologic]] subtype
* Poor [[prognostic]] factors include:<ref name="pmid11505068">{{cite journal| author=Esnaola NF, Rubin BP, Baldini EH, Vasudevan N, Demetri GD, Fletcher CD et al.| title=Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma. | journal=Ann Surg | year= 2001 | volume= 234 | issue= 2 | pages= 215-23 | pmid=11505068 | doi= | pmc=1422009 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11505068  }}</ref>
** [[Metastatic]] disease at presentation
** Poor response to [[chemotherapy]]


== Diagnosis ==  
== Diagnosis ==  
=== Symptoms ===
=== Symptoms ===
*Sarcoma botryoides may be initially asymptomatic.
*Sarcoma botryoides may be initially [[asymptomatic]].
*Symptoms of sarcoma botryoides may include the following:
*A classic textbook presentation of sarcoma botryoides is a ‘grape-like’ [[Vaginal discharge|vaginal tissue loss]].<ref name="pmid25519859" />
*Symptoms of sarcoma botryoides may include the following:<ref name="sadma" />
:*[[Vaginal bleeding]]
:*[[Vaginal bleeding]]
:*[[Vaginal discharge]]
:**Watery
:**Blood-tinged
:**Malodorous
:*[[Irritability]]  
:*[[Irritability]]  
:*Watery, blood-tinged, or malodorous vaginal discharge


=== Physical Examination ===
=== Physical Examination ===
*Physical examination showed no abnormalities, but vaginal examination showed abnormal vaginal tissue protruding through the vagina introitus.<ref name="pmid25519859" />
*[[Physical examination]] showed no abnormalities, but [[vaginal]] examination showed abnormal [[vaginal]] tissue protruding through the vagina introitus.<ref name="pmid25519859" />
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with sarcoma botryoides.  
*There are no specific [[laboratory]] findings associated with sarcoma botryoides.  
   
   
===Imaging Findings===  
===Imaging Findings===  
*There are no specific imaging findings associated with sarcoma botryoides.<ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref>
*There are no specific [[imaging]] findings associated with sarcoma botryoides.<ref name="sadma">Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016 </ref>
*MRI of the abdominal region shows a solid lesion arising from the vagina and is:<ref name="pmid25519859" />
*MRI of the [[abdominal]] region shows a solid [[lesion]] arising from the [[vagina]] and is:<ref name="pmid25519859" />
**non-homogeneous
**non-homogeneous
**Polycyclic
**[[Polycyclic]]
**Grape-like
**Grape-like


== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*The classical presentation should alarm every doctor since a rapid diagnosis followed by treatment is necessary to prevent death.<ref name="pmid25519859" />
*The most effective treatment for sarcoma botryoides has not been well established yet and is still subject to on going [[research]].
*The mainstay of therapy for sarcoma botryoides, include:
*Due to the lack of data on [[Surgery|surgical]] [[Intervention (counseling)|interventions]] and [[histopathological]] [[prognostic]] factors of sarcoma botryoides cases and the variation in management approaches, it is difficult to set a definitive treatment path.<ref name="pmid26357564" />
*From the available [[Literature review|literature]], it appears that limited surgery with [[Adjuvant chemotherapy|adjuvant multi-agent chemotherapy]] is feasible in the treatment of sarcoma bortyoides.<ref name="pmid26357564">{{cite journal| author=Neha B, Manjunath AP, Girija S, Pratap K| title=Botryoid Rhabdomyosarcoma of the Cervix: Case report with review of the literature. | journal=Sultan Qaboos Univ Med J | year= 2015 | volume= 15 | issue= 3 | pages= e433-7 | pmid=26357564 | doi=10.18295/squmj.2015.15.03.022 | pmc=4554283 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26357564  }}</ref>  
*The optimal [[chemotherapy]] for sarcoma botryoides, include:
:*[[Vincristine]]
:*[[Vincristine]]
:*[[Cyclophosphamide]]
:*[[Cyclophosphamide]]
Line 99: Line 122:


=== Surgery ===
=== Surgery ===
*Surgery is the mainstay of therapy for sarcoma botryoides.
*[[Surgery]] is the mainstay of therapy for sarcoma botryoides.
*Patients with favorable [[prognostic]] parameters can effectively be treated by [[surgery]].<ref name="pmid195314572">{{cite journal| author=Koukourakis GV, Kouloulias V, Zacharias G, Maravelis G, Papadimitriou C, Platoni K et al.| title=Embryonal rhabdomyosarcoma of the uterine cervix. | journal=Clin Transl Oncol | year= 2009 | volume= 11 | issue= 6 | pages= 399-402 | pmid=19531457 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19531457  }}</ref>
*Patients with unfavorable [[prognostic]] parameters seem to benefit from a multimodality approach including [[surgery]] plus [[Adjuvant chemotherapy|adjuvant chemothe]]<nowiki/>[[Adjuvant chemotherapy|rap]]<nowiki/>y and [[radiotherapy]].<ref name="pmid195314572" />


=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for sarcoma botryoides.
*There are no [[Primary prevention|primary preventive]] measures available for sarcoma botryoides.


==References==
==References==

Latest revision as of 01:06, 3 June 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Roukoz A. Karam, M.D.[2] Maria Fernanda Villarreal, M.D. [3]

Synonyms and keywords: Vaginal sarcoma; Embryonal vaginal rhabdomyosarcoma; Botryoid tumor

Overview

Sarcoma botryoides is a rare subtype of embryonal rhabdomyosarcoma. The botryoides subtype of embryonal rhabdomyosarcoma accounts for about 10% of all rhabdomyosarcoma cases and arises from the sub-mucosal surface of body orifices such as the vagina, bladder and cervix. The most common clinical finding is vaginal bleeding. Sarcoma botryoides was first described by Guersant in 1854. Genes involved in the development of sarcoma botryoides include K-RAS gene and P53 oncogene. It is more commonly observed among girls aged 3 to 8 years old. If left untreated, patients with sarcoma botryoides may progress to develop direct extension to pelvic soft tissue structures. The mainstay of therapy for sarcoma botryoides is surgery and adjuvant multi-agent chemotherapy.

Historical Perspective

  • Sarcoma botryoides was first described by Guersant in 1854.[1]
    • The patient, a 13-month-old infant, had a polyp of the vagina that grew quite rapidly and resulted in death 2 months after it was first noted.
    • Granicher is said to have been the first to observe sarcoma botryoides at birth.
  • Pfannenstiel in 1892 actually was the first to call attention to the "grapelike" appearance of the tumor.[2]
    • The term “sarcoma botryoides” based on the tumor's gross appearance, has persisted in literature.

Classification

Pathophysiology

  • The pathogenesis of sarcoma botryoides is not fully understood.
  • Genes involved in the development of sarcoma botryoides, include:
  • Grape-like morphology
  • Fleshy nodular polypoid projections

Causes

  • There are no established causes for sarcoma botryoides.[3]

Differentiating Sarcoma Botryoides from Other Diseases

Epidemiology and Demographics

Incidence

  • Sarcoma botryoides is very rare.
  • The prevalence of sarcoma botryoides remains unknown.[3]

Age

  • The incidence of sarcoma botryoides decreases with age; the median age at diagnosis is 3 years.[6]
  • Sarcoma botryoides is more commonly observed among patients aged 3 to 8 years old.
  • Sarcoma botryoides is more commonly observed among young children.

Gender

  • Sarcoma botryoides affects females more frequently than males.

Race

  • There is no racial predilection to sarcoma botryoides.

Risk Factors

  • There are no risk factors associated with the development of sarcoma botryoides.[7]

Natural History, Complications and Prognosis

  • The majority of patients with sarcoma botryoides remain asymptomatic.
  • If left untreated, patients with sarcoma botryoides may progress to develop direct extension to pelvic soft tissue structures.
  • If a patient presents with the classical presentation of a grape-like vaginal tissue, every doctor should be alarmed and commence rapid diagnosis followed by therapy.[8]
  • Common complications of sarcoma botryoides, include:

Diagnosis

Symptoms

  • Sarcoma botryoides may be initially asymptomatic.
  • A classic textbook presentation of sarcoma botryoides is a ‘grape-like’ vaginal tissue loss.[8]
  • Symptoms of sarcoma botryoides may include the following:[3]

Physical Examination

Laboratory Findings

  • There are no specific laboratory findings associated with sarcoma botryoides.

Imaging Findings

Treatment

Medical Therapy

Surgery

Prevention

References

  1. ↑ Rao, P. L. N. G.; Aithala, G.; Warrier, P. K. R. (1983). "Sarcoma botryoides of the vagina". The Indian Journal of Pediatrics. 50 (4): 445–448. doi:10.1007/BF02753388. ISSN 0019-5456.
  2. ↑ Hellriegel, W. (1985). "Primäre Sarkome der Weichteile". 19 / 6: 25–109. doi:10.1007/978-3-642-82387-9_2. ISSN 0085-1396.
  3. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016
  4. ↑ Palazzo JP, Gibas Z, Dunton CJ, Talerman A (1993). "Cytogenetic study of botryoid rhabdomyosarcoma of the uterine cervix". Virchows Arch A Pathol Anat Histopathol. 422 (1): 87–91. PMID 8438559.
  5. ↑ Zanetta G, Rota SM, Lissoni A, Chiari S, Bratina G, Mangioni C (1999). "Conservative treatment followed by chemotherapy with doxorubicin and ifosfamide for cervical sarcoma botryoides in young females". Br J Cancer. 80 (3–4): 403–6. doi:10.1038/sj.bjc.6690370. PMC 2362332. PMID 10408845.
  6. ↑ Hilgers RD (1975). "Pelvic exenteration for vaginal embryonal rhabdomyosarcoma: a review". Obstet Gynecol. 45 (2): 175–80. PMID 1090863.
  7. ↑ Comprehensive treatment for sarcoma botryoides of uterine cervix. Chinese Journal of Cancer Research June 1991, Volume 3, Issue 2, pp 68-70 <nowiki>
  8. ↑ 8.0 8.1 8.2 8.3 van Sambeeck SJ, Mavinkurve-Groothuis AM, Flucke U, Dors N (2014). "Sarcoma botryoides in an infant". BMJ Case Rep. 2014. doi:10.1136/bcr-2013-202080. PMC 4275735. PMID 25519859.
  9. ↑ 9.0 9.1 9.2 Koukourakis GV, Kouloulias V, Zacharias G, Maravelis G, Papadimitriou C, Platoni K; et al. (2009). "Embryonal rhabdomyosarcoma of the uterine cervix". Clin Transl Oncol. 11 (6): 399–402. PMID 19531457.
  10. ↑ Esnaola NF, Rubin BP, Baldini EH, Vasudevan N, Demetri GD, Fletcher CD; et al. (2001). "Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma". Ann Surg. 234 (2): 215–23. PMC 1422009. PMID 11505068.
  11. ↑ 11.0 11.1 Neha B, Manjunath AP, Girija S, Pratap K (2015). "Botryoid Rhabdomyosarcoma of the Cervix: Case report with review of the literature". Sultan Qaboos Univ Med J. 15 (3): e433–7. doi:10.18295/squmj.2015.15.03.022. PMC 4554283. PMID 26357564.
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