Sarcoma botryoides: Difference between revisions
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==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. There are no established causes for sarcoma botryoides. Sarcoma botryoides is more commonly observed among girls aged 3 to 8 years old. 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. | '''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. Sarcoma botryoides is more commonly observed among girls aged 3 to 8 years old. 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> | ||
==Historical Perspective== | ==Historical Perspective== | ||
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==Pathophysiology== | ==Pathophysiology== | ||
*The pathogenesis of sarcoma botryoides is not fully understood. | *The pathogenesis of sarcoma botryoides is not fully understood.<ref name="sarcoma"> Sarcoma Botryoides. https://en.wikipedia.org/wiki/Sarcoma_botryoides Accessed on May 11, 2016 </ref> | ||
*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: | *On gross pathology, characteristic findings of sarcoma botryoides, include:<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> | ||
:*Grape-like morphology | :*Grape-like morphology | ||
:*Fleshy nodular polypoid projections | :*Fleshy nodular polypoid projections | ||
*On microscopic histopathological analysis, characteristic findings of sarcoma botryoides, include: | *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> | ||
:*Rhabdomyoblasts that may contain cross-striations | :*Rhabdomyoblasts that may contain cross-striations | ||
:*Tumor cells are crowded in a distinct layer beneath the vaginal epithelium (cambium layer) | :*Tumor cells are crowded in a distinct layer beneath the vaginal epithelium (cambium layer) | ||
| Line 32: | Line 32: | ||
==Causes== | ==Causes== | ||
* There are no established causes for sarcoma botryoides. | * There are no established causes for 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> | ||
==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="sarcoma"> Sarcoma Botryoides. https://en.wikipedia.org/wiki/Sarcoma_botryoides Accessed on May 11, 2016 </ref> | ||
:*Squamous cell vaginal carcinoma | :*Squamous cell vaginal carcinoma | ||
:*Vaginal [[adenocarcinoma]] | :*Vaginal [[adenocarcinoma]] | ||
| Line 42: | Line 42: | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
* Sarcoma botryoides is very rare. | * Sarcoma botryoides is very rare. | ||
* The prevalence of sarcoma botryoides remains unknown. | * 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> | ||
===Age=== | ===Age=== | ||
*The median age at diagnosis is 3 years (approximately 38.3 months). | *The median age at diagnosis is 3 years (approximately 38.3 months). | ||
*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.<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 young children. | *Sarcoma botryoides is more commonly observed among young children. | ||
| Line 58: | Line 58: | ||
== 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. | ||
*Early clinical features include vaginal discharge, and vaginal bleeding. | *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 left untreated, patients with sarcoma botryoides may progress to develop direct extension to pelvic soft tissue structures. | *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%. | *Prognosis is generally poor, and the median survival rate of patients with sarcoma botryoides is approximately 10 to 35%.<ref name="sarcoma"> Sarcoma Botryoides. https://en.wikipedia.org/wiki/Sarcoma_botryoides Accessed on May 11, 2016 </ref> | ||
== Diagnosis == | == Diagnosis == | ||
=== Symptoms === | === Symptoms === | ||
*Sarcoma botryoides may be initially asymptomatic. | *Sarcoma botryoides may be initially asymptomatic. | ||
*Symptoms of sarcoma botryoides may include the following: | *Symptoms of sarcoma botryoides may include the following:<ref name="sarcoma"> Sarcoma Botryoides. https://en.wikipedia.org/wiki/Sarcoma_botryoides Accessed on May 11, 2016 </ref> | ||
:*[[Vaginal bleeding]] | :*[[Vaginal bleeding]] | ||
:*[[Irritability]] | :*[[Irritability]] | ||
| Line 75: | Line 75: | ||
=== Physical Examination === | === Physical Examination === | ||
*Patients with sarcoma botryoides usually are well-appearing. | *Patients with sarcoma botryoides usually are well-appearing. | ||
*Physical examination may be remarkable for: | *Physical examination may be remarkable for:<ref name="sarcoma"> Sarcoma Botryoides. https://en.wikipedia.org/wiki/Sarcoma_botryoides Accessed on May 11, 2016 </ref> | ||
:*Soft nodules that fill and sometimes protrude from the vagina | :*Soft nodules that fill and sometimes protrude from the vagina | ||
:*Polypoid mass | :*Polypoid mass | ||
| Line 83: | Line 83: | ||
===Imaging Findings=== | ===Imaging Findings=== | ||
*There are no specific imaging findings associated with sarcoma botryoides | *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> | ||
== Treatment == | == Treatment == | ||
=== Medical Therapy === | === Medical Therapy === | ||
*The mainstay of therapy for sarcoma botryoides, include: | *The mainstay of therapy for sarcoma botryoides, include:<ref name="sarcoma"> Sarcoma Botryoides. https://en.wikipedia.org/wiki/Sarcoma_botryoides Accessed on May 11, 2016 </ref> | ||
:*[[Vincristine]] | :*[[Vincristine]] | ||
:*[[Cyclophosphamide]] | :*[[Cyclophosphamide]] | ||
Revision as of 19:08, 11 May 2016
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]
Synonyms and keywords: Synonym 1; Synonym 2; Synonym 3
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.[1] There are no established causes for sarcoma botryoides. Sarcoma botryoides is more commonly observed among girls aged 3 to 8 years old. 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.[1]
Historical Perspective
- Sarcoma botryoides was first discovered by Alexander Brunschwig, an American physician in 1946.
Classification
- There is no classification system for sarcoma botryoides.
Pathophysiology
- The pathogenesis of sarcoma botryoides is not fully understood.[1]
- Genes involved in the development of sarcoma botryoides, include:
- K-ras gene
- P53 oncogene
- Grape-like morphology
- Fleshy nodular polypoid projections
- On microscopic histopathological analysis, characteristic findings of sarcoma botryoides, include:[2]
- Rhabdomyoblasts that may contain cross-striations
- Tumor cells are crowded in a distinct layer beneath the vaginal epithelium (cambium layer)
- Spindle-shaped tumor cells that are desmin positive
- Randomly arranged small cells
- Myxoid matrix
- Tadpole-like morphology
- Positive for Desmin, MyoD1
Causes
- There are no established causes for sarcoma botryoides.[2]
Differentiating Sarcoma Botryoides from Other Diseases
- Sarcoma botryoides must be differentiated from other tumors that cause vaginal bleeding, such as:[1]
- Squamous cell vaginal carcinoma
- Vaginal adenocarcinoma
- Melanoma metastasis
Epidemiology and Demographics
- Sarcoma botryoides is very rare.
- The prevalence of sarcoma botryoides remains unknown.[2]
Age
- The median age at diagnosis is 3 years (approximately 38.3 months).
- Sarcoma botryoides is more commonly observed among patients aged 3 to 8 years old.[1]
- Sarcoma botryoides is more commonly observed among young children.
Gender
- Sarcoma botryoides affects females more frequently than males.
Race
- There is no racial predilection for sarcoma botryoides.
Risk Factors
- There are no risk factors associated with the development of sarcoma botryoides.
Natural History, Complications and Prognosis
- The majority of patients with sarcoma botryoides remain asymptomatic.
- Early clinical features include vaginal discharge, and vaginal bleeding.[2]
- If left untreated, patients with sarcoma botryoides may progress to develop direct extension to pelvic soft tissue structures.
- Common complications of sarcoma botryoides, include:
- Prognosis is generally poor, and the median survival rate of patients with sarcoma botryoides is approximately 10 to 35%.[1]
Diagnosis
Symptoms
- Sarcoma botryoides may be initially asymptomatic.
- Symptoms of sarcoma botryoides may include the following:[1]
- Vaginal bleeding
- Irritability
- Watery, blood-tinged, or malodorous vaginal discharge
Physical Examination
- Patients with sarcoma botryoides usually are well-appearing.
- Physical examination may be remarkable for:[1]
- Soft nodules that fill and sometimes protrude from the vagina
- Polypoid mass
Laboratory Findings
- There are no specific laboratory findings associated with sarcoma botryoides.
Imaging Findings
- There are no specific imaging findings associated with sarcoma botryoides.[2]
Treatment
Medical Therapy
- The mainstay of therapy for sarcoma botryoides, include:[1]
Surgery
- Surgery is the mainstay of therapy for sarcoma botryoides.
Prevention
- There are no primary preventive measures available for sarcoma botryoides.
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 Sarcoma Botryoides. https://en.wikipedia.org/wiki/Sarcoma_botryoides Accessed on May 11, 2016
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 Sarcoma Botryoides. Libre Pathology https://librepathology.org/w/index.php?title=Rhabdomyosarcoma&redirect=no#Subtypes_of_embryonal_RMS Accessed on May 11, 2016