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{{Vulvar cancer}}
{{Vulvar cancer}}
==Overview==
==Overview==
Vulvar cancer accounts for about 5% of cancers of the female genital system in the United States. The vulva is the area immediately external to the vagina, including the mons pubis, [[labia]], [[clitoris]], [[bartholin gland]]<nowiki/>s, and perineum. The labia majora are the most common site of vulvar carcinoma involvement and account for about 50% of cases. The labia minora account for 15% to 20% of vulvar carcinoma cases. The [[clitoris]] and [[Bartholin gland]]<nowiki/>s are less frequently involved. Lesions are multifocal in about 5% of cases. About 90% of vulvar carcinomas are squamous cell cancers. The [[vulvar intraepithelial neoplasia]]<nowiki/>s (VIN), may be precursors to invasive squamous cell cancers.<ref>General Information About Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq Accessed on october 1, 2015</ref> Common risk factors in the development of vulvar cancer are [[human papilloma virus]], [[human immunodeficiency virus]], [[vulvar intraepithelial neoplasia]], and [[lichen sclerosus]].<ref>Risk factors for vulvar cancer. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/vulvar/risks/?region=ns Accessed on September 27, 2015</ref> Vulvar cancer may be classified according to histology into 3 groups: vulvar carcinoma, vulvar sarcoma, and vulvar malignant [[melanoma]].<ref>{{cite book | last = Hoffman | first = Barbara | title = Williams gynecology | publisher = McGraw-Hill Medical | location = New York | year = 2012 | isbn = 9780071716727 }}</ref> If left untreated, patients with vulvar cancer may progress to develop [[ulcer]] on vulva or wart-like patches on vulva, and chronic [[pruritus]] of the vulva. Common complications of vulvar cancer include [[fistula]] and [[metastasis]]. [[Prognosis]] is generally good, and the 5 year survival rate of patients with vulvar cancer is approximately 71.2%. Common [[physical examination]] findings of vulvar cancer include vulvar [[ulcer]]s, mass, and [[inguinal]] [[lymphadenopathy]]. Vulvar [[biopsy]] is the confirmatory test for the diagnosis of vulvar cancer or precancerous lesions.<ref name="xyz">Diagnosing vulvar cancer. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/vulvar/diagnosis/?region=ns Accessed on october 1, 2015</ref> The optimal therapy for vulvar cancer depends on the stage at diagnosis.<ref name="xyz">Stage 0 Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_70 Accessed on September 25, 2015</ref><ref name="abc">Stage I Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_76 Accessed on September 25, 2015</ref><ref name="zzz">Stage II Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_83 Accessed on September 25, 2015</ref><ref name="xxx">Stage III Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_70 Accessed on September 25, 2015</ref><ref name="yyy">Stage IV Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_96 Accessed on September 25, 2015</ref> The feasibility of [[surgery]] depends on the stage of vulvar cancer at diagnosis.<ref name="xyz">Stage 0 Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_70 Accessed on September 25, 2015</ref><ref name="xxx">Stage I Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_76 Accessed on September 25, 2015</ref><ref name="zzz">Stage II Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_83 Accessed on September 25, 2015</ref><ref name="yyy">Stage III Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_70 Accessed on September 25, 2015</ref><ref name="abc">Stage IV Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_96 Accessed on September 25, 2015</ref>
Vulvar cancer accounts for about 5% of cancers of the female genital system in the United States. The vulva is the area immediately external to the vagina, including the mons pubis, [[labia]], [[clitoris]], [[bartholin gland]]<nowiki/>s, and perineum. The labia majora are the most common site of vulvar carcinoma involvement and account for about 50% of cases. The labia minora account for 15% to 20% of vulvar carcinoma cases. The [[clitoris]] and [[Bartholin gland]]<nowiki/>s are less frequently involved. Lesions are multifocal in about 5% of cases. About 90% of vulvar carcinomas are squamous cell cancers. The [[vulvar intraepithelial neoplasia]]<nowiki/>s (VIN), may be precursors to invasive squamous cell cancers. Common risk factors in the development of vulvar cancer are [[human papilloma virus]], [[human immunodeficiency virus]], [[vulvar intraepithelial neoplasia]], and [[lichen sclerosus]].  Vulvar cancer may be classified according to histology into 3 groups: vulvar carcinoma, vulvar sarcoma, and vulvar malignant [[melanoma]]. If left untreated, patients with vulvar cancer may progress to develop [[ulcer]] on vulva or wart-like patches on vulva, and chronic [[pruritus]] of the vulva. Common complications of vulvar cancer include [[fistula]] and [[metastasis]]. [[Prognosis]] is generally good, and the 5 year survival rate of patients with vulvar cancer is approximately 71.2%. Common [[physical examination]] findings of vulvar cancer include vulvar [[ulcer]]s, mass, and [[inguinal]] [[lymphadenopathy]]. Vulvar [[biopsy]] is the confirmatory test for the diagnosis of vulvar cancer or precancerous lesions.The optimal therapy for vulvar cancer depends on the stage at diagnosis.The feasibility of [[surgery]] depends on the stage of vulvar cancer at diagnosis.
 
 
==Classification==
==Classification==
Vulvar cancer may be classified according to histology into 3 groups vulvar carcinoma, vulvar [[sarcoma]], and vulvar malignant [[melanoma]].<ref>{{cite book | last = Hoffman | first = Barbara | title = Williams gynecology | publisher = McGraw-Hill Medical | location = New York | year = 2012 | isbn = 9780071716727 }}</ref>
Vulvar cancer may be classified according to histology into 3 groups vulvar carcinoma, vulvar [[sarcoma]], and vulvar malignant [[melanoma]].
==Pathophysiology==
==Pathophysiology==
Development of vulvar cancer is the result of multiple [[genetic mutations]].  
Development of vulvar cancer is the result of multiple [[genetic mutations]].  
==Causes==
==Causes==
Vulvar cancer may be caused by either [[HPV]] infection, or vulvar dermatoses.<ref name="xyz">The Histopathology of Vulvar Neoplasia. Wilkinson, E, Glob. libr. women's med.http://www.glowm.com/section_view/heading/The%2520Histopathology%2520of%2520Vulvar%2520Neoplasia/item/256#13421  URL Accessed on September 30, 2015</ref>
Vulvar cancer may be caused by either [[HPV]] infection, or vulvar dermatoses.
==Differential Diagnosis==
==Differential Diagnosis==
Vulvar cancer must be differentiated from other neoplastic vulvar lesions, non [[neoplastic]] vulvar lesions, and infectious disease lesions of [[vulva]].
Vulvar cancer must be differentiated from other neoplastic vulvar lesions, non [[neoplastic]] vulvar lesions, and infectious disease lesions of [[vulva]].
==Epidemiology and Demographics==
==Epidemiology and Demographics==
In 2012, the [[incidence]] of  vulvar [[cancer]] was estimated to be 2.4 per 100,000 females in the United States. The [[incidence]] of vulvar [[cancer]] increases with age, the median age at [[diagnosis]] is 68 years.  Vulvar cancer is more prevalent in the caucasian race.<ref>SEER Stat Fact Sheets: Vulvar Cancer.  Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/statfacts/html/vulva.html Accessed on September 21, 2015</ref>
In 2012, the [[incidence]] of  vulvar [[cancer]] was estimated to be 2.4 per 100,000 females in the United States. The [[incidence]] of vulvar [[cancer]] increases with age, the median age at [[diagnosis]] is 68 years.  Vulvar cancer is more prevalent in the caucasian race.


==Risk factors==
==Risk factors==
Common risk factors in the development of vulvar cancer are [[human papilloma virus]], [[human immunodeficiency virus]], [[vulvar intraepithelial neoplasia]], and [[lichen sclerosus]].<ref>Risk factors for vulvar cancer. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/vulvar/risks/?region=ns Accessed on September 27, 2015</ref>
Common risk factors in the development of vulvar cancer are [[human papilloma virus]], [[human immunodeficiency virus]], [[vulvar intraepithelial neoplasia]], and [[lichen sclerosus]].  
==Screening==
==Screening==
[[Screening]] for vulvar cancer is not recommended.<ref>What Should I Know About Screening? Gynecologic Cancers. http://www.cdc.gov/cancer/vagvulv/basic_info/screening.htm  Accessed on September 30, 2015</ref>
[[Screening]] for vulvar cancer is not recommended.
==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==
If left untreated, patients with vulvar cancer may progress to develop [[ulcer]] on vulva or wart-like patches on vulva, and chronic [[pruritus]] of the vulva. Common [[complications]] of vulvar cancer include  fistula and [[metastasis]]. Prognosis is generally good and the 5 year survival rate of patients with vulvar cancer is approximately 71.2%.
If left untreated, patients with vulvar cancer may progress to develop [[ulcer]] on vulva or wart-like patches on vulva, and chronic [[pruritus]] of the vulva. Common [[complications]] of vulvar cancer include  fistula and [[metastasis]]. Prognosis is generally good and the 5 year survival rate of patients with vulvar cancer is approximately 71.2%.
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==Diagnosis==
==Diagnosis==
===Staging===
===Staging===
According to the FIGO [[cancer staging]] system, there are 4 stages of vulvar cancer.<ref name="figo">{{cite web | url = http://www.igcs.org/guidelines/guideline_staging-booklet.pdf| author = International Federation of Gynecologists and Obstetricians (FIGO)| title = Staging classification and clinical practice guidelines of gynaecologic cancers | year = 2000 |accessdate = 2006-10-13}}</ref>
According to the FIGO [[cancer staging]] system, there are 4 stages of vulvar cancer.
===History and Symptoms===
===History and Symptoms===
[[Symptoms]] of vulvar cancer include vulvar [[ulcer]]s, lumps, discoloration, and chronic [[pruritis]] of the vulva.<ref>Signs and symptoms of vulvar cancer. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/vulvar/signs-and-symptoms/?region=nAccessed on September 26, 2015</ref>
[[Symptoms]] of vulvar cancer include vulvar [[ulcer]]s, lumps, discoloration, and chronic [[pruritis]] of the vulva.
===Physical Examination===
===Physical Examination===
Common [[physical examination]] findings of vulvar cancer include vulvar [[ulcer]]s, mass, and [[inguinal]] [[lymphadenopathy]].
Common [[physical examination]] findings of vulvar cancer include vulvar [[ulcer]]s, mass, and [[inguinal]] [[lymphadenopathy]].
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[[Chest radiography]] (CXR) may be helpful in the diagnosis of [[pulmonary]] [[metastasis]] of vulvar cancer. The characteristic findings of [[pulmonary]] [[metastasis]] are peripheral, rounded nodules of variable size scattered throughout both [[lungs]].
[[Chest radiography]] (CXR) may be helpful in the diagnosis of [[pulmonary]] [[metastasis]] of vulvar cancer. The characteristic findings of [[pulmonary]] [[metastasis]] are peripheral, rounded nodules of variable size scattered throughout both [[lungs]].
===CT scan===
===CT scan===
A [[CT scan]] for vulvar cancer may be helpful in determination of [[tumor]] size, tumor shape, and to detect [[metastasis]] of vulvar cancer.<ref name="xyz">Vulvar Cancer. American Cancer Society. http://www.cancer.org/acs/groups/cid/documents/webcontent/003147-pdf.pdf on October 1, 2015</ref>
A [[CT scan]] for vulvar cancer may be helpful in determination of [[tumor]] size, tumor shape, and to detect [[metastasis]] of vulvar cancer.
===MRI===
===MRI===
MRI is useful in accurately assessing the size of vulval lesion and assessing [[groin]] [[lymph node]] [[metastasis]].<ref>Primary vulval cancer. Radiopaedia.org. http://radiopaedia.org/articles/primary-vulval-cancer Accessed on october 1, 2015</ref>
MRI is useful in accurately assessing the size of vulval lesion and assessing [[groin]] [[lymph node]] [[metastasis]].
===Other Diagnostic Studies===
===Other Diagnostic Studies===
Vulvar [[biopsy]] is the confirmatory test for the [[diagnosis]] of vulvar cancer or precancerous lesions.<ref name="xyz">Diagnosing vulvar cancer. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/vulvar/diagnosis/?region=ns Accessed on october 1, 2015</ref>
Vulvar [[biopsy]] is the confirmatory test for the [[diagnosis]] of vulvar cancer or precancerous lesions.
==Treatment==
==Treatment==
===Medical therapy===
===Medical therapy===
The optimal therapy for vulvar cancer depends on the stage at diagnosis.<ref name="xyz">Stage 0 Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_70 Accessed on September 25, 2015</ref><ref name="abc">Stage I Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_76 Accessed on September 25, 2015</ref><ref name="zzz">Stage II Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_83 Accessed on September 25, 2015</ref><ref name="xxx">Stage III Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_70 Accessed on September 25, 2015</ref><ref name="yyy">Stage IV Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_96 Accessed on September 25, 2015</ref>
The optimal therapy for vulvar cancer depends on the stage at diagnosis.
===Surgery===
===Surgery===
The feasibility of [[surgery]] depends on the stage of vulvar cancer at diagnosis.<ref name="xyz">Stage 0 Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_70 Accessed on September 25, 2015</ref><ref name="xxx">Stage I Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_76 Accessed on September 25, 2015</ref><ref name="zzz">Stage II Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_83 Accessed on September 25, 2015</ref><ref name="yyy">Stage III Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_70 Accessed on September 25, 2015</ref><ref name="abc">Stage IV Vulvar Cancer. National Cancer Institute. http://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq#section/_96 Accessed on September 25, 2015</ref>
The feasibility of [[surgery]] depends on the stage of vulvar cancer at diagnosis.
===Primary Prevention===
===Primary Prevention===
Prevention of vulvar cancer includes a comprehensive approach involving awareness, screening, and preventative vaccinations.
Prevention of vulvar cancer includes a comprehensive approach involving awareness, screening, and preventative vaccinations.

Latest revision as of 22:28, 24 May 2019


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Syed Musadiq Ali M.B.B.S.[2] Monalisa Dmello, M.B,B.S., M.D. [3]

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Overview

Vulvar cancer accounts for about 5% of cancers of the female genital system in the United States. The vulva is the area immediately external to the vagina, including the mons pubis, labia, clitoris, bartholin glands, and perineum. The labia majora are the most common site of vulvar carcinoma involvement and account for about 50% of cases. The labia minora account for 15% to 20% of vulvar carcinoma cases. The clitoris and Bartholin glands are less frequently involved. Lesions are multifocal in about 5% of cases. About 90% of vulvar carcinomas are squamous cell cancers. The vulvar intraepithelial neoplasias (VIN), may be precursors to invasive squamous cell cancers. Common risk factors in the development of vulvar cancer are human papilloma virus, human immunodeficiency virus, vulvar intraepithelial neoplasia, and lichen sclerosus. Vulvar cancer may be classified according to histology into 3 groups: vulvar carcinoma, vulvar sarcoma, and vulvar malignant melanoma. If left untreated, patients with vulvar cancer may progress to develop ulcer on vulva or wart-like patches on vulva, and chronic pruritus of the vulva. Common complications of vulvar cancer include fistula and metastasis. Prognosis is generally good, and the 5 year survival rate of patients with vulvar cancer is approximately 71.2%. Common physical examination findings of vulvar cancer include vulvar ulcers, mass, and inguinal lymphadenopathy. Vulvar biopsy is the confirmatory test for the diagnosis of vulvar cancer or precancerous lesions.The optimal therapy for vulvar cancer depends on the stage at diagnosis.The feasibility of surgery depends on the stage of vulvar cancer at diagnosis.


Classification

Vulvar cancer may be classified according to histology into 3 groups vulvar carcinoma, vulvar sarcoma, and vulvar malignant melanoma.

Pathophysiology

Development of vulvar cancer is the result of multiple genetic mutations.

Causes

Vulvar cancer may be caused by either HPV infection, or vulvar dermatoses.

Differential Diagnosis

Vulvar cancer must be differentiated from other neoplastic vulvar lesions, non neoplastic vulvar lesions, and infectious disease lesions of vulva.

Epidemiology and Demographics

In 2012, the incidence of vulvar cancer was estimated to be 2.4 per 100,000 females in the United States. The incidence of vulvar cancer increases with age, the median age at diagnosis is 68 years. Vulvar cancer is more prevalent in the caucasian race.

Risk factors

Common risk factors in the development of vulvar cancer are human papilloma virus, human immunodeficiency virus, vulvar intraepithelial neoplasia, and lichen sclerosus.

Screening

Screening for vulvar cancer is not recommended.

Natural History, Complications and Prognosis

If left untreated, patients with vulvar cancer may progress to develop ulcer on vulva or wart-like patches on vulva, and chronic pruritus of the vulva. Common complications of vulvar cancer include fistula and metastasis. Prognosis is generally good and the 5 year survival rate of patients with vulvar cancer is approximately 71.2%.

Diagnosis

Staging

According to the FIGO cancer staging system, there are 4 stages of vulvar cancer.

History and Symptoms

Symptoms of vulvar cancer include vulvar ulcers, lumps, discoloration, and chronic pruritis of the vulva.

Physical Examination

Common physical examination findings of vulvar cancer include vulvar ulcers, mass, and inguinal lymphadenopathy.

Chest X-ray

Chest radiography (CXR) may be helpful in the diagnosis of pulmonary metastasis of vulvar cancer. The characteristic findings of pulmonary metastasis are peripheral, rounded nodules of variable size scattered throughout both lungs.

CT scan

A CT scan for vulvar cancer may be helpful in determination of tumor size, tumor shape, and to detect metastasis of vulvar cancer.

MRI

MRI is useful in accurately assessing the size of vulval lesion and assessing groin lymph node metastasis.

Other Diagnostic Studies

Vulvar biopsy is the confirmatory test for the diagnosis of vulvar cancer or precancerous lesions.

Treatment

Medical therapy

The optimal therapy for vulvar cancer depends on the stage at diagnosis.

Surgery

The feasibility of surgery depends on the stage of vulvar cancer at diagnosis.

Primary Prevention

Prevention of vulvar cancer includes a comprehensive approach involving awareness, screening, and preventative vaccinations.

References