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Vulvar cancer: epidemiology, clinical presentation, and management options

Identifieur interne : 000D21 ( Pmc/Checkpoint ); précédent : 000D20; suivant : 000D22

Vulvar cancer: epidemiology, clinical presentation, and management options

Auteurs : Ibrahim Alkatout [Allemagne] ; Melanie Schubert [Allemagne] ; Nele Garbrecht [Allemagne] ; Marion Tina Weigel [Allemagne] ; Walter Jonat [Allemagne] ; Christoph Mundhenke [Allemagne] ; Veronika Günther [Allemagne]

Source :

RBID : PMC:4374790

Abstract

Epidemiology

Vulvar cancer can be classified into two groups according to predisposing factors: the first type correlates with a HPV infection and occurs mostly in younger patients. The second group is not HPV associated and occurs often in elderly women without neoplastic epithelial disorders.

Histology

Squamous cell carcinoma (SCC) is the most common malignant tumor of the vulva (95%).

Clinical features

Pruritus is the most common and long-lasting reported symptom of vulvar cancer, followed by vulvar bleeding, discharge, dysuria, and pain.

Therapy

The gold standard for even a small invasive carcinoma of the vulva was historically radical vulvectomy with removal of the tumor with a wide margin followed by an en bloc resection of the inguinal and often the pelvic lymph nodes. Currently, a more individualized and less radical treatment is suggested: a radical wide local excision is possible in the case of localized lesions (T1). A sentinel lymph node (SLN) biopsy may be performed to reduce wound complications and lymphedema.

Prognosis

The survival of patients with vulvar cancer is good when convenient therapy is arranged quickly after initial diagnosis. Inguinal and/or femoral node involvement is the most significant prognostic factor for survival.


Url:
DOI: 10.2147/IJWH.S68979
PubMed: 25848321
PubMed Central: 4374790


Affiliations:


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PMC:4374790

Le document en format XML

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<p>Vulvar cancer can be classified into two groups according to predisposing factors: the first type correlates with a HPV infection and occurs mostly in younger patients. The second group is not HPV associated and occurs often in elderly women without neoplastic epithelial disorders.</p>
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<p>Pruritus is the most common and long-lasting reported symptom of vulvar cancer, followed by vulvar bleeding, discharge, dysuria, and pain.</p>
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<title>Therapy</title>
<p>The gold standard for even a small invasive carcinoma of the vulva was historically radical vulvectomy with removal of the tumor with a wide margin followed by an en bloc resection of the inguinal and often the pelvic lymph nodes. Currently, a more individualized and less radical treatment is suggested: a radical wide local excision is possible in the case of localized lesions (T1). A sentinel lymph node (SLN) biopsy may be performed to reduce wound complications and lymphedema.</p>
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<p>The survival of patients with vulvar cancer is good when convenient therapy is arranged quickly after initial diagnosis. Inguinal and/or femoral node involvement is the most significant prognostic factor for survival.</p>
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<pmc article-type="review-article">
<pmc-dir>properties open_access</pmc-dir>
<front>
<journal-meta>
<journal-id journal-id-type="nlm-ta">Int J Womens Health</journal-id>
<journal-id journal-id-type="iso-abbrev">Int J Womens Health</journal-id>
<journal-id journal-id-type="publisher-id">International Journal of Women’s Health</journal-id>
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<journal-title>International Journal of Women's Health</journal-title>
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<issn pub-type="epub">1179-1411</issn>
<publisher>
<publisher-name>Dove Medical Press</publisher-name>
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</journal-meta>
<article-meta>
<article-id pub-id-type="pmid">25848321</article-id>
<article-id pub-id-type="pmc">4374790</article-id>
<article-id pub-id-type="doi">10.2147/IJWH.S68979</article-id>
<article-id pub-id-type="publisher-id">ijwh-7-305</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Review</subject>
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<title-group>
<article-title>Vulvar cancer: epidemiology, clinical presentation, and management options</article-title>
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<given-names>Ibrahim</given-names>
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<given-names>Melanie</given-names>
</name>
<xref ref-type="aff" rid="af1-ijwh-7-305">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Garbrecht</surname>
<given-names>Nele</given-names>
</name>
<xref ref-type="aff" rid="af2-ijwh-7-305">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Weigel</surname>
<given-names>Marion Tina</given-names>
</name>
<xref ref-type="aff" rid="af1-ijwh-7-305">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Jonat</surname>
<given-names>Walter</given-names>
</name>
<xref ref-type="aff" rid="af1-ijwh-7-305">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Mundhenke</surname>
<given-names>Christoph</given-names>
</name>
<xref ref-type="aff" rid="af1-ijwh-7-305">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Günther</surname>
<given-names>Veronika</given-names>
</name>
<xref ref-type="aff" rid="af1-ijwh-7-305">1</xref>
</contrib>
</contrib-group>
<aff id="af1-ijwh-7-305">
<label>1</label>
Department of Gynecology and Obstetrics, University Hospitals Schleswig-Holstein, Campus Kiel, Kiel, Germany</aff>
<aff id="af2-ijwh-7-305">
<label>2</label>
Institute for Pathology, University Hospitals Schleswig-Holstein, Campus Kiel, Kiel, Germany</aff>
<author-notes>
<corresp id="c1-ijwh-7-305">Correspondence: Ibrahim Alkatout, Department of Gynecology and Obstetrics, University Hospitals Schleswig-Holstein, Campus Kiel, Arnold-Heller Str 3, House 24, 24105 Kiel, Germany, Tel +49 431 597 2100, Fax +49 431 597 2116, Email
<email>ibrahim.alkatout@uksh.de</email>
</corresp>
</author-notes>
<pub-date pub-type="collection">
<year>2015</year>
</pub-date>
<pub-date pub-type="epub">
<day>20</day>
<month>3</month>
<year>2015</year>
</pub-date>
<volume>7</volume>
<fpage>305</fpage>
<lpage>313</lpage>
<permissions>
<copyright-statement>© 2015 Alkatout et al. This work is published by Dove Medical Press Limited, and licensed under Creative Commons Attribution – Non Commercial (unported, v3.0) License</copyright-statement>
<copyright-year>2015</copyright-year>
<license>
<license-p>The full terms of the License are available at
<ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by-nc/3.0/">http://creativecommons.org/licenses/by-nc/3.0/</ext-link>
. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.</license-p>
</license>
</permissions>
<abstract>
<sec>
<title>Epidemiology</title>
<p>Vulvar cancer can be classified into two groups according to predisposing factors: the first type correlates with a HPV infection and occurs mostly in younger patients. The second group is not HPV associated and occurs often in elderly women without neoplastic epithelial disorders.</p>
</sec>
<sec>
<title>Histology</title>
<p>Squamous cell carcinoma (SCC) is the most common malignant tumor of the vulva (95%).</p>
</sec>
<sec>
<title>Clinical features</title>
<p>Pruritus is the most common and long-lasting reported symptom of vulvar cancer, followed by vulvar bleeding, discharge, dysuria, and pain.</p>
</sec>
<sec>
<title>Therapy</title>
<p>The gold standard for even a small invasive carcinoma of the vulva was historically radical vulvectomy with removal of the tumor with a wide margin followed by an en bloc resection of the inguinal and often the pelvic lymph nodes. Currently, a more individualized and less radical treatment is suggested: a radical wide local excision is possible in the case of localized lesions (T1). A sentinel lymph node (SLN) biopsy may be performed to reduce wound complications and lymphedema.</p>
</sec>
<sec>
<title>Prognosis</title>
<p>The survival of patients with vulvar cancer is good when convenient therapy is arranged quickly after initial diagnosis. Inguinal and/or femoral node involvement is the most significant prognostic factor for survival.</p>
</sec>
</abstract>
<kwd-group>
<title>Keywords</title>
<kwd>vulvar cancer</kwd>
<kwd>HPV infection</kwd>
<kwd>radical vulvectomy</kwd>
<kwd>groin dissection</kwd>
<kwd>sentinel lymph node biopsy</kwd>
<kwd>overall survival</kwd>
</kwd-group>
</article-meta>
</front>
<floats-group>
<fig id="f1-ijwh-7-305" position="float">
<label>Figure 1</label>
<caption>
<p>Lymphatic drainage of the vulva.</p>
</caption>
<graphic xlink:href="ijwh-7-305Fig1"></graphic>
</fig>
<fig id="f2-ijwh-7-305" position="float">
<label>Figure 2</label>
<caption>
<p>Keratinizing squamous cell carcinoma of the vulva (Hematoxylin and eosin stain, ×5).</p>
</caption>
<graphic xlink:href="ijwh-7-305Fig2"></graphic>
</fig>
<fig id="f3-ijwh-7-305" position="float">
<label>Figure 3</label>
<caption>
<p>Elastica van Giesson stained (5× magnification) pT1G2 vulvar carcinoma.</p>
</caption>
<graphic xlink:href="ijwh-7-305Fig3"></graphic>
</fig>
<fig id="f4-ijwh-7-305" position="float">
<label>Figure 4</label>
<caption>
<p>Transition from normal epithelia to squamous cell carcinoma of the vulva (Hematoxylin and eosin stain, ×5).</p>
</caption>
<graphic xlink:href="ijwh-7-305Fig4"></graphic>
</fig>
<fig id="f5-ijwh-7-305" position="float">
<label>Figure 5</label>
<caption>
<p>Standard of LNE in patients with vulvar cancer.</p>
<p>
<bold>Abbreviations:</bold>
LNE, lymphonodectomy; SLN, sentinel lymph node.</p>
</caption>
<graphic xlink:href="ijwh-7-305Fig5"></graphic>
</fig>
<table-wrap id="t1-ijwh-7-305" position="float">
<label>Table 1</label>
<caption>
<p>Staging vulvar cancer (TNM and International Federation of Gynecology and Obstetrics, FIGO)</p>
</caption>
<table frame="hsides" rules="groups">
<thead>
<tr>
<th colspan="4" valign="top" align="left" rowspan="1">Primary tumor (T)
<hr></hr>
</th>
</tr>
<tr>
<th valign="top" align="left" rowspan="1" colspan="1">TNM categories</th>
<th valign="top" align="left" rowspan="1" colspan="1">FIGO stages</th>
<th valign="top" align="left" rowspan="1" colspan="1">Definition</th>
<th valign="top" align="left" rowspan="1" colspan="1">Surgery</th>
</tr>
</thead>
<tbody>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">TX</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
<td valign="top" align="left" rowspan="1" colspan="1">Primary tumor cannot be assessed</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">T0</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
<td valign="top" align="left" rowspan="1" colspan="1">No evidence of primary tumor</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">Tis</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
<td valign="top" align="left" rowspan="1" colspan="1">Carcinoma in situ</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">T1a</td>
<td valign="top" align="left" rowspan="1" colspan="1">IA</td>
<td valign="top" align="left" rowspan="1" colspan="1">Lesions 2 cm or less in size, confined to the vulva or perineum and with stromal invasion 1.0 mm or less</td>
<td valign="top" align="left" rowspan="1" colspan="1">WLE, no LNE</td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">T1b</td>
<td valign="top" align="left" rowspan="1" colspan="1">IB</td>
<td valign="top" align="left" rowspan="1" colspan="1">Lesions more than 2 cm size or any size with stromal invasion more than 1.0 mm, confined to the vulva or perineum</td>
<td valign="top" align="left" rowspan="1" colspan="1">WLE, LNE ipsilateral</td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">T2</td>
<td valign="top" align="left" rowspan="1" colspan="1">II</td>
<td valign="top" align="left" rowspan="1" colspan="1">Tumor of any size with extension to adjacent perineal structures (lower/distal 1/3 urethra, lower/distal 1/3 vagina, anal involvement)</td>
<td valign="top" align="left" rowspan="1" colspan="1">Modified radical vulvectomy (hemivulvectomy, anterior or posterior vulvectomy), LNE bilateral</td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">T3</td>
<td valign="top" align="left" rowspan="1" colspan="1">IVA</td>
<td valign="top" align="left" rowspan="1" colspan="1">Tumor of any size with extension to any of the following: upper/proximal 2/3 urethra, upper/proximal 2/3 vagina, bladder mucosa, rectal mucosa or fixed to pelvic bone</td>
<td valign="top" align="left" rowspan="1" colspan="1">Neoadjuvant chemoradiation and selected surgery, no LNE</td>
</tr>
<tr>
<td colspan="4" valign="top" align="left" rowspan="1">
<bold>Regional lymph nodes (N)</bold>
</td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">NX</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
<td valign="top" align="left" rowspan="1" colspan="1">Regional lymph nodes cannot be assessed</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">N0</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
<td valign="top" align="left" rowspan="1" colspan="1">No regional lymph node metastasis</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">N1</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
<td valign="top" align="left" rowspan="1" colspan="1">One or two regional lymph nodes with the following features</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1"> N1a</td>
<td valign="top" align="left" rowspan="1" colspan="1">IIIA</td>
<td valign="top" align="left" rowspan="1" colspan="1">One or two node metastases, each 5 mm or less</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1"> N1b</td>
<td valign="top" align="left" rowspan="1" colspan="1">IIIA</td>
<td valign="top" align="left" rowspan="1" colspan="1">One lymph node metastasis 5 mm or greater</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">N2</td>
<td valign="top" align="left" rowspan="1" colspan="1">IIIB</td>
<td valign="top" align="left" rowspan="1" colspan="1">Regional lymph node metastasis with the following features</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1"> N2a</td>
<td valign="top" align="left" rowspan="1" colspan="1">IIIB</td>
<td valign="top" align="left" rowspan="1" colspan="1">Three or more lymph node metastases each less than 5 mm</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1"> N2b</td>
<td valign="top" align="left" rowspan="1" colspan="1">IIIB</td>
<td valign="top" align="left" rowspan="1" colspan="1">Two or more lymph node metastases 5 mm or greater</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1"> N2c</td>
<td valign="top" align="left" rowspan="1" colspan="1">IIIC</td>
<td valign="top" align="left" rowspan="1" colspan="1">Lymph node metastasis with extracapsular spread</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">N3</td>
<td valign="top" align="left" rowspan="1" colspan="1">IVA</td>
<td valign="top" align="left" rowspan="1" colspan="1">Fixed or ulcerated regional lymph node metastasis</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td colspan="4" valign="top" align="left" rowspan="1">
<bold>Distant metastasis (M)</bold>
</td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">M0</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
<td valign="top" align="left" rowspan="1" colspan="1">No distant metastasis</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">M1</td>
<td valign="top" align="left" rowspan="1" colspan="1">IVB</td>
<td valign="top" align="left" rowspan="1" colspan="1">Distant metastasis (including pelvic lymph node metastasis)</td>
<td valign="top" align="left" rowspan="1" colspan="1"></td>
</tr>
</tbody>
</table>
<table-wrap-foot>
<fn id="tfn1-ijwh-7-305">
<p>
<bold>Abbreviations:</bold>
WLE, wide local excision; LNE, lymphonodectomy; FIGO, International Federation of Gynecology and Obstetrics.</p>
</fn>
</table-wrap-foot>
</table-wrap>
<table-wrap id="t2-ijwh-7-305" position="float">
<label>Table 2</label>
<caption>
<p>Survival by FIGO stage for patients with vulvar cancer 1999–2001, FIGO statistics</p>
</caption>
<table frame="hsides" rules="groups">
<thead>
<tr>
<th rowspan="2" valign="top" align="left" colspan="1">FIGO stage</th>
<th rowspan="2" valign="top" align="left" colspan="1">Number of patients</th>
<th colspan="3" valign="top" align="left" rowspan="1">Overall survival
<hr></hr>
</th>
</tr>
<tr>
<th valign="top" align="left" rowspan="1" colspan="1">1 year</th>
<th valign="top" align="left" rowspan="1" colspan="1">2 years</th>
<th valign="top" align="left" rowspan="1" colspan="1">5 years</th>
</tr>
</thead>
<tbody>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">I</td>
<td valign="top" align="left" rowspan="1" colspan="1">286</td>
<td valign="top" align="left" rowspan="1" colspan="1">96.4</td>
<td valign="top" align="left" rowspan="1" colspan="1">90.4</td>
<td valign="top" align="left" rowspan="1" colspan="1">78.5</td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">II</td>
<td valign="top" align="left" rowspan="1" colspan="1">266</td>
<td valign="top" align="left" rowspan="1" colspan="1">87.6</td>
<td valign="top" align="left" rowspan="1" colspan="1">73.2</td>
<td valign="top" align="left" rowspan="1" colspan="1">58.8</td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">III</td>
<td valign="top" align="left" rowspan="1" colspan="1">216</td>
<td valign="top" align="left" rowspan="1" colspan="1">74.7</td>
<td valign="top" align="left" rowspan="1" colspan="1">53.8</td>
<td valign="top" align="left" rowspan="1" colspan="1">43.2</td>
</tr>
<tr>
<td valign="top" align="left" rowspan="1" colspan="1">IV</td>
<td valign="top" align="left" rowspan="1" colspan="1">71</td>
<td valign="top" align="left" rowspan="1" colspan="1">35.3</td>
<td valign="top" align="left" rowspan="1" colspan="1">16.9</td>
<td valign="top" align="left" rowspan="1" colspan="1">13.0</td>
</tr>
</tbody>
</table>
<table-wrap-foot>
<fn id="tfn2-ijwh-7-305">
<p>
<bold>Note:</bold>
Modified from International Journal of Gynecology & Obstetrics; 95 Suppl 1; Beller U, Quinn MA, Benedet JL, et al. Carcinoma of the Vulva. S7–27. Copyright © 2006, with permission from Elsevier.
<xref rid="b69-ijwh-7-305" ref-type="bibr">69</xref>
</p>
</fn>
<fn id="tfn3-ijwh-7-305">
<p>
<bold>Abbreviation:</bold>
FIGO, International Federation of Gynecology and Obstetrics.</p>
</fn>
</table-wrap-foot>
</table-wrap>
</floats-group>
</pmc>
<affiliations>
<list>
<country>
<li>Allemagne</li>
</country>
<region>
<li>Schleswig-Holstein</li>
</region>
<settlement>
<li>Kiel</li>
</settlement>
</list>
<tree>
<country name="Allemagne">
<region name="Schleswig-Holstein">
<name sortKey="Alkatout, Ibrahim" sort="Alkatout, Ibrahim" uniqKey="Alkatout I" first="Ibrahim" last="Alkatout">Ibrahim Alkatout</name>
</region>
<name sortKey="Garbrecht, Nele" sort="Garbrecht, Nele" uniqKey="Garbrecht N" first="Nele" last="Garbrecht">Nele Garbrecht</name>
<name sortKey="Gunther, Veronika" sort="Gunther, Veronika" uniqKey="Gunther V" first="Veronika" last="Günther">Veronika Günther</name>
<name sortKey="Jonat, Walter" sort="Jonat, Walter" uniqKey="Jonat W" first="Walter" last="Jonat">Walter Jonat</name>
<name sortKey="Mundhenke, Christoph" sort="Mundhenke, Christoph" uniqKey="Mundhenke C" first="Christoph" last="Mundhenke">Christoph Mundhenke</name>
<name sortKey="Schubert, Melanie" sort="Schubert, Melanie" uniqKey="Schubert M" first="Melanie" last="Schubert">Melanie Schubert</name>
<name sortKey="Weigel, Marion Tina" sort="Weigel, Marion Tina" uniqKey="Weigel M" first="Marion Tina" last="Weigel">Marion Tina Weigel</name>
</country>
</tree>
</affiliations>
</record>

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