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Fibroepithelial polyp of the vulva accompanied by lymphangioma circumscriptum

Identifieur interne : 002357 ( Pmc/Corpus ); précédent : 002356; suivant : 002358

Fibroepithelial polyp of the vulva accompanied by lymphangioma circumscriptum

Auteurs : Min Hee Lee ; Ji Young Hwang ; Jin Hee Lee ; Dong Hoon Kim ; Seung Hun Song

Source :

RBID : PMC:5547092

Abstract

Fibroepithelial polyp (FEP) and lymphangioma circumscriptum (LC) of the vulva are rare diseases that occur generally in young to middle-aged women. The pathogenesis of FEP is not clearly understood. Several previous cases have suggested that a hormonal influence may be a predisposing condition for developing FEP with or without chronic inflammation. A 68-year-old postmenopausal woman presented with a history of multiple raised lesions on the vulva with a 1-year duration. Additionally, a pedunculated mass had appeared 6 months earlier in the left lower labia minora. The patient underwent simple excision by electrocautery. The final pathological diagnosis was FEP with LC. In this case, the development of FEP was associated only with chronic inflammation induced by LC. Therefore, the case supports the hypothesis that a chronic inflammatory process may be related to the development of FEP even without hormonal influence.


Url:
DOI: 10.5468/ogs.2017.60.4.401
PubMed: 28791276
PubMed Central: 5547092

Links to Exploration step

PMC:5547092

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<p>Fibroepithelial polyp (FEP) and lymphangioma circumscriptum (LC) of the vulva are rare diseases that occur generally in young to middle-aged women. The pathogenesis of FEP is not clearly understood. Several previous cases have suggested that a hormonal influence may be a predisposing condition for developing FEP with or without chronic inflammation. A 68-year-old postmenopausal woman presented with a history of multiple raised lesions on the vulva with a 1-year duration. Additionally, a pedunculated mass had appeared 6 months earlier in the left lower labia minora. The patient underwent simple excision by electrocautery. The final pathological diagnosis was FEP with LC. In this case, the development of FEP was associated only with chronic inflammation induced by LC. Therefore, the case supports the hypothesis that a chronic inflammatory process may be related to the development of FEP even without hormonal influence.</p>
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<journal-meta>
<journal-id journal-id-type="nlm-ta">Obstet Gynecol Sci</journal-id>
<journal-id journal-id-type="iso-abbrev">Obstet Gynecol Sci</journal-id>
<journal-id journal-id-type="publisher-id">OGS</journal-id>
<journal-title-group>
<journal-title>Obstetrics & Gynecology Science</journal-title>
</journal-title-group>
<issn pub-type="ppub">2287-8572</issn>
<issn pub-type="epub">2287-8580</issn>
<publisher>
<publisher-name>Korean Society of Obstetrics and Gynecology; Korean Society of Contraception and Reproductive Health; Korean Society of Gynecologic Endocrinology; Korean Society of Gynecologic Endoscopy and Minimal Invasive Surgery; Korean Society of Maternal Fetal Medicine; Korean Society of Ultrasound in Obstetrics and Gynecology; Korean Urogynecologic Society</publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="pmid">28791276</article-id>
<article-id pub-id-type="pmc">5547092</article-id>
<article-id pub-id-type="doi">10.5468/ogs.2017.60.4.401</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Case Report</subject>
<subj-group subj-group-type="subheading">
<subject>General Gynecology</subject>
</subj-group>
</subj-group>
</article-categories>
<title-group>
<article-title>Fibroepithelial polyp of the vulva accompanied by lymphangioma circumscriptum</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname>Lee</surname>
<given-names>Min Hee</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Hwang</surname>
<given-names>Ji Young</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Lee</surname>
<given-names>Jin Hee</given-names>
</name>
<xref ref-type="aff" rid="A2">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Kim</surname>
<given-names>Dong Hoon</given-names>
</name>
<xref ref-type="aff" rid="A3">3</xref>
</contrib>
<contrib contrib-type="author" corresp="yes">
<contrib-id contrib-id-type="orcid" authenticated="true">https://orcid.org/0000-0002-9192-8984</contrib-id>
<name>
<surname>Song</surname>
<given-names>Seung Hun</given-names>
</name>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
</contrib-group>
<aff id="A1">
<label>1</label>
Department of Obstetrics and Gynecology, Gumi CHA Hospital, CHA University, Gumi, Korea.</aff>
<aff id="A2">
<label>2</label>
Department of Radiology, Gumi CHA Hospital, CHA University, Gumi, Korea.</aff>
<aff id="A3">
<label>3</label>
Department of Pathology, Gumi CHA Hospital, CHA University, Gumi, Korea.</aff>
<aff id="A4">
<label>4</label>
Department of Obstetrics and Gynecology, CHA Bundang Medical Center, CHA University, Seongnam, Korea.</aff>
<author-notes>
<corresp>Corresponding author: Seung Hun Song. Department of Obstetrics and Gynecology, CHA Bundang Medical Center, CHA University, 59 Yatap-ro, Bundang-gu, Seongnam 13496, Korea. Tel: +82-31-780-6191, Fax: +82-82-31-780-5600,
<email>shsong8@gmail.com</email>
</corresp>
</author-notes>
<pub-date pub-type="ppub">
<month>7</month>
<year>2017</year>
</pub-date>
<pub-date pub-type="epub">
<day>14</day>
<month>7</month>
<year>2017</year>
</pub-date>
<volume>60</volume>
<issue>4</issue>
<fpage>401</fpage>
<lpage>404</lpage>
<history>
<date date-type="received">
<day>06</day>
<month>12</month>
<year>2016</year>
</date>
<date date-type="rev-recd">
<day>22</day>
<month>2</month>
<year>2017</year>
</date>
<date date-type="accepted">
<day>27</day>
<month>3</month>
<year>2017</year>
</date>
</history>
<permissions>
<copyright-statement>Copyright © 2017 Korean Society of Obstetrics and Gynecology</copyright-statement>
<copyright-year>2017</copyright-year>
<license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc/3.0/">
<license-p>Articles published in Obstet Gynecol Sci are open-access, distributed under the terms of the Creative Commons Attribution Non-Commercial License (
<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>
) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p>
</license>
</permissions>
<abstract>
<p>Fibroepithelial polyp (FEP) and lymphangioma circumscriptum (LC) of the vulva are rare diseases that occur generally in young to middle-aged women. The pathogenesis of FEP is not clearly understood. Several previous cases have suggested that a hormonal influence may be a predisposing condition for developing FEP with or without chronic inflammation. A 68-year-old postmenopausal woman presented with a history of multiple raised lesions on the vulva with a 1-year duration. Additionally, a pedunculated mass had appeared 6 months earlier in the left lower labia minora. The patient underwent simple excision by electrocautery. The final pathological diagnosis was FEP with LC. In this case, the development of FEP was associated only with chronic inflammation induced by LC. Therefore, the case supports the hypothesis that a chronic inflammatory process may be related to the development of FEP even without hormonal influence.</p>
</abstract>
<kwd-group>
<kwd>Lymphangioma</kwd>
<kwd>Neoplasms, fibroepithelial</kwd>
<kwd>Postmenopause</kwd>
<kwd>Vulva</kwd>
</kwd-group>
</article-meta>
</front>
<body>
<sec sec-type="intro">
<title>Introduction</title>
<p>Fibroepithelial polyp (FEP) of the vulva is a very rare and benign disorder that occurs generally in young to middle-aged women [
<xref rid="B1" ref-type="bibr">1</xref>
]. Vulvar FEP appears to have a hormonal association, as patients may be pregnant at diagnosis or have a history of hormonal therapy [
<xref rid="B2" ref-type="bibr">2</xref>
]. Lymphangioma circumscriptum (LC) of the vulva is a very rare and benign disorder of the lymphatics located in the deep dermal and subcutaneous tissues. Vulvar LC is characterized by dilation of superficial dermal lymphatic channels, resulting in localized papules and vesicles.</p>
<p>The first case of vulvar FEP was described by Ostor et al. [
<xref rid="B3" ref-type="bibr">3</xref>
] in 1988. Since then, two additional cases have been reported, associated with other diseases (congenital lymphedema [
<xref rid="B4" ref-type="bibr">4</xref>
] and psoriasis [
<xref rid="B5" ref-type="bibr">5</xref>
]). However, there has not been a reported FEP case associated with LC. We present the first report of a case of vulvar FEP in a postmenopausal woman that is uniquely associated with vulvar LC.</p>
</sec>
<sec>
<title>Case report</title>
<p>A 68-year-old postmenopausal woman presented with a history of multiple raised lesions over the vulva with a 1-year duration. Furthermore, a pedunculated solid mass appeared 6 months after the lesions in the left lower labia minora. The patient was asymptomatic initially, but later experienced malodorous discharge and pruritis. She had a clinical history of radiation therapy due to early stage of rectal cancer at 30 years ago and lymphadenopathy of the left inguinal region more than 5 years ago.</p>
<p>On physical examination, there were multiple frog spawnlike lesions measuring about 10 cm×5 cm on both sides of the vulva with a malodorous discharge. A large (5 cm×3.5 cm) pedunculated polyp was observed in the left lower labia minora. This pedunculated polyp had an ulcerated surface and was firm-to-hard in consistency. There were several grouped papules of similar morphology over the left upper thigh (
<xref ref-type="fig" rid="F1">Fig. 1A</xref>
). Laboratory evaluation including hematologic, urinary, and tumor markers were within normal limits. Physical examination and ultrasonography of pelvic organs revealed no abnormal findings. Subsequently, enhanced magnetic resonance imaging scans demonstrated a 3.8 cm×3.3 cm×2 cm, pedunculated, exophytic lesion with connection to the left vulva. The preoperative imaging diagnosis was a benign tumor, such as fibroma or leiomyoma. The patient underwent simple excision by electrocautery and a carbon dioxide (CO
<sub>2</sub>
) laser was used to vaporize the lesion superficially. The postoperative course was uneventful. Six months later, the patient visited the hospital for a postoperative check-up. A physical examination revealed the absence of any gynecological abnormality (
<xref ref-type="fig" rid="F1">Fig. 1B</xref>
).</p>
<p>The lesions were composed of large pedunculated nodules and cluster of small polyps. The large nodules were well covered by hyperplastic epidermis and cored with edematous fibrous tissue with some inflammatory cells (
<xref ref-type="fig" rid="F2">Fig. 2A</xref>
). The stromal cells were reactive for smooth muscle actin but not for S-100. The large nodules also exhibited focal dilated lymphatics near the papillary dermis. The small polyps exhibited typical LC in the papillary dermis, confirmed through immunostaining for lymphatic marker, D2-40 (
<xref ref-type="fig" rid="F2">Fig. 2B</xref>
). The final pathological diagnosis was FEP with LC and the lesions were considered to be acquired lesions.</p>
<p>The report complies with the current laws of our country, and informed consent was obtained from the patients' who participated. The patient's information was enrolled in a paper approved by the Gumi CHA hospital institutional review board.</p>
</sec>
<sec sec-type="discussion">
<title>Discussion</title>
<p>FEP is a rare, locally infiltrative but non-metastasizing tumor of pelvic soft tissue seen mostly in women of child-bearing age [
<xref rid="B1" ref-type="bibr">1</xref>
]. The most common clinical presentation of FEP is as a painless mass. Our patient had a 5-cm-sized pedunculated painless mass with a cluster of small polypoid LC lesions. Acquired LC of the vulva is also very rare [
<xref rid="B6" ref-type="bibr">6</xref>
]. The lesions typically appear approximately 7 to 15 years after lymph node dissection and therapy of the genitalia [
<xref rid="B7" ref-type="bibr">7</xref>
]. In the case, she had a history of low anterior resection for rectal cancer and underwent radiotherapy over 31 years ago. The most characteristic clinical presentation of vulvar LC is multiple small raised frog spawn-like lesions with watery discharge and sometimes coexistent lymphedema extending to the adjacent abdomen or thigh skin.</p>
<p>The normal treatment for FEP is surgical excision, but there is no standard management for vulvar LC. Consequently, excisional surgery and CO
<sub>2</sub>
laser are the most commonly used treatment modalities. The CO
<sub>2</sub>
laser therapy has been selected as an effective treatment for LC. This treatment is good for small, superficial lesions. In the present case, excision was performed by electrocautery while CO
<sub>2</sub>
laser therapy was utilized for superficial LC lesions.</p>
<p>The pathogenesis of FEP of the vulva is not clearly understood. Some authors suggest a reactive hyperplastic process involving the distinctive subepithelial mesenchyma of the lower female genital tract [
<xref rid="B8" ref-type="bibr">8</xref>
]. Several previous cases suggested a hormonal influence with or without chronic inflammation as a predisposing condition for developing FEP. One previous case suggested hormonal changes induced by pregnancy as a predisposing factor for the development of FEP [
<xref rid="B9" ref-type="bibr">9</xref>
]. In addition, two cases demonstrated that hormonal influence with chronic inflammation is a predisposing factor for FEP. The first of those cases was a 16-year-old girl who developed FEP in association with congenital lymphedema; and the second was a 35-year-old woman with psoriasis who developed a 15 cm FEP localized in the left labium [
<xref rid="B4" ref-type="bibr">4</xref>
<xref rid="B5" ref-type="bibr">5</xref>
]. Histologically, the stromal cells of FEP have estrogen and progesterone receptors. Thus, hormonal influence may be a prerequisite condition for the development of FEP, as shown through an analysis of previous cases, clinical features, and histological findings. However, in the present case, the development of FEP was not associated with hormonal change. Instead, a chronic inflammation process induced by LC may have been the only predisposing factor. The patient was a postmenopausal woman who had not received hormonal therapy and had developed the LC lesion first, followed 6 months later by the FEP.</p>
<p>To the best our knowledge, this is the first reported case in the literature of FEP with LC. Furthermore, chronic inflammation induced by LC was the predisposing condition for the development of FEP. Hence, this case could support the hypothesis that a chronic inflammatory process is related to the development of FEP even without hormonal influence.</p>
</sec>
</body>
<back>
<fn-group>
<fn fn-type="COI-statement">
<p>
<bold>Conflict of interest:</bold>
No potential conflict of interest relevant to this article was reported.</p>
</fn>
</fn-group>
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<floats-group>
<fig id="F1" orientation="portrait" position="float">
<label>Fig. 1</label>
<caption>
<title>Clinical appearance of the vulva. (A) Preoperation. Multiple frog spawn-like lesions and a large (5 cm×3.5 cm) pedunculated polyp were present. (B) Postoperation. The lesions were no longer present. M, fibroepithelial polyp.</title>
</caption>
<graphic xlink:href="ogs-60-401-g001"></graphic>
</fig>
<fig id="F2" orientation="portrait" position="float">
<label>Fig. 2</label>
<caption>
<title>Pathologic findings of lesions. (A) The large nodule revealed diffuse fibrous stroma and hyperplastic epidermis where few lymphatic vessels were dilated (trichrome stain, ×40). (B) The dilated lymphatic vessels were lined by endothelial cells positive for D2-40. Adjacent small blood vessels were unstained by D2-40 (immunostain for D2-40, ×200).</title>
</caption>
<graphic xlink:href="ogs-60-401-g002"></graphic>
</fig>
</floats-group>
</pmc>
</record>

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