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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Aggressive Angiomyxoma Involving Penis and Urethra – A Case Report</title><author><name sortKey="Damodaran, Shivashankar" sort="Damodaran, Shivashankar" uniqKey="Damodaran S" first="Shivashankar" last="Damodaran">Shivashankar Damodaran</name><affiliation><nlm:aff id="aff1">Department of Urology, University of Wisconsin, Madison, WI 53705, USA</nlm:aff></affiliation><affiliation><nlm:aff id="aff2">Department of General Surgery, Shri Sathya Sai Medical College, Chennai, India</nlm:aff></affiliation></author><author><name sortKey="Gengan, Devakannan" sort="Gengan, Devakannan" uniqKey="Gengan D" first="Devakannan" last="Gengan">Devakannan Gengan</name><affiliation><nlm:aff id="aff2">Department of General Surgery, Shri Sathya Sai Medical College, Chennai, India</nlm:aff></affiliation></author><author><name sortKey="Walling, Sashi T" sort="Walling, Sashi T" uniqKey="Walling S" first="Sashi T." last="Walling">Sashi T. Walling</name><affiliation><nlm:aff id="aff2">Department of General Surgery, Shri Sathya Sai Medical College, Chennai, India</nlm:aff></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">28462172</idno><idno type="pmc">5408144</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5408144</idno><idno type="RBID">PMC:5408144</idno><idno type="doi">10.1016/j.eucr.2017.03.019</idno><date when="2017">2017</date><idno type="wicri:Area/Pmc/Corpus">002411</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">002411</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Aggressive Angiomyxoma Involving Penis and Urethra – A Case Report</title><author><name sortKey="Damodaran, Shivashankar" sort="Damodaran, Shivashankar" uniqKey="Damodaran S" first="Shivashankar" last="Damodaran">Shivashankar Damodaran</name><affiliation><nlm:aff id="aff1">Department of Urology, University of Wisconsin, Madison, WI 53705, USA</nlm:aff></affiliation><affiliation><nlm:aff id="aff2">Department of General Surgery, Shri Sathya Sai Medical College, Chennai, India</nlm:aff></affiliation></author><author><name sortKey="Gengan, Devakannan" sort="Gengan, Devakannan" uniqKey="Gengan D" first="Devakannan" last="Gengan">Devakannan Gengan</name><affiliation><nlm:aff id="aff2">Department of General Surgery, Shri Sathya Sai Medical College, Chennai, India</nlm:aff></affiliation></author><author><name sortKey="Walling, Sashi T" sort="Walling, Sashi T" uniqKey="Walling S" first="Sashi T." last="Walling">Sashi T. Walling</name><affiliation><nlm:aff id="aff2">Department of General Surgery, Shri Sathya Sai Medical College, Chennai, India</nlm:aff></affiliation></author></analytic><series><title level="j">Urology Case Reports</title><idno type="eISSN">2214-4420</idno><imprint><date when="2017">2017</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Aggressive angiomyxoma is a rare benign mesenchymal stromal tumour, characterized by locally infiltrative nature and a tendency for recurrence. Only a few cases of penile involvement have been reported in the literature so far. We report a case of aggressive penile angiomyxoma in a sixty-two-year-old obese, diabetic male patient. He presented with obstructive lower urinary tract symptoms (LUTS) and diffuse enlargement of the penis and scrotum. He was managed with excision, reduction scrotoplasty, internal urethrotomy, followed by Leuprolide therapy for prevention of recurrence. He is on follow up for 20 months without recurrence and obstructive symptoms.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Rocco, Francesco" uniqKey="Rocco F">Francesco Rocco</name></author><author><name sortKey="Cozzi, Gabriele" uniqKey="Cozzi G">Gabriele Cozzi</name></author><author><name sortKey="Spinelli, Matteo Giulio" uniqKey="Spinelli M">Matteo Giulio Spinelli</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Chihara, Y" uniqKey="Chihara Y">Y. Chihara</name></author><author><name sortKey="Fujimoto, K" uniqKey="Fujimoto K">K. Fujimoto</name></author><author><name sortKey="Takada, S" uniqKey="Takada S">S. Takada</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Morag, R" uniqKey="Morag R">R. Morag</name></author><author><name sortKey="Fridman, E" uniqKey="Fridman E">E. Fridman</name></author><author><name sortKey="Mor, Y" uniqKey="Mor Y">Y. Mor</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Xambre, L" uniqKey="Xambre L">L. Xambre</name></author><author><name sortKey="Cerqueira, M" uniqKey="Cerqueira M">M. Cerqueira</name></author><author><name sortKey="Guimaraes, I" uniqKey="Guimaraes I">I. Guimaraes</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Wang, Yi Chen" uniqKey="Wang Y">Yi-Chen Wang</name></author><author><name sortKey="Li, Xiao Ming" uniqKey="Li X">Xiao-Ming Li</name></author><author><name sortKey="Zhong, Gan Ping" uniqKey="Zhong G">Gan-Ping Zhong</name></author></analytic></biblStruct></listBibl></div1></back></TEI><pmc article-type="case-report"><pmc-dir>properties open_access</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Urol Case Rep</journal-id><journal-id journal-id-type="iso-abbrev">Urol Case Rep</journal-id><journal-title-group><journal-title>Urology Case Reports</journal-title></journal-title-group><issn pub-type="epub">2214-4420</issn><publisher><publisher-name>Elsevier</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">28462172</article-id><article-id pub-id-type="pmc">5408144</article-id><article-id pub-id-type="publisher-id">S2214-4420(17)30037-2</article-id><article-id pub-id-type="doi">10.1016/j.eucr.2017.03.019</article-id><article-categories><subj-group subj-group-type="heading"><subject>Oncology</subject></subj-group></article-categories><title-group><article-title>Aggressive Angiomyxoma Involving Penis and Urethra – A Case Report</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Damodaran</surname><given-names>Shivashankar</given-names></name><email>sdamodaran@wisc.edu</email><xref rid="aff1" ref-type="aff">a</xref><xref rid="aff2" ref-type="aff">b</xref><xref rid="cor1" ref-type="corresp">∗</xref></contrib><contrib contrib-type="author"><name><surname>Gengan</surname><given-names>Devakannan</given-names></name><xref rid="aff2" ref-type="aff">b</xref></contrib><contrib contrib-type="author"><name><surname>Walling</surname><given-names>Sashi T.</given-names></name><xref rid="aff2" ref-type="aff">b</xref></contrib></contrib-group><aff id="aff1"><label>a</label>Department of Urology, University of Wisconsin, Madison, WI 53705, USA</aff><aff id="aff2"><label>b</label>Department of General Surgery, Shri Sathya Sai Medical College, Chennai, India</aff><author-notes><corresp id="cor1"><label>∗</label>Corresponding author. Department of Urology, University of Wisconsin School of Medicine and Public Health, 7037 WIMR, 1111 Highland Avenue, Madison, WI 53705, USA. Fax: +1 608 262 6453.Department of UrologyUniversity of Wisconsin School of Medicine and Public Health7037 WIMR1111 Highland AvenueMadisonWI53705USA <email>sdamodaran@wisc.edu</email></corresp></author-notes><pub-date pub-type="pmc-release"><day>27</day><month>4</month><year>2017</year></pub-date><pmc-comment> PMC Release delay is 0 months and 0 days and was based on .</pmc-comment>
<pub-date pub-type="collection"><month>7</month><year>2017</year></pub-date><pub-date pub-type="epub"><day>27</day><month>4</month><year>2017</year></pub-date><volume>13</volume><fpage>110</fpage><lpage>112</lpage><history><date date-type="received"><day>8</day><month>2</month><year>2017</year></date><date date-type="accepted"><day>16</day><month>3</month><year>2017</year></date></history><permissions><copyright-statement>© 2017 Published by Elsevier Inc.</copyright-statement><copyright-year>2017</copyright-year><copyright-holder></copyright-holder><license license-type="CC BY-NC-ND" xlink:href="http://creativecommons.org/licenses/by-nc-nd/4.0/"><license-p>This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).</license-p></license></permissions><abstract id="abs0010a"><p>Aggressive angiomyxoma is a rare benign mesenchymal stromal tumour, characterized by locally infiltrative nature and a tendency for recurrence. Only a few cases of penile involvement have been reported in the literature so far. We report a case of aggressive penile angiomyxoma in a sixty-two-year-old obese, diabetic male patient. He presented with obstructive lower urinary tract symptoms (LUTS) and diffuse enlargement of the penis and scrotum. He was managed with excision, reduction scrotoplasty, internal urethrotomy, followed by Leuprolide therapy for prevention of recurrence. He is on follow up for 20 months without recurrence and obstructive symptoms.</p></abstract><kwd-group id="kwrds0010"><title>Keywords</title><kwd>Aggressive angiomyxoma</kwd><kwd>Penile mass</kwd><kwd>Hormone therapy</kwd></kwd-group></article-meta></front><body><sec id="sec1"><title>Introduction</title><p>Aggressive angiomyxoma is a rare mesenchymal stromal tumor first described by Steeper and Rosai in 1983, as a benign soft tissue tumor occurring in female pelvis and perineum. This tumor had a prominent angiomatous component, characterized by large thick walled vessels without arborization. Later reports attribute this tumor to adult females exclusively, occurring in pelvis, perineum, vulva and buttock.<xref rid="bib1" ref-type="bibr"><sup>1</sup></xref> Aggressive angiomyxoma occurs rarely in men and only a very few cases involving the penis and urethra have been reported in the literature so far. We report a case of a 62-year-old obese and diabetic male, who presented with diffuse enlargement of the penis and scrotum and obstructive LUTS.</p></sec><sec id="sec2"><title>Case history</title><p>A 62-year-old obese man (92 kg – BMI 33) presented with an enlarging penile and scrotal mass and severe obstructive lower urinary tract symptoms (IPSS score 31) to our OPD. He was diabetic and had multiple comorbidities including coronary artery disease, hypertension, hyperlipidemia and a diabetic foot ulcer. On examination, his penis was grossly enlarged and deformed. The prepuce was part of the diffuse swelling and was adherent to the glans (<xref rid="fig1" ref-type="fig">Fig. 1</xref>). Since the meatus was inaccessible for cannulation, a retrograde urethrogram could not be done. The scrotum was also moderately enlarged and had a similar consistency as that of penis. The testes were barely palpable. Lower limb examination was done to rule out a concurrent filarial lymphedema, as the penile mass was suspicious for a filarial ram horn penis and since the patient came from an area endemic for filariasis. However, a filarial work up was negative.<fig id="fig1"><label>Figure 1</label><caption><p>Preoperative picture of aggressive AAM involving the penis and scrotum. The meatus was completely occluded and patient had severe obstructive LUTS.</p></caption><alt-text id="altt080">Figure 1</alt-text><graphic xlink:href="gr1"></graphic></fig></p><p>In view of the poor performance status of the patient and multiple comorbidities including a poorly controlled diabetes, it was decided to do excise the involved tissues for biopsy and do a primary closure. During surgery, resection of the prepuce was technically demanding, as the angiomyxoma had infiltrated the glanular tissues. Hence, a limited resection of preputial tissue was done and a ventral meatotomy was done. This was followed by a reduction scrotoplasty and primary closure (<xref rid="fig2" ref-type="fig">Fig. 2</xref>).<fig id="fig2"><label>Figure 2</label><caption><p>Postoperative picture after a limited resection, ventral meatotomy and scrotoplasty. Urethroscopy showing AAM tissue inside penile urethra.</p></caption><alt-text id="altt180">Figure 2</alt-text><graphic xlink:href="gr2"></graphic></fig></p><p>A diagnostic urethroscopy showed diffuse narrowing in the penile urethra, with segmental stricture in the mid penile urethra (<xref rid="fig2" ref-type="fig">Fig. 2</xref>). The narrowed portion was lined by white, vascular tissue which had a gelatin like consistency. We performed an internal urethrotomy, in view of his comorbidities. Post procedure, a 16 French silastic catheter was introduced into the bladder and was retained for 2 weeks. Histopathological examination of the penile and scrotum specimens confirmed the tumor to be an aggressive angiomyxoma (<xref rid="fig3" ref-type="fig">Fig. 3</xref>).<fig id="fig3"><label>Figure 3</label><caption><p>Histopathology showing characteristic thick walled blood vessels and dense, relatively acellular stroma.</p></caption><alt-text id="altt380">Figure 3</alt-text><graphic xlink:href="gr3"></graphic></fig></p><p>Due to the high likelihood of recurrence of aggressive angiomyxoma, the patient was started on LHRH agonist therapy (Leuprolide 3.75 mg IM once a month). The tumor had been shown to express androgen receptors and found to be susceptible to hormone therapy from previous studies.<xref rid="bib2" ref-type="bibr"><sup>2</sup></xref> The patient has opted out of a definitive surgical resection due to his comorbidities and is on regular follow up for the past 20 months (monthly physical and office based urethroscopy once every 3 months).</p></sec><sec id="sec3"><title>Discussion</title><p>Aggressive angiomyxoma is a rare benign myxomatous tumor of males. Previous reports of AAM in males, attribute scrotum (38%), spermatic cord (33%), perineal region (13%), and intrapelvic organs such as the bladder (8%) to be the most commonly involved.<xref rid="bib3" ref-type="bibr"><sup>3</sup></xref> Involvement of penis is very rare. To the best of our knowledge only two cases of penoscrotal involvement by AAM have been reported in the literature so far. Rocco et al reported a case of massive recurring angiomyxoma of the scrotum obscuring the penis, in an obese man, which was managed by scrotal resection, bilateral orchidopexy and transposition of the penis. The patient soon developed a recurrence that involved the external meatus.<xref rid="bib1" ref-type="bibr"><sup>1</sup></xref> This was the only case reported so far with urethral involvement.</p><p>Xambre et al<xref rid="bib4" ref-type="bibr"><sup>4</sup></xref> reported on a case of aggressive angiomyxoma of the penoscrotum in a young male which was treated with wide local excision and plastic surgical reconstruction. Both the above mentioned reports highlight the infiltrative nature and tendency for local recurrence. Wang et al<xref rid="bib5" ref-type="bibr"><sup>5</sup></xref> have reported a case of superficial angiomyxoma of the penis, which unlike AAM had well defined borders and was easily resectable. In our case, the infiltrative lesion could only be partially resected form glanular tissues and a neomeatus was fashioned after the partial resection and a ventral meatotomy.</p><p>AAM is microscopically a stroma rich tumor, with low cellularity. Prominent blood vessels with hyaline thick walls and the anomalous muscular artery are the characteristic features. High proliferating cell nuclear antigen (PCNA) immunoexpression, together with the lack of expression of the p21 protein are associated with a high number of recurrences.<xref rid="bib3" ref-type="bibr"><sup>3</sup></xref> The tumor has been shown to express varying proportion of androgen, estrogen and progesterone receptors, making it amenable to hormonal manipulation.<xref rid="bib2" ref-type="bibr"><sup>2</sup></xref></p><p>AAM should be distinguished from other benign tumors affecting the pelvis and the genital tract, such as intramuscular myxoma, myxoid neurofibroma, myxoid or spindle cell lipoma, superficial angiomyxoma, angiomyofibroblastoma and angiomyolipoma; in the differential diagnosis one should also consider some malignant tumors with myxoid stroma, such as myxoid liposarcoma, myxoid malignant fibrous histiocytoma, and embryonal rhabdomyosarcoma.</p><p>Surgery is the mainstay of treatment for AAM. Incomplete resection can lead to recurrence. Recurrence rates can vary between 36 and 72 percent.<xref rid="bib2" ref-type="bibr"><sup>2</sup></xref> In our patient, multiple comorbidities precluded a complete excision and we had to resort to partial excision, scrotoplasty, neomeatus reconstruction internal urethrotomy, followed by hormonal therapy. Regular follow up is essential for AAM after surgery, in view of its locally recurrent nature and the optimal duration has to be determined from larger prospective studies.</p></sec><sec id="sec4"><title>Conflicts of interest</title><p>None.</p></sec></body><back><ref-list id="cebib0010"><title>References</title><ref id="bib1"><label>1</label><element-citation publication-type="journal" id="sref1"><person-group person-group-type="author"><name><surname>Rocco</surname><given-names>Francesco</given-names></name><name><surname>Cozzi</surname><given-names>Gabriele</given-names></name><name><surname>Spinelli</surname><given-names>Matteo Giulio</given-names></name></person-group><article-title>Massive recurring angiomyxoma of the scrotum in a obese man</article-title><source>Rare Tumors</source><volume>3</volume><year>2011</year><fpage>e31</fpage><pub-id pub-id-type="pmid">22066038</pub-id></element-citation></ref><ref id="bib2"><label>2</label><element-citation publication-type="journal" id="sref2"><person-group person-group-type="author"><name><surname>Chihara</surname><given-names>Y.</given-names></name><name><surname>Fujimoto</surname><given-names>K.</given-names></name><name><surname>Takada</surname><given-names>S.</given-names></name></person-group><article-title>Aggressive angiomyxoma in the scrotum expressing androgen and progesterone receptors</article-title><source>Int J Urol</source><volume>10</volume><year>2003</year><fpage>672</fpage><lpage>675</lpage><pub-id pub-id-type="pmid">14633074</pub-id></element-citation></ref><ref id="bib3"><label>3</label><element-citation publication-type="journal" id="sref3"><person-group person-group-type="author"><name><surname>Morag</surname><given-names>R.</given-names></name><name><surname>Fridman</surname><given-names>E.</given-names></name><name><surname>Mor</surname><given-names>Y.</given-names></name></person-group><article-title>Aggressive angiomyxoma of the scrotum mimicking huge hydrocele: case report and literature review</article-title><source>Case Rep Med</source><volume>2009</volume><year>2009</year><fpage>157624</fpage><pub-id pub-id-type="pmid">19707474</pub-id></element-citation></ref><ref id="bib4"><label>4</label><element-citation publication-type="journal" id="sref4"><person-group person-group-type="author"><name><surname>Xambre</surname><given-names>L.</given-names></name><name><surname>Cerqueira</surname><given-names>M.</given-names></name><name><surname>Guimaraes</surname><given-names>I.</given-names></name></person-group><article-title>Penoscrotal aggressive angiomyxoma</article-title><source>Actas Urol Esp</source><volume>33</volume><issue>3</issue><year>2009 Mar</year><fpage>321</fpage><lpage>326</lpage><comment>Spanish. PubMed PMID: 19537073</comment><pub-id pub-id-type="pmid">19537073</pub-id></element-citation></ref><ref id="bib5"><label>5</label><element-citation publication-type="journal" id="sref5"><person-group person-group-type="author"><name><surname>Wang</surname><given-names>Yi-Chen</given-names></name><name><surname>Li</surname><given-names>Xiao-Ming</given-names></name><name><surname>Zhong</surname><given-names>Gan-Ping</given-names></name></person-group><article-title>Superficial angiomyxoma of penis: a case report of a 6-year follow-up</article-title><source>Asian J Androl</source><year>1/3/2016</year></element-citation></ref></ref-list></back></pmc></record>Pour manipuler ce document sous Unix (Dilib)
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