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Long-term outcome of patients with perianal Paget's disease
Perianal Paget's disease (PPD) is a rare intraepithelial adenocarcinoma with a significant rate of recurrence after treatment and high risk of progression to an invasive cancer. Fourteen patients with a mean follow-up longer than 5 years were studied to determine the outcome after surgical trea...
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| Published in: | Annals of surgical oncology 1997-09, Vol.4 (6), p.475-480 |
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| container_end_page | 480 |
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| container_title | Annals of surgical oncology |
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| creator | Marchesa, P Fazio, V W Oliart, S Goldblum, J R Lavery, I C Milsom, J W |
| description | Perianal Paget's disease (PPD) is a rare intraepithelial adenocarcinoma with a significant rate of recurrence after treatment and high risk of progression to an invasive cancer.
Fourteen patients with a mean follow-up longer than 5 years were studied to determine the outcome after surgical treatment. The immunohistochemical accumulation of p53 protein also was assessed in tissue specimens to evaluate its prognostic role in patients with PPD.
Four patients were excluded because of progression to invasive malignancy at the time of diagnosis. Two patients underwent local excision (LE) with macroscopic clearance of the surgical margins; the remaining eight patients underwent wide local excision (WLE), i.e., > 1 cm microscopic clearance of the surgical margins. The actuarial 8-year recurrence rate for patients treated with LE and WLE was 100% and 50% (SE = 17.7), respectively. Progression to invasive carcinoma occurred after a median time of 56 months (range 23-72) in two patients treated with LE and in one of eight patients treated with WLE. All four patients with recurrence after WLE were successfully treated (no further recurrence) with a second WLE. Actuarial 8-year survival was 0% in the LE group and 40% (SE = 21.9) in the WLE group. There was no p53 protein accumulation in any of the ten patients with PPD.
Survival of patients with PPD treated by WLE was higher than that for those treated with LE. Thus, wide local excision is recommended over limited local excision as a preferred treatment for PPD. Follow-up longer than 5 years seems to be indicated because of the risk of late progression to invasive cancer. When PPD does recur, a second WLE may be curative. The absence of accumulated p53 protein suggests that this marker may not have a prognostic role in PPD. |
| doi_str_mv | 10.1007/BF02303671 |
| format | article |
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Fourteen patients with a mean follow-up longer than 5 years were studied to determine the outcome after surgical treatment. The immunohistochemical accumulation of p53 protein also was assessed in tissue specimens to evaluate its prognostic role in patients with PPD.
Four patients were excluded because of progression to invasive malignancy at the time of diagnosis. Two patients underwent local excision (LE) with macroscopic clearance of the surgical margins; the remaining eight patients underwent wide local excision (WLE), i.e., > 1 cm microscopic clearance of the surgical margins. The actuarial 8-year recurrence rate for patients treated with LE and WLE was 100% and 50% (SE = 17.7), respectively. Progression to invasive carcinoma occurred after a median time of 56 months (range 23-72) in two patients treated with LE and in one of eight patients treated with WLE. All four patients with recurrence after WLE were successfully treated (no further recurrence) with a second WLE. Actuarial 8-year survival was 0% in the LE group and 40% (SE = 21.9) in the WLE group. There was no p53 protein accumulation in any of the ten patients with PPD.
Survival of patients with PPD treated by WLE was higher than that for those treated with LE. Thus, wide local excision is recommended over limited local excision as a preferred treatment for PPD. Follow-up longer than 5 years seems to be indicated because of the risk of late progression to invasive cancer. When PPD does recur, a second WLE may be curative. The absence of accumulated p53 protein suggests that this marker may not have a prognostic role in PPD.</description><identifier>ISSN: 1068-9265</identifier><identifier>EISSN: 1534-4681</identifier><identifier>DOI: 10.1007/BF02303671</identifier><identifier>PMID: 9309336</identifier><language>eng</language><publisher>United States: Springer Nature B.V</publisher><subject>Adenocarcinoma ; Adult ; Aged ; Anal Gland Neoplasms - metabolism ; Anal Gland Neoplasms - mortality ; Anal Gland Neoplasms - surgery ; Animals ; Antibodies, Monoclonal ; Biomarkers, Tumor - biosynthesis ; Biomarkers, Tumor - immunology ; Disease Progression ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Invasiveness ; Male ; Malignancy ; Middle Aged ; Neoplasm Recurrence, Local ; p53 Protein ; Paget Disease, Extramammary - metabolism ; Paget Disease, Extramammary - mortality ; Paget Disease, Extramammary - surgery ; Paget's disease ; Pagets disease ; Prognosis ; Proteins ; Survival ; Survival Rate ; Time Factors ; Treatment Outcome ; Tumor Suppressor Protein p53 - biosynthesis ; Tumor Suppressor Protein p53 - immunology</subject><ispartof>Annals of surgical oncology, 1997-09, Vol.4 (6), p.475-480</ispartof><rights>The Society of Surgical Oncology, Inc 1997.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c351t-ef65ad4afe3975f8d4101ada1484ea39d054d10fb4d9f140696a59c390d50bba3</citedby><cites>FETCH-LOGICAL-c351t-ef65ad4afe3975f8d4101ada1484ea39d054d10fb4d9f140696a59c390d50bba3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,786,790,27957,27958</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/9309336$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Marchesa, P</creatorcontrib><creatorcontrib>Fazio, V W</creatorcontrib><creatorcontrib>Oliart, S</creatorcontrib><creatorcontrib>Goldblum, J R</creatorcontrib><creatorcontrib>Lavery, I C</creatorcontrib><creatorcontrib>Milsom, J W</creatorcontrib><title>Long-term outcome of patients with perianal Paget's disease</title><title>Annals of surgical oncology</title><addtitle>Ann Surg Oncol</addtitle><description>Perianal Paget's disease (PPD) is a rare intraepithelial adenocarcinoma with a significant rate of recurrence after treatment and high risk of progression to an invasive cancer.
Fourteen patients with a mean follow-up longer than 5 years were studied to determine the outcome after surgical treatment. The immunohistochemical accumulation of p53 protein also was assessed in tissue specimens to evaluate its prognostic role in patients with PPD.
Four patients were excluded because of progression to invasive malignancy at the time of diagnosis. Two patients underwent local excision (LE) with macroscopic clearance of the surgical margins; the remaining eight patients underwent wide local excision (WLE), i.e., > 1 cm microscopic clearance of the surgical margins. The actuarial 8-year recurrence rate for patients treated with LE and WLE was 100% and 50% (SE = 17.7), respectively. Progression to invasive carcinoma occurred after a median time of 56 months (range 23-72) in two patients treated with LE and in one of eight patients treated with WLE. All four patients with recurrence after WLE were successfully treated (no further recurrence) with a second WLE. Actuarial 8-year survival was 0% in the LE group and 40% (SE = 21.9) in the WLE group. There was no p53 protein accumulation in any of the ten patients with PPD.
Survival of patients with PPD treated by WLE was higher than that for those treated with LE. Thus, wide local excision is recommended over limited local excision as a preferred treatment for PPD. Follow-up longer than 5 years seems to be indicated because of the risk of late progression to invasive cancer. When PPD does recur, a second WLE may be curative. The absence of accumulated p53 protein suggests that this marker may not have a prognostic role in PPD.</description><subject>Adenocarcinoma</subject><subject>Adult</subject><subject>Aged</subject><subject>Anal Gland Neoplasms - metabolism</subject><subject>Anal Gland Neoplasms - mortality</subject><subject>Anal Gland Neoplasms - surgery</subject><subject>Animals</subject><subject>Antibodies, Monoclonal</subject><subject>Biomarkers, Tumor - biosynthesis</subject><subject>Biomarkers, Tumor - immunology</subject><subject>Disease Progression</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Invasiveness</subject><subject>Male</subject><subject>Malignancy</subject><subject>Middle Aged</subject><subject>Neoplasm Recurrence, Local</subject><subject>p53 Protein</subject><subject>Paget Disease, Extramammary - metabolism</subject><subject>Paget Disease, Extramammary - mortality</subject><subject>Paget Disease, Extramammary - surgery</subject><subject>Paget's disease</subject><subject>Pagets disease</subject><subject>Prognosis</subject><subject>Proteins</subject><subject>Survival</subject><subject>Survival Rate</subject><subject>Time Factors</subject><subject>Treatment Outcome</subject><subject>Tumor Suppressor Protein p53 - biosynthesis</subject><subject>Tumor Suppressor Protein p53 - immunology</subject><issn>1068-9265</issn><issn>1534-4681</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1997</creationdate><recordtype>article</recordtype><recordid>eNpd0E1Lw0AQBuBFlFqrF-9CQFAQorPZj2TxpMWqUNCDnsMmO1tTkmzMbhD_vSktCp5mDg8vMy8hpxSuKUB6c7-AhAGTKd0jUyoYj7nM6P64g8xilUhxSI68XwPQlIGYkIlioBiTU3K7dO0qDtg3kRtC6RqMnI06HSpsg4--qvARddhXutV19KpXGC59ZCqP2uMxObC69niymzPyvnh4mz_Fy5fH5_ndMi6ZoCFGK4U2XFtkKhU2M5wC1UZTnnHUTBkQ3FCwBTfKUg5SSS1UyRQYAUWh2YxcbHO73n0O6EPeVL7EutYtusHn6fhNBkk6wvN_cO2Gfrzc5wmnQkiVqY262qqyd973aPOurxrdf-cU8k2f-V-fIz7bRQ5Fg-aX7gpkPzYcbdc</recordid><startdate>19970901</startdate><enddate>19970901</enddate><creator>Marchesa, P</creator><creator>Fazio, V W</creator><creator>Oliart, S</creator><creator>Goldblum, J R</creator><creator>Lavery, I C</creator><creator>Milsom, J W</creator><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7TO</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>H94</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>7X8</scope></search><sort><creationdate>19970901</creationdate><title>Long-term outcome of patients with perianal Paget's disease</title><author>Marchesa, P ; Fazio, V W ; Oliart, S ; Goldblum, J R ; Lavery, I C ; Milsom, J W</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c351t-ef65ad4afe3975f8d4101ada1484ea39d054d10fb4d9f140696a59c390d50bba3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1997</creationdate><topic>Adenocarcinoma</topic><topic>Adult</topic><topic>Aged</topic><topic>Anal Gland Neoplasms - metabolism</topic><topic>Anal Gland Neoplasms - mortality</topic><topic>Anal Gland Neoplasms - surgery</topic><topic>Animals</topic><topic>Antibodies, Monoclonal</topic><topic>Biomarkers, Tumor - biosynthesis</topic><topic>Biomarkers, Tumor - immunology</topic><topic>Disease Progression</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>Invasiveness</topic><topic>Male</topic><topic>Malignancy</topic><topic>Middle Aged</topic><topic>Neoplasm Recurrence, Local</topic><topic>p53 Protein</topic><topic>Paget Disease, Extramammary - metabolism</topic><topic>Paget Disease, Extramammary - mortality</topic><topic>Paget Disease, Extramammary - surgery</topic><topic>Paget's disease</topic><topic>Pagets disease</topic><topic>Prognosis</topic><topic>Proteins</topic><topic>Survival</topic><topic>Survival Rate</topic><topic>Time Factors</topic><topic>Treatment Outcome</topic><topic>Tumor Suppressor Protein p53 - biosynthesis</topic><topic>Tumor Suppressor Protein p53 - immunology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Marchesa, P</creatorcontrib><creatorcontrib>Fazio, V W</creatorcontrib><creatorcontrib>Oliart, S</creatorcontrib><creatorcontrib>Goldblum, J R</creatorcontrib><creatorcontrib>Lavery, I C</creatorcontrib><creatorcontrib>Milsom, J W</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>ProQuest Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni)</collection><collection>ProQuest Central</collection><collection>AUTh Library subscriptions: ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>PML(ProQuest Medical Library)</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>MEDLINE - Academic</collection><jtitle>Annals of surgical oncology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Marchesa, P</au><au>Fazio, V W</au><au>Oliart, S</au><au>Goldblum, J R</au><au>Lavery, I C</au><au>Milsom, J W</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Long-term outcome of patients with perianal Paget's disease</atitle><jtitle>Annals of surgical oncology</jtitle><addtitle>Ann Surg Oncol</addtitle><date>1997-09-01</date><risdate>1997</risdate><volume>4</volume><issue>6</issue><spage>475</spage><epage>480</epage><pages>475-480</pages><issn>1068-9265</issn><eissn>1534-4681</eissn><notes>ObjectType-Article-1</notes><notes>SourceType-Scholarly Journals-1</notes><notes>ObjectType-Feature-2</notes><notes>content type line 23</notes><abstract>Perianal Paget's disease (PPD) is a rare intraepithelial adenocarcinoma with a significant rate of recurrence after treatment and high risk of progression to an invasive cancer.
Fourteen patients with a mean follow-up longer than 5 years were studied to determine the outcome after surgical treatment. The immunohistochemical accumulation of p53 protein also was assessed in tissue specimens to evaluate its prognostic role in patients with PPD.
Four patients were excluded because of progression to invasive malignancy at the time of diagnosis. Two patients underwent local excision (LE) with macroscopic clearance of the surgical margins; the remaining eight patients underwent wide local excision (WLE), i.e., > 1 cm microscopic clearance of the surgical margins. The actuarial 8-year recurrence rate for patients treated with LE and WLE was 100% and 50% (SE = 17.7), respectively. Progression to invasive carcinoma occurred after a median time of 56 months (range 23-72) in two patients treated with LE and in one of eight patients treated with WLE. All four patients with recurrence after WLE were successfully treated (no further recurrence) with a second WLE. Actuarial 8-year survival was 0% in the LE group and 40% (SE = 21.9) in the WLE group. There was no p53 protein accumulation in any of the ten patients with PPD.
Survival of patients with PPD treated by WLE was higher than that for those treated with LE. Thus, wide local excision is recommended over limited local excision as a preferred treatment for PPD. Follow-up longer than 5 years seems to be indicated because of the risk of late progression to invasive cancer. When PPD does recur, a second WLE may be curative. The absence of accumulated p53 protein suggests that this marker may not have a prognostic role in PPD.</abstract><cop>United States</cop><pub>Springer Nature B.V</pub><pmid>9309336</pmid><doi>10.1007/BF02303671</doi><tpages>6</tpages></addata></record> |
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| subjects | Adenocarcinoma Adult Aged Anal Gland Neoplasms - metabolism Anal Gland Neoplasms - mortality Anal Gland Neoplasms - surgery Animals Antibodies, Monoclonal Biomarkers, Tumor - biosynthesis Biomarkers, Tumor - immunology Disease Progression Female Follow-Up Studies Humans Immunohistochemistry Invasiveness Male Malignancy Middle Aged Neoplasm Recurrence, Local p53 Protein Paget Disease, Extramammary - metabolism Paget Disease, Extramammary - mortality Paget Disease, Extramammary - surgery Paget's disease Pagets disease Prognosis Proteins Survival Survival Rate Time Factors Treatment Outcome Tumor Suppressor Protein p53 - biosynthesis Tumor Suppressor Protein p53 - immunology |
| title | Long-term outcome of patients with perianal Paget's disease |
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