Retrospective Analysis of Prognostic Factors in Pediatric Patients with Adrenocortical Tumor from Unique Tertiary Center with Long-Term Follow-Up
Pediatric adrenocortical tumors (PACTs) represent rare causes of malignancies. However, the south/southeast regions of Brazil are known to have a high incidence of PACTs because of the founder effect associated with a germline pathogenic variant of tumor suppressor gene TP53. We aimed to retrospecti...
Gespeichert in:
| Veröffentlicht in: | Journal of clinical medicine 2022-11, Vol.11 (22), p.6641 |
|---|---|
| Hauptverfasser: | , , , , , , , , , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | |
|---|---|
| container_issue | 22 |
| container_start_page | 6641 |
| container_title | Journal of clinical medicine |
| container_volume | 11 |
| creator | Bachega, Fernanda S Suartz, Caio V Almeida, Madson Q Brondani, Vania B Charchar, Helaine L S Lacombe, Amanda M F Martins-Filho, Sebastião N Soares, Iberê C Zerbini, Maria Claudia N Dénes, Francisco T Mendonca, Berenice Lopes, Roberto I Latronico, Ana Claudia Fragoso, Maria Candida B V |
| description | Pediatric adrenocortical tumors (PACTs) represent rare causes of malignancies. However, the south/southeast regions of Brazil are known to have a high incidence of PACTs because of the founder effect associated with a germline pathogenic variant of tumor suppressor gene TP53. We aimed to retrospectively analyze the types of variables among hormone production, radiological imaging, tumor staging, histological and genetic features that were associated with the occurrence of malignancy in 95 patients (71% females) with PACTs from a unique center. The worst prognosis was associated with those aged > 3 years (p < 0.05), high serum levels of 11-desoxicortisol (p < 0.001), tumor weight ≥ 200 g (p < 0.001), tumor size ≥ 5 cm (p < 0.05), Weiss score ≥ 5 (p < 0.05), Wieneke index ≥ 3 (p < 0.001) and Ki67 ≥ 15% (p < 0.05). Furthermore, patients with MacFarlane stage IV had an overall survival rate almost two times shorter than patients with other stages (p < 0.001). Additionally, the subtractions of BUB1B-PINK1 ( |
| doi_str_mv | 10.3390/jcm11226641 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_9692695</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2739438693</sourcerecordid><originalsourceid>FETCH-LOGICAL-c339t-6f67530724f0b60b8f099d2e8d29cafaca42488ce2fe6115d714500dbdcbdcf93</originalsourceid><addsrcrecordid>eNpdkc1qGzEUhYfSkoQ0q-6LoJtCmFZ_oxltCsbEbcFQU-y1kDWSIzMjuZImIY-RN-4NToJbIZDQ_c7lXJ2q-kDwF8Yk_ro3IyGUCsHJm-qC4ratMevY25P7eXWV8x7D6jpOSXtWnTPBGcj4RfX425YU88Ga4u8smgU9PGSfUXRoleIuxFy8QQttSkwZ-YBWtve6JHhc6eJtKBnd-3KLZn2yIZqYgNcDWk9jTMilOKJN8H8mi9YWSjo9oDmIbDqqljHsaqiMaBGHId7Xm8P76p3TQ7ZXz-dltVncrOc_6uWv7z_ns2VtYPBSCyfahuGWcoe3Am87h6Xsqe16Ko122mhOedcZS50VhDR9S3iDcb_tDWwn2WX17dj3MG1H2xtwlfSgDsmP4FJF7dW_leBv1S7eKSkkFbKBBp-fG6QIA-aiRp-NHQYdbJyyoi3HDW6ZwIB--g_dxynBXz9RTHLWCcmAuj5SBhLJybpXMwSrp7TVSdpAfzz1_8q-ZMv-AoXAqLI</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2739438693</pqid></control><display><type>article</type><title>Retrospective Analysis of Prognostic Factors in Pediatric Patients with Adrenocortical Tumor from Unique Tertiary Center with Long-Term Follow-Up</title><source>PubMed Central Open Access</source><source>MDPI - Multidisciplinary Digital Publishing Institute</source><source>EZB-FREE-00999 freely available EZB journals</source><source>PubMed Central</source><creator>Bachega, Fernanda S ; Suartz, Caio V ; Almeida, Madson Q ; Brondani, Vania B ; Charchar, Helaine L S ; Lacombe, Amanda M F ; Martins-Filho, Sebastião N ; Soares, Iberê C ; Zerbini, Maria Claudia N ; Dénes, Francisco T ; Mendonca, Berenice ; Lopes, Roberto I ; Latronico, Ana Claudia ; Fragoso, Maria Candida B V</creator><creatorcontrib>Bachega, Fernanda S ; Suartz, Caio V ; Almeida, Madson Q ; Brondani, Vania B ; Charchar, Helaine L S ; Lacombe, Amanda M F ; Martins-Filho, Sebastião N ; Soares, Iberê C ; Zerbini, Maria Claudia N ; Dénes, Francisco T ; Mendonca, Berenice ; Lopes, Roberto I ; Latronico, Ana Claudia ; Fragoso, Maria Candida B V</creatorcontrib><description><![CDATA[Pediatric adrenocortical tumors (PACTs) represent rare causes of malignancies. However, the south/southeast regions of Brazil are known to have a high incidence of PACTs because of the founder effect associated with a germline pathogenic variant of tumor suppressor gene TP53. We aimed to retrospectively analyze the types of variables among hormone production, radiological imaging, tumor staging, histological and genetic features that were associated with the occurrence of malignancy in 95 patients (71% females) with PACTs from a unique center. The worst prognosis was associated with those aged > 3 years (p < 0.05), high serum levels of 11-desoxicortisol (p < 0.001), tumor weight ≥ 200 g (p < 0.001), tumor size ≥ 5 cm (p < 0.05), Weiss score ≥ 5 (p < 0.05), Wieneke index ≥ 3 (p < 0.001) and Ki67 ≥ 15% (p < 0.05). Furthermore, patients with MacFarlane stage IV had an overall survival rate almost two times shorter than patients with other stages (p < 0.001). Additionally, the subtractions of BUB1B-PINK1 (<6.95) expression (p < 0.05) and IGF-IR overexpression (p = 0.0001) were associated with malignant behavior. These results helped identify patients who are likely to have an aggressive course; further multicenter prospective studies are required to confirm our results. In conclusion, PACTs with these patterns of prognostic factors could be treated using an adjuvant approach that may improve the overall survival in such patients.]]></description><identifier>ISSN: 2077-0383</identifier><identifier>EISSN: 2077-0383</identifier><identifier>DOI: 10.3390/jcm11226641</identifier><identifier>PMID: 36431124</identifier><language>eng</language><publisher>Switzerland: MDPI AG</publisher><subject>Age ; Females ; Medical prognosis ; Metastasis ; Pediatrics ; Review ; Tumors</subject><ispartof>Journal of clinical medicine, 2022-11, Vol.11 (22), p.6641</ispartof><rights>2022 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>2022 by the authors. 2022</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c339t-6f67530724f0b60b8f099d2e8d29cafaca42488ce2fe6115d714500dbdcbdcf93</citedby><cites>FETCH-LOGICAL-c339t-6f67530724f0b60b8f099d2e8d29cafaca42488ce2fe6115d714500dbdcbdcf93</cites><orcidid>0000-0001-6383-4668 ; 0000-0002-1364-5508 ; 0000-0002-2957-6148 ; 0000-0002-6636-7679 ; 0000-0002-7408-9816</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC9692695/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC9692695/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,885,27924,27925,53791,53793</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/36431124$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Bachega, Fernanda S</creatorcontrib><creatorcontrib>Suartz, Caio V</creatorcontrib><creatorcontrib>Almeida, Madson Q</creatorcontrib><creatorcontrib>Brondani, Vania B</creatorcontrib><creatorcontrib>Charchar, Helaine L S</creatorcontrib><creatorcontrib>Lacombe, Amanda M F</creatorcontrib><creatorcontrib>Martins-Filho, Sebastião N</creatorcontrib><creatorcontrib>Soares, Iberê C</creatorcontrib><creatorcontrib>Zerbini, Maria Claudia N</creatorcontrib><creatorcontrib>Dénes, Francisco T</creatorcontrib><creatorcontrib>Mendonca, Berenice</creatorcontrib><creatorcontrib>Lopes, Roberto I</creatorcontrib><creatorcontrib>Latronico, Ana Claudia</creatorcontrib><creatorcontrib>Fragoso, Maria Candida B V</creatorcontrib><title>Retrospective Analysis of Prognostic Factors in Pediatric Patients with Adrenocortical Tumor from Unique Tertiary Center with Long-Term Follow-Up</title><title>Journal of clinical medicine</title><addtitle>J Clin Med</addtitle><description><![CDATA[Pediatric adrenocortical tumors (PACTs) represent rare causes of malignancies. However, the south/southeast regions of Brazil are known to have a high incidence of PACTs because of the founder effect associated with a germline pathogenic variant of tumor suppressor gene TP53. We aimed to retrospectively analyze the types of variables among hormone production, radiological imaging, tumor staging, histological and genetic features that were associated with the occurrence of malignancy in 95 patients (71% females) with PACTs from a unique center. The worst prognosis was associated with those aged > 3 years (p < 0.05), high serum levels of 11-desoxicortisol (p < 0.001), tumor weight ≥ 200 g (p < 0.001), tumor size ≥ 5 cm (p < 0.05), Weiss score ≥ 5 (p < 0.05), Wieneke index ≥ 3 (p < 0.001) and Ki67 ≥ 15% (p < 0.05). Furthermore, patients with MacFarlane stage IV had an overall survival rate almost two times shorter than patients with other stages (p < 0.001). Additionally, the subtractions of BUB1B-PINK1 (<6.95) expression (p < 0.05) and IGF-IR overexpression (p = 0.0001) were associated with malignant behavior. These results helped identify patients who are likely to have an aggressive course; further multicenter prospective studies are required to confirm our results. In conclusion, PACTs with these patterns of prognostic factors could be treated using an adjuvant approach that may improve the overall survival in such patients.]]></description><subject>Age</subject><subject>Females</subject><subject>Medical prognosis</subject><subject>Metastasis</subject><subject>Pediatrics</subject><subject>Review</subject><subject>Tumors</subject><issn>2077-0383</issn><issn>2077-0383</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><recordid>eNpdkc1qGzEUhYfSkoQ0q-6LoJtCmFZ_oxltCsbEbcFQU-y1kDWSIzMjuZImIY-RN-4NToJbIZDQ_c7lXJ2q-kDwF8Yk_ro3IyGUCsHJm-qC4ratMevY25P7eXWV8x7D6jpOSXtWnTPBGcj4RfX425YU88Ga4u8smgU9PGSfUXRoleIuxFy8QQttSkwZ-YBWtve6JHhc6eJtKBnd-3KLZn2yIZqYgNcDWk9jTMilOKJN8H8mi9YWSjo9oDmIbDqqljHsaqiMaBGHId7Xm8P76p3TQ7ZXz-dltVncrOc_6uWv7z_ns2VtYPBSCyfahuGWcoe3Am87h6Xsqe16Ko122mhOedcZS50VhDR9S3iDcb_tDWwn2WX17dj3MG1H2xtwlfSgDsmP4FJF7dW_leBv1S7eKSkkFbKBBp-fG6QIA-aiRp-NHQYdbJyyoi3HDW6ZwIB--g_dxynBXz9RTHLWCcmAuj5SBhLJybpXMwSrp7TVSdpAfzz1_8q-ZMv-AoXAqLI</recordid><startdate>20221109</startdate><enddate>20221109</enddate><creator>Bachega, Fernanda S</creator><creator>Suartz, Caio V</creator><creator>Almeida, Madson Q</creator><creator>Brondani, Vania B</creator><creator>Charchar, Helaine L S</creator><creator>Lacombe, Amanda M F</creator><creator>Martins-Filho, Sebastião N</creator><creator>Soares, Iberê C</creator><creator>Zerbini, Maria Claudia N</creator><creator>Dénes, Francisco T</creator><creator>Mendonca, Berenice</creator><creator>Lopes, Roberto I</creator><creator>Latronico, Ana Claudia</creator><creator>Fragoso, Maria Candida B V</creator><general>MDPI AG</general><general>MDPI</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0001-6383-4668</orcidid><orcidid>https://orcid.org/0000-0002-1364-5508</orcidid><orcidid>https://orcid.org/0000-0002-2957-6148</orcidid><orcidid>https://orcid.org/0000-0002-6636-7679</orcidid><orcidid>https://orcid.org/0000-0002-7408-9816</orcidid></search><sort><creationdate>20221109</creationdate><title>Retrospective Analysis of Prognostic Factors in Pediatric Patients with Adrenocortical Tumor from Unique Tertiary Center with Long-Term Follow-Up</title><author>Bachega, Fernanda S ; Suartz, Caio V ; Almeida, Madson Q ; Brondani, Vania B ; Charchar, Helaine L S ; Lacombe, Amanda M F ; Martins-Filho, Sebastião N ; Soares, Iberê C ; Zerbini, Maria Claudia N ; Dénes, Francisco T ; Mendonca, Berenice ; Lopes, Roberto I ; Latronico, Ana Claudia ; Fragoso, Maria Candida B V</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c339t-6f67530724f0b60b8f099d2e8d29cafaca42488ce2fe6115d714500dbdcbdcf93</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2022</creationdate><topic>Age</topic><topic>Females</topic><topic>Medical prognosis</topic><topic>Metastasis</topic><topic>Pediatrics</topic><topic>Review</topic><topic>Tumors</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Bachega, Fernanda S</creatorcontrib><creatorcontrib>Suartz, Caio V</creatorcontrib><creatorcontrib>Almeida, Madson Q</creatorcontrib><creatorcontrib>Brondani, Vania B</creatorcontrib><creatorcontrib>Charchar, Helaine L S</creatorcontrib><creatorcontrib>Lacombe, Amanda M F</creatorcontrib><creatorcontrib>Martins-Filho, Sebastião N</creatorcontrib><creatorcontrib>Soares, Iberê C</creatorcontrib><creatorcontrib>Zerbini, Maria Claudia N</creatorcontrib><creatorcontrib>Dénes, Francisco T</creatorcontrib><creatorcontrib>Mendonca, Berenice</creatorcontrib><creatorcontrib>Lopes, Roberto I</creatorcontrib><creatorcontrib>Latronico, Ana Claudia</creatorcontrib><creatorcontrib>Fragoso, Maria Candida B V</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</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 Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Access via ProQuest (Open Access)</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Journal of clinical medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Bachega, Fernanda S</au><au>Suartz, Caio V</au><au>Almeida, Madson Q</au><au>Brondani, Vania B</au><au>Charchar, Helaine L S</au><au>Lacombe, Amanda M F</au><au>Martins-Filho, Sebastião N</au><au>Soares, Iberê C</au><au>Zerbini, Maria Claudia N</au><au>Dénes, Francisco T</au><au>Mendonca, Berenice</au><au>Lopes, Roberto I</au><au>Latronico, Ana Claudia</au><au>Fragoso, Maria Candida B V</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Retrospective Analysis of Prognostic Factors in Pediatric Patients with Adrenocortical Tumor from Unique Tertiary Center with Long-Term Follow-Up</atitle><jtitle>Journal of clinical medicine</jtitle><addtitle>J Clin Med</addtitle><date>2022-11-09</date><risdate>2022</risdate><volume>11</volume><issue>22</issue><spage>6641</spage><pages>6641-</pages><issn>2077-0383</issn><eissn>2077-0383</eissn><abstract><![CDATA[Pediatric adrenocortical tumors (PACTs) represent rare causes of malignancies. However, the south/southeast regions of Brazil are known to have a high incidence of PACTs because of the founder effect associated with a germline pathogenic variant of tumor suppressor gene TP53. We aimed to retrospectively analyze the types of variables among hormone production, radiological imaging, tumor staging, histological and genetic features that were associated with the occurrence of malignancy in 95 patients (71% females) with PACTs from a unique center. The worst prognosis was associated with those aged > 3 years (p < 0.05), high serum levels of 11-desoxicortisol (p < 0.001), tumor weight ≥ 200 g (p < 0.001), tumor size ≥ 5 cm (p < 0.05), Weiss score ≥ 5 (p < 0.05), Wieneke index ≥ 3 (p < 0.001) and Ki67 ≥ 15% (p < 0.05). Furthermore, patients with MacFarlane stage IV had an overall survival rate almost two times shorter than patients with other stages (p < 0.001). Additionally, the subtractions of BUB1B-PINK1 (<6.95) expression (p < 0.05) and IGF-IR overexpression (p = 0.0001) were associated with malignant behavior. These results helped identify patients who are likely to have an aggressive course; further multicenter prospective studies are required to confirm our results. In conclusion, PACTs with these patterns of prognostic factors could be treated using an adjuvant approach that may improve the overall survival in such patients.]]></abstract><cop>Switzerland</cop><pub>MDPI AG</pub><pmid>36431124</pmid><doi>10.3390/jcm11226641</doi><orcidid>https://orcid.org/0000-0001-6383-4668</orcidid><orcidid>https://orcid.org/0000-0002-1364-5508</orcidid><orcidid>https://orcid.org/0000-0002-2957-6148</orcidid><orcidid>https://orcid.org/0000-0002-6636-7679</orcidid><orcidid>https://orcid.org/0000-0002-7408-9816</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 2077-0383 |
| ispartof | Journal of clinical medicine, 2022-11, Vol.11 (22), p.6641 |
| issn | 2077-0383 2077-0383 |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_9692695 |
| source | PubMed Central Open Access; MDPI - Multidisciplinary Digital Publishing Institute; EZB-FREE-00999 freely available EZB journals; PubMed Central |
| subjects | Age Females Medical prognosis Metastasis Pediatrics Review Tumors |
| title | Retrospective Analysis of Prognostic Factors in Pediatric Patients with Adrenocortical Tumor from Unique Tertiary Center with Long-Term Follow-Up |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-18T21%3A41%3A29IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Retrospective%20Analysis%20of%20Prognostic%20Factors%20in%20Pediatric%20Patients%20with%20Adrenocortical%20Tumor%20from%20Unique%20Tertiary%20Center%20with%20Long-Term%20Follow-Up&rft.jtitle=Journal%20of%20clinical%20medicine&rft.au=Bachega,%20Fernanda%20S&rft.date=2022-11-09&rft.volume=11&rft.issue=22&rft.spage=6641&rft.pages=6641-&rft.issn=2077-0383&rft.eissn=2077-0383&rft_id=info:doi/10.3390/jcm11226641&rft_dat=%3Cproquest_pubme%3E2739438693%3C/proquest_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2739438693&rft_id=info:pmid/36431124&rfr_iscdi=true |