Malignancies in Prader-Willi Syndrome: Results From a Large International Cohort and Literature Review
Abstract Context Prader-Willi syndrome (PWS) is a complex disorder combining hypothalamic dysfunction, neurodevelopmental delay, hypotonia, and hyperphagia with risk of obesity and its complications. PWS is caused by the loss of expression of the PWS critical region, a cluster of paternally expresse...
Gespeichert in:
Veröffentlicht in: | The journal of clinical endocrinology and metabolism 2023-12, Vol.108 (12), p.e1720-e1730 |
---|---|
Hauptverfasser: | , , , , , , , , , , , , , , |
Format: | Artikel |
Sprache: | eng |
Schlagworte: | |
Online-Zugang: | Volltext |
Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
container_end_page | e1730 |
---|---|
container_issue | 12 |
container_start_page | e1720 |
container_title | The journal of clinical endocrinology and metabolism |
container_volume | 108 |
creator | Pellikaan, Karlijn Nguyen, Naomi Q C Rosenberg, Anna G W Coupaye, Muriel Goldstone, Anthony P Høybye, Charlotte Markovic, Tania Grugni, Graziano Crinò, Antonino Caixàs, Assumpta Poitou, Christine Corripio, Raquel Nieuwenhuize, Rosa M van der Lely, Aart J de Graaff, Laura C G |
description | Abstract
Context
Prader-Willi syndrome (PWS) is a complex disorder combining hypothalamic dysfunction, neurodevelopmental delay, hypotonia, and hyperphagia with risk of obesity and its complications. PWS is caused by the loss of expression of the PWS critical region, a cluster of paternally expressed genes on chromosome 15q11.2-q13. As life expectancy of patients with PWS increases, age-related diseases like malignancies might pose a new threat to health.
Objective
To investigate the prevalence and risk factors of malignancies in patients with PWS and to provide clinical recommendations for cancer screening.
Methods
We included 706 patients with PWS (160 children, 546 adults). We retrospectively collected data from medical records on past or current malignancies, the type of malignancy, and risk factors for malignancy. Additionally, we searched the literature for information about the relationship between genes on chromosome 15q11.2-q13 and malignancies.
Results
Seven adults (age range, 18-55 years) had been diagnosed with a malignancy (acute lymphoblastic leukemia, intracranial hemangiopericytoma, melanoma, stomach adenocarcinoma, biliary cancer, parotid adenocarcinoma, and colon cancer). All patients with a malignancy had a paternal 15q11-13 deletion. The literature review showed that several genes on chromosome 15q11.2-q13 are related to malignancies.
Conclusion
Malignancies are rare in patients with PWS. Therefore, screening for malignancies is only indicated when clinically relevant symptoms are present, such as unexplained weight loss, loss of appetite, symptoms suggestive of paraneoplastic syndrome, or localizing symptoms. Given the increased cancer risk associated with obesity, which is common in PWS, participation in national screening programs should be encouraged. |
doi_str_mv | 10.1210/clinem/dgad312 |
format | Article |
fullrecord | <record><control><sourceid>gale_swepu</sourceid><recordid>TN_cdi_swepub_primary_oai_swepub_ki_se_668336</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><galeid>A775958545</galeid><oup_id>10.1210/clinem/dgad312</oup_id><sourcerecordid>A775958545</sourcerecordid><originalsourceid>FETCH-LOGICAL-c502t-97efae4cacf34553e193429645346ae8b667adb7a10021961ba17878f162f64c3</originalsourceid><addsrcrecordid>eNqFks2LFDEQxYMo7jh69SgBL3ro3Xx0J93elsHVhRHFD_QWatLVY9Z0MibdLvvfm2HGFWRBckhS-b3iPVKEPOXslAvOzqx3Acezfgu95OIeWfCubirNO32fLBgTvOq0-HZCHuV8xRiv60Y-JCdSC6VryRZkeAfebQME6zBTF-iHBD2m6qvz3tFPN6FPccRX9CPm2U-ZXpQrBbqGtEV6GSZMASYXA3i6it9jmiiEnq5deYBpTliEvxxePyYPBvAZnxz3Jfly8frz6m21fv_mcnW-rmzDxFS84gBYW7CDrJtGIu9kLTpVXNcKsN0opaHfaOD7aJ3iG-C61e3AlRhUbeWSVIe--Rp388bskhsh3ZgIzhxLP8oJjVKtlKrwLw78LsWfM-bJjC5b9B4Cxjkb0QohdccaWdDn_6BXcS7pfaG64pXxlrG_1BY8GheGOCWw-6bmXOuma9qmhFmS0zuosnocnY0BB1fqdwlsijknHG6TcWb2g2AOg2COg1AEz45u582I_S3-5-cL8PIAxHn3v2a_AXRgvPw</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2955301800</pqid></control><display><type>article</type><title>Malignancies in Prader-Willi Syndrome: Results From a Large International Cohort and Literature Review</title><source>Oxford University Press Journals All Titles (1996-Current)</source><source>MEDLINE</source><source>Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals</source><source>Alma/SFX Local Collection</source><source>SWEPUB Freely available online</source><creator>Pellikaan, Karlijn ; Nguyen, Naomi Q C ; Rosenberg, Anna G W ; Coupaye, Muriel ; Goldstone, Anthony P ; Høybye, Charlotte ; Markovic, Tania ; Grugni, Graziano ; Crinò, Antonino ; Caixàs, Assumpta ; Poitou, Christine ; Corripio, Raquel ; Nieuwenhuize, Rosa M ; van der Lely, Aart J ; de Graaff, Laura C G</creator><creatorcontrib>Pellikaan, Karlijn ; Nguyen, Naomi Q C ; Rosenberg, Anna G W ; Coupaye, Muriel ; Goldstone, Anthony P ; Høybye, Charlotte ; Markovic, Tania ; Grugni, Graziano ; Crinò, Antonino ; Caixàs, Assumpta ; Poitou, Christine ; Corripio, Raquel ; Nieuwenhuize, Rosa M ; van der Lely, Aart J ; de Graaff, Laura C G</creatorcontrib><description>Abstract
Context
Prader-Willi syndrome (PWS) is a complex disorder combining hypothalamic dysfunction, neurodevelopmental delay, hypotonia, and hyperphagia with risk of obesity and its complications. PWS is caused by the loss of expression of the PWS critical region, a cluster of paternally expressed genes on chromosome 15q11.2-q13. As life expectancy of patients with PWS increases, age-related diseases like malignancies might pose a new threat to health.
Objective
To investigate the prevalence and risk factors of malignancies in patients with PWS and to provide clinical recommendations for cancer screening.
Methods
We included 706 patients with PWS (160 children, 546 adults). We retrospectively collected data from medical records on past or current malignancies, the type of malignancy, and risk factors for malignancy. Additionally, we searched the literature for information about the relationship between genes on chromosome 15q11.2-q13 and malignancies.
Results
Seven adults (age range, 18-55 years) had been diagnosed with a malignancy (acute lymphoblastic leukemia, intracranial hemangiopericytoma, melanoma, stomach adenocarcinoma, biliary cancer, parotid adenocarcinoma, and colon cancer). All patients with a malignancy had a paternal 15q11-13 deletion. The literature review showed that several genes on chromosome 15q11.2-q13 are related to malignancies.
Conclusion
Malignancies are rare in patients with PWS. Therefore, screening for malignancies is only indicated when clinically relevant symptoms are present, such as unexplained weight loss, loss of appetite, symptoms suggestive of paraneoplastic syndrome, or localizing symptoms. Given the increased cancer risk associated with obesity, which is common in PWS, participation in national screening programs should be encouraged.</description><identifier>ISSN: 0021-972X</identifier><identifier>ISSN: 1945-7197</identifier><identifier>EISSN: 1945-7197</identifier><identifier>DOI: 10.1210/clinem/dgad312</identifier><identifier>PMID: 37267430</identifier><language>eng</language><publisher>US: Oxford University Press</publisher><subject>Acute lymphoblastic leukemia ; Adenocarcinoma ; Adolescent ; Adult ; Age ; Appetite loss ; Autoimmune diseases ; Body weight loss ; Cancer ; Cancer screening ; Child ; Chromosome 15 ; Chromosome deletion ; Chromosomes ; Colon cancer ; Colorectal cancer ; Diagnosis ; Fathers ; Genes ; Health aspects ; Humans ; Hyperphagia ; Hypothalamus ; Life span ; Literature reviews ; Lymphatic leukemia ; Malignancy ; Medical records ; Medical screening ; Melanoma ; Middle Aged ; Neurodevelopmental disorders ; Obesity ; Paraneoplastic syndrome ; Patients ; Prader-Willi syndrome ; Prader-Willi Syndrome - complications ; Prader-Willi Syndrome - diagnosis ; Prader-Willi Syndrome - epidemiology ; Retrospective Studies ; Risk factors ; Weight control ; Young Adult</subject><ispartof>The journal of clinical endocrinology and metabolism, 2023-12, Vol.108 (12), p.e1720-e1730</ispartof><rights>The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. 2023</rights><rights>The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society.</rights><rights>COPYRIGHT 2023 Oxford University Press</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c502t-97efae4cacf34553e193429645346ae8b667adb7a10021961ba17878f162f64c3</citedby><cites>FETCH-LOGICAL-c502t-97efae4cacf34553e193429645346ae8b667adb7a10021961ba17878f162f64c3</cites><orcidid>0000-0002-0738-0275 ; 0000-0001-7769-6331 ; 0000-0002-0295-7063 ; 0000-0001-8179-7071 ; 0000-0002-1059-0126</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,314,550,776,780,881,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37267430$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttp://kipublications.ki.se/Default.aspx?queryparsed=id:155156711$$DView record from Swedish Publication Index$$Hfree_for_read</backlink></links><search><creatorcontrib>Pellikaan, Karlijn</creatorcontrib><creatorcontrib>Nguyen, Naomi Q C</creatorcontrib><creatorcontrib>Rosenberg, Anna G W</creatorcontrib><creatorcontrib>Coupaye, Muriel</creatorcontrib><creatorcontrib>Goldstone, Anthony P</creatorcontrib><creatorcontrib>Høybye, Charlotte</creatorcontrib><creatorcontrib>Markovic, Tania</creatorcontrib><creatorcontrib>Grugni, Graziano</creatorcontrib><creatorcontrib>Crinò, Antonino</creatorcontrib><creatorcontrib>Caixàs, Assumpta</creatorcontrib><creatorcontrib>Poitou, Christine</creatorcontrib><creatorcontrib>Corripio, Raquel</creatorcontrib><creatorcontrib>Nieuwenhuize, Rosa M</creatorcontrib><creatorcontrib>van der Lely, Aart J</creatorcontrib><creatorcontrib>de Graaff, Laura C G</creatorcontrib><title>Malignancies in Prader-Willi Syndrome: Results From a Large International Cohort and Literature Review</title><title>The journal of clinical endocrinology and metabolism</title><addtitle>J Clin Endocrinol Metab</addtitle><description>Abstract
Context
Prader-Willi syndrome (PWS) is a complex disorder combining hypothalamic dysfunction, neurodevelopmental delay, hypotonia, and hyperphagia with risk of obesity and its complications. PWS is caused by the loss of expression of the PWS critical region, a cluster of paternally expressed genes on chromosome 15q11.2-q13. As life expectancy of patients with PWS increases, age-related diseases like malignancies might pose a new threat to health.
Objective
To investigate the prevalence and risk factors of malignancies in patients with PWS and to provide clinical recommendations for cancer screening.
Methods
We included 706 patients with PWS (160 children, 546 adults). We retrospectively collected data from medical records on past or current malignancies, the type of malignancy, and risk factors for malignancy. Additionally, we searched the literature for information about the relationship between genes on chromosome 15q11.2-q13 and malignancies.
Results
Seven adults (age range, 18-55 years) had been diagnosed with a malignancy (acute lymphoblastic leukemia, intracranial hemangiopericytoma, melanoma, stomach adenocarcinoma, biliary cancer, parotid adenocarcinoma, and colon cancer). All patients with a malignancy had a paternal 15q11-13 deletion. The literature review showed that several genes on chromosome 15q11.2-q13 are related to malignancies.
Conclusion
Malignancies are rare in patients with PWS. Therefore, screening for malignancies is only indicated when clinically relevant symptoms are present, such as unexplained weight loss, loss of appetite, symptoms suggestive of paraneoplastic syndrome, or localizing symptoms. Given the increased cancer risk associated with obesity, which is common in PWS, participation in national screening programs should be encouraged.</description><subject>Acute lymphoblastic leukemia</subject><subject>Adenocarcinoma</subject><subject>Adolescent</subject><subject>Adult</subject><subject>Age</subject><subject>Appetite loss</subject><subject>Autoimmune diseases</subject><subject>Body weight loss</subject><subject>Cancer</subject><subject>Cancer screening</subject><subject>Child</subject><subject>Chromosome 15</subject><subject>Chromosome deletion</subject><subject>Chromosomes</subject><subject>Colon cancer</subject><subject>Colorectal cancer</subject><subject>Diagnosis</subject><subject>Fathers</subject><subject>Genes</subject><subject>Health aspects</subject><subject>Humans</subject><subject>Hyperphagia</subject><subject>Hypothalamus</subject><subject>Life span</subject><subject>Literature reviews</subject><subject>Lymphatic leukemia</subject><subject>Malignancy</subject><subject>Medical records</subject><subject>Medical screening</subject><subject>Melanoma</subject><subject>Middle Aged</subject><subject>Neurodevelopmental disorders</subject><subject>Obesity</subject><subject>Paraneoplastic syndrome</subject><subject>Patients</subject><subject>Prader-Willi syndrome</subject><subject>Prader-Willi Syndrome - complications</subject><subject>Prader-Willi Syndrome - diagnosis</subject><subject>Prader-Willi Syndrome - epidemiology</subject><subject>Retrospective Studies</subject><subject>Risk factors</subject><subject>Weight control</subject><subject>Young Adult</subject><issn>0021-972X</issn><issn>1945-7197</issn><issn>1945-7197</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><sourceid>TOX</sourceid><sourceid>EIF</sourceid><sourceid>D8T</sourceid><recordid>eNqFks2LFDEQxYMo7jh69SgBL3ro3Xx0J93elsHVhRHFD_QWatLVY9Z0MibdLvvfm2HGFWRBckhS-b3iPVKEPOXslAvOzqx3Acezfgu95OIeWfCubirNO32fLBgTvOq0-HZCHuV8xRiv60Y-JCdSC6VryRZkeAfebQME6zBTF-iHBD2m6qvz3tFPN6FPccRX9CPm2U-ZXpQrBbqGtEV6GSZMASYXA3i6it9jmiiEnq5deYBpTliEvxxePyYPBvAZnxz3Jfly8frz6m21fv_mcnW-rmzDxFS84gBYW7CDrJtGIu9kLTpVXNcKsN0opaHfaOD7aJ3iG-C61e3AlRhUbeWSVIe--Rp388bskhsh3ZgIzhxLP8oJjVKtlKrwLw78LsWfM-bJjC5b9B4Cxjkb0QohdccaWdDn_6BXcS7pfaG64pXxlrG_1BY8GheGOCWw-6bmXOuma9qmhFmS0zuosnocnY0BB1fqdwlsijknHG6TcWb2g2AOg2COg1AEz45u582I_S3-5-cL8PIAxHn3v2a_AXRgvPw</recordid><startdate>20231201</startdate><enddate>20231201</enddate><creator>Pellikaan, Karlijn</creator><creator>Nguyen, Naomi Q C</creator><creator>Rosenberg, Anna G W</creator><creator>Coupaye, Muriel</creator><creator>Goldstone, Anthony P</creator><creator>Høybye, Charlotte</creator><creator>Markovic, Tania</creator><creator>Grugni, Graziano</creator><creator>Crinò, Antonino</creator><creator>Caixàs, Assumpta</creator><creator>Poitou, Christine</creator><creator>Corripio, Raquel</creator><creator>Nieuwenhuize, Rosa M</creator><creator>van der Lely, Aart J</creator><creator>de Graaff, Laura C G</creator><general>Oxford University Press</general><scope>TOX</scope><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>7QP</scope><scope>7T5</scope><scope>7TM</scope><scope>H94</scope><scope>K9.</scope><scope>7X8</scope><scope>ADTPV</scope><scope>AOWAS</scope><scope>D8T</scope><scope>ZZAVC</scope><orcidid>https://orcid.org/0000-0002-0738-0275</orcidid><orcidid>https://orcid.org/0000-0001-7769-6331</orcidid><orcidid>https://orcid.org/0000-0002-0295-7063</orcidid><orcidid>https://orcid.org/0000-0001-8179-7071</orcidid><orcidid>https://orcid.org/0000-0002-1059-0126</orcidid></search><sort><creationdate>20231201</creationdate><title>Malignancies in Prader-Willi Syndrome: Results From a Large International Cohort and Literature Review</title><author>Pellikaan, Karlijn ; Nguyen, Naomi Q C ; Rosenberg, Anna G W ; Coupaye, Muriel ; Goldstone, Anthony P ; Høybye, Charlotte ; Markovic, Tania ; Grugni, Graziano ; Crinò, Antonino ; Caixàs, Assumpta ; Poitou, Christine ; Corripio, Raquel ; Nieuwenhuize, Rosa M ; van der Lely, Aart J ; de Graaff, Laura C G</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c502t-97efae4cacf34553e193429645346ae8b667adb7a10021961ba17878f162f64c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>Acute lymphoblastic leukemia</topic><topic>Adenocarcinoma</topic><topic>Adolescent</topic><topic>Adult</topic><topic>Age</topic><topic>Appetite loss</topic><topic>Autoimmune diseases</topic><topic>Body weight loss</topic><topic>Cancer</topic><topic>Cancer screening</topic><topic>Child</topic><topic>Chromosome 15</topic><topic>Chromosome deletion</topic><topic>Chromosomes</topic><topic>Colon cancer</topic><topic>Colorectal cancer</topic><topic>Diagnosis</topic><topic>Fathers</topic><topic>Genes</topic><topic>Health aspects</topic><topic>Humans</topic><topic>Hyperphagia</topic><topic>Hypothalamus</topic><topic>Life span</topic><topic>Literature reviews</topic><topic>Lymphatic leukemia</topic><topic>Malignancy</topic><topic>Medical records</topic><topic>Medical screening</topic><topic>Melanoma</topic><topic>Middle Aged</topic><topic>Neurodevelopmental disorders</topic><topic>Obesity</topic><topic>Paraneoplastic syndrome</topic><topic>Patients</topic><topic>Prader-Willi syndrome</topic><topic>Prader-Willi Syndrome - complications</topic><topic>Prader-Willi Syndrome - diagnosis</topic><topic>Prader-Willi Syndrome - epidemiology</topic><topic>Retrospective Studies</topic><topic>Risk factors</topic><topic>Weight control</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Pellikaan, Karlijn</creatorcontrib><creatorcontrib>Nguyen, Naomi Q C</creatorcontrib><creatorcontrib>Rosenberg, Anna G W</creatorcontrib><creatorcontrib>Coupaye, Muriel</creatorcontrib><creatorcontrib>Goldstone, Anthony P</creatorcontrib><creatorcontrib>Høybye, Charlotte</creatorcontrib><creatorcontrib>Markovic, Tania</creatorcontrib><creatorcontrib>Grugni, Graziano</creatorcontrib><creatorcontrib>Crinò, Antonino</creatorcontrib><creatorcontrib>Caixàs, Assumpta</creatorcontrib><creatorcontrib>Poitou, Christine</creatorcontrib><creatorcontrib>Corripio, Raquel</creatorcontrib><creatorcontrib>Nieuwenhuize, Rosa M</creatorcontrib><creatorcontrib>van der Lely, Aart J</creatorcontrib><creatorcontrib>de Graaff, Laura C G</creatorcontrib><collection>Oxford Journals Open Access Collection</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Immunology Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><collection>SwePub</collection><collection>SwePub Articles</collection><collection>SWEPUB Freely available online</collection><collection>SwePub Articles full text</collection><jtitle>The journal of clinical endocrinology and metabolism</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Pellikaan, Karlijn</au><au>Nguyen, Naomi Q C</au><au>Rosenberg, Anna G W</au><au>Coupaye, Muriel</au><au>Goldstone, Anthony P</au><au>Høybye, Charlotte</au><au>Markovic, Tania</au><au>Grugni, Graziano</au><au>Crinò, Antonino</au><au>Caixàs, Assumpta</au><au>Poitou, Christine</au><au>Corripio, Raquel</au><au>Nieuwenhuize, Rosa M</au><au>van der Lely, Aart J</au><au>de Graaff, Laura C G</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Malignancies in Prader-Willi Syndrome: Results From a Large International Cohort and Literature Review</atitle><jtitle>The journal of clinical endocrinology and metabolism</jtitle><addtitle>J Clin Endocrinol Metab</addtitle><date>2023-12-01</date><risdate>2023</risdate><volume>108</volume><issue>12</issue><spage>e1720</spage><epage>e1730</epage><pages>e1720-e1730</pages><issn>0021-972X</issn><issn>1945-7197</issn><eissn>1945-7197</eissn><abstract>Abstract
Context
Prader-Willi syndrome (PWS) is a complex disorder combining hypothalamic dysfunction, neurodevelopmental delay, hypotonia, and hyperphagia with risk of obesity and its complications. PWS is caused by the loss of expression of the PWS critical region, a cluster of paternally expressed genes on chromosome 15q11.2-q13. As life expectancy of patients with PWS increases, age-related diseases like malignancies might pose a new threat to health.
Objective
To investigate the prevalence and risk factors of malignancies in patients with PWS and to provide clinical recommendations for cancer screening.
Methods
We included 706 patients with PWS (160 children, 546 adults). We retrospectively collected data from medical records on past or current malignancies, the type of malignancy, and risk factors for malignancy. Additionally, we searched the literature for information about the relationship between genes on chromosome 15q11.2-q13 and malignancies.
Results
Seven adults (age range, 18-55 years) had been diagnosed with a malignancy (acute lymphoblastic leukemia, intracranial hemangiopericytoma, melanoma, stomach adenocarcinoma, biliary cancer, parotid adenocarcinoma, and colon cancer). All patients with a malignancy had a paternal 15q11-13 deletion. The literature review showed that several genes on chromosome 15q11.2-q13 are related to malignancies.
Conclusion
Malignancies are rare in patients with PWS. Therefore, screening for malignancies is only indicated when clinically relevant symptoms are present, such as unexplained weight loss, loss of appetite, symptoms suggestive of paraneoplastic syndrome, or localizing symptoms. Given the increased cancer risk associated with obesity, which is common in PWS, participation in national screening programs should be encouraged.</abstract><cop>US</cop><pub>Oxford University Press</pub><pmid>37267430</pmid><doi>10.1210/clinem/dgad312</doi><orcidid>https://orcid.org/0000-0002-0738-0275</orcidid><orcidid>https://orcid.org/0000-0001-7769-6331</orcidid><orcidid>https://orcid.org/0000-0002-0295-7063</orcidid><orcidid>https://orcid.org/0000-0001-8179-7071</orcidid><orcidid>https://orcid.org/0000-0002-1059-0126</orcidid><oa>free_for_read</oa></addata></record> |
fulltext | fulltext |
identifier | ISSN: 0021-972X |
ispartof | The journal of clinical endocrinology and metabolism, 2023-12, Vol.108 (12), p.e1720-e1730 |
issn | 0021-972X 1945-7197 1945-7197 |
language | eng |
recordid | cdi_swepub_primary_oai_swepub_ki_se_668336 |
source | Oxford University Press Journals All Titles (1996-Current); MEDLINE; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Alma/SFX Local Collection; SWEPUB Freely available online |
subjects | Acute lymphoblastic leukemia Adenocarcinoma Adolescent Adult Age Appetite loss Autoimmune diseases Body weight loss Cancer Cancer screening Child Chromosome 15 Chromosome deletion Chromosomes Colon cancer Colorectal cancer Diagnosis Fathers Genes Health aspects Humans Hyperphagia Hypothalamus Life span Literature reviews Lymphatic leukemia Malignancy Medical records Medical screening Melanoma Middle Aged Neurodevelopmental disorders Obesity Paraneoplastic syndrome Patients Prader-Willi syndrome Prader-Willi Syndrome - complications Prader-Willi Syndrome - diagnosis Prader-Willi Syndrome - epidemiology Retrospective Studies Risk factors Weight control Young Adult |
title | Malignancies in Prader-Willi Syndrome: Results From a Large International Cohort and Literature Review |
url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-06T16%3A05%3A26IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_swepu&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Malignancies%20in%20Prader-Willi%20Syndrome:%20Results%20From%20a%20Large%20International%20Cohort%20and%20Literature%20Review&rft.jtitle=The%20journal%20of%20clinical%20endocrinology%20and%20metabolism&rft.au=Pellikaan,%20Karlijn&rft.date=2023-12-01&rft.volume=108&rft.issue=12&rft.spage=e1720&rft.epage=e1730&rft.pages=e1720-e1730&rft.issn=0021-972X&rft.eissn=1945-7197&rft_id=info:doi/10.1210/clinem/dgad312&rft_dat=%3Cgale_swepu%3EA775958545%3C/gale_swepu%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2955301800&rft_id=info:pmid/37267430&rft_galeid=A775958545&rft_oup_id=10.1210/clinem/dgad312&rfr_iscdi=true |