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Alongside endocrine neoplasms, these syndromes present with diverse cutaneous manifestations, offering valuable diagnostic clues for early recognition and management. This systematic review and meta\u2010analysis aimed to evaluate and synthesize the dermatologic features associated with these syndromes. Following PRISMA 2020 guidelines, a systematic search of MEDLINE, Cochrane Library, and Embase databases was conducted. Eligible publications included original articles, case reports, and case series with detailed dermatological descriptions of patients with the aforementioned syndromes. Data extraction and risk of bias assessment were performed independently by multiple reviewers. Statistical analyses and data visualization were conducted using program package R. A total of 217 studies comprising 833 patients were included: 276 MEN1, 48 MEN2A, 9 MEN2B, 121 CNC, and 452 PHTS cases. Distinct dermatologic patterns emerged within each syndrome: angiofibromas, collagenomas, and lipomas in MEN1; cutaneous lichen amyloidosis in MEN2A; mucosal neuromas in MEN2B; lentiginosis and cutaneous myxomas in CNC; and trichilemmomas, papillomatous papules, and acral keratoses in PHTS. Melanoma prevalence was 2.2% in PHTS and 2.5% in MEN1 patients, underscoring the need for dermatologic vigilance. This review highlights the role of dermatologic assessment in identifying endocrine tumor syndromes, with cutaneous findings often serving as early, accessible markers of systemic disease. Enhanced awareness of these manifestations can facilitate timely genetic evaluation, cancer surveillance, and multidisciplinary intervention.<\/jats:p>\n                  <jats:p>\n                    <jats:bold>Trial Registration:<\/jats:bold>\n                    PROSPERO registration number: CRD42024558093\n                  <\/jats:p>","DOI":"10.1111\/ijd.70118","type":"journal-article","created":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T10:27:02Z","timestamp":1761820022000},"page":"464-488","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Dermatologic Features of Endocrine Tumor Syndromes\u2014Systematic Review and Meta\u2010Analysis"],"prefix":"10.1111","volume":"65","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9614-4724","authenticated-orcid":false,"given":"S\u00e1ra","family":"P\u00e1lla","sequence":"first","affiliation":[{"name":"Department of Dermatology, Venereology and Dermatooncology Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zseraldin","family":"Metyovinyi","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Dermatooncology Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fanni Ad\u00e9l","family":"Meznerics","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Dermatooncology Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3281-8435","authenticated-orcid":false,"given":"Arash","family":"Mirzahosseini","sequence":"additional","affiliation":[{"name":"Department of Pharmaceutical Chemistry Semmelweis University  Budapest Hungary"},{"name":"Center for Pharmacology and Drug Research &amp; Development Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judit","family":"T\u0151ke","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Oncology ENETS Center of Excellence, Faculty of Medicine Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikl\u00f3s","family":"T\u00f3th","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Oncology ENETS Center of Excellence, Faculty of Medicine Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henriett","family":"Butz","sequence":"additional","affiliation":[{"name":"Department of Molecular Genetics and the National Tumor Biology Laboratory National Institute of Oncology  Budapest Hungary"},{"name":"Department of Oncology Biobank National Institute of Oncology  Budapest Hungary"},{"name":"Hungarian Research Network HUN\u2010REN\u2010OOI\u2010TTK\u2010HCEMM Oncogenomics Research Group  Budapest Hungary"},{"name":"Department of Laboratory Medicine Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Attila","family":"Pat\u00f3cs","sequence":"additional","affiliation":[{"name":"Department of Molecular Genetics and the National Tumor Biology Laboratory National Institute of Oncology  Budapest Hungary"},{"name":"Hungarian Research Network HUN\u2010REN\u2010OOI\u2010TTK\u2010HCEMM Oncogenomics Research Group  Budapest Hungary"},{"name":"Department of Laboratory Medicine Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e1rta","family":"Medvecz","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Dermatooncology Semmelweis University  Budapest Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,10,30]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2000.104681"},{"key":"e_1_2_9_3_1","article-title":"Dermatologic Manifestations of Endocrine Disorders","volume":"13","author":"Raj R.","year":"2021","journal-title":"Cureus"},{"key":"e_1_2_9_4_1","unstructured":"L.YehiaandC.Eng \u201cPTEN Hamartoma Tumor Syndrome \u201dGeneReviews1993\u20132025."},{"key":"e_1_2_9_5_1","unstructured":"C. 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