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Today, the routine use of comprehensive germline panels has shifted the focus from solely pathogenic\n                    <jats:italic>BRCA1\/2<\/jats:italic>\n                    variants to include numerous pathogenic variants of other high- and moderate-risk genes. A broad spectrum of genetic alterations has been identified as causative for Hereditary Breast and Ovarian Cancer syndrome\u00a0(HBOC), encompassing not only\n                    <jats:italic>BRCA1<\/jats:italic>\n                    and\n                    <jats:italic>BRCA2<\/jats:italic>\n                    , but also\n                    <jats:italic>PALB2<\/jats:italic>\n                    ,\n                    <jats:italic>ATM<\/jats:italic>\n                    ,\n                    <jats:italic>BARD1<\/jats:italic>\n                    ,\n                    <jats:italic>CHEK2<\/jats:italic>\n                    ,\n                    <jats:italic>BRIP1<\/jats:italic>\n                    ,\n                    <jats:italic>RAD51C<\/jats:italic>\n                    , and\n                    <jats:italic>RAD51D<\/jats:italic>\n                    . Beyond HBOC, numerous additional hereditary tumor syndromes are of significance in senologic and\/or gynecologic oncology, including Li-Fraumeni syndrome, Lynch syndrome,\n                    <jats:italic>DICER1<\/jats:italic>\n                    syndrome, Hereditary Diffuse Gastric Cancer, Neurofibromatosis type 1, Peutz-Jeghers syndrome,\n                    <jats:italic>PTEN<\/jats:italic>\n                    hamartoma tumor syndrome, Tuberous Sclerosis, and pathogenic variants in\n                    <jats:italic>NBN<\/jats:italic>\n                    and\n                    <jats:italic>SMARCA4<\/jats:italic>\n                    . Affected individuals are offered specialized surveillance to enable early detection or even prevention of cancer. In addition to regular clinical examinations and imaging, preventive strategies may include risk-reducing surgery. Pathogenic germline variants also influence therapeutic management of cancer patients. For specific indications, targeted therapies are available, for example PARP [poly (ADP-ribose) polymerase] inhibitors for pathogenic\n                    <jats:italic>BRCA<\/jats:italic>\n                    variant carriers across multiple tumor entities. Optimal management requires interdisciplinary coordination, encompassing genetic counseling, early detection, and risk-reducing strategies within specialized centers. This review provides a comprehensive overview of hereditary tumor syndromes predisposing to breast and gynecologic malignancies, with a focus on genetic basis, associated cancer risks, and implications for clinical management. By delineating these syndromes, it aims to assist clinicians in recognizing hereditary cancer predisposition and in guiding affected individuals within routine senologic and gynecologic practice.\n                  <\/jats:p>","DOI":"10.1007\/s00404-025-08270-6","type":"journal-article","created":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T15:51:22Z","timestamp":1768319482000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Genetic tumor syndromes in female cancer: insights into inherited cancer predisposition and clinical implications"],"prefix":"10.1007","volume":"313","author":[{"given":"Annika","family":"Kr\u00fcckel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Gocke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuel","family":"H\u00f6rner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katharina","family":"Keller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolin","family":"M\u00fcller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lena","family":"Br\u00fcckner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"Heindl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolin C.","family":"Hack","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias W.","family":"Beckmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niklas","family":"Amann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,1,13]]},"reference":[{"key":"8270_CR1","doi-asserted-by":"publisher","first-page":"199","DOI":"10.1159\/000516376","volume":"17","author":"K Rhiem","year":"2022","unstructured":"Rhiem K, Auber B, Briest S et al (2022) Consensus recommendations of the German consortium for hereditary breast and ovarian cancer. 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NA also received support for attending meetings from AstraZeneca, Novartis, Pfizer. LB received lecture fees from Gilead, as well as support for attending meetings from Lilly, Gilead and AstraZeneca. CCH received honoraria from AstraZeneca, Daiichi Sankyo, Eisai, Novartis, Pfizer, Roche, Gilead and MSD as well as support for attending meetings from Daiichi Sankyo. MH received research support from Helsinn Healthcare SA, honoraria from Lilly Deutschland GmbH and travel support from Novartis, Lilly Deutschland GmbH, and AstraZeneca. AK received lecture fees from Gilead and Novartis, honoria for written scientific work from Georg Thieme Verlag as well as support for attending meetings from Lilly.  CM received honoraria for scientific work from Novartis.  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