[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-337744-105":59,"doc-detail-337744-en":130},{"code":4,"msg":5,"data":6},0,"success",[7,13,18,23,28,33,38,43,48,51,55],{"id":8,"doc_module":4,"doc_module_name":9,"category_name":10,"show_sort_weight":11,"slug":12},1,"Document","Story & Novel",90,"story-novel",{"id":14,"doc_module":4,"doc_module_name":9,"category_name":15,"show_sort_weight":16,"slug":17},2,"Literature",80,"literature",{"id":19,"doc_module":4,"doc_module_name":9,"category_name":20,"show_sort_weight":21,"slug":22},4,"Exam",70,"exam",{"id":24,"doc_module":4,"doc_module_name":9,"category_name":25,"show_sort_weight":26,"slug":27},5,"Comic",60,"comic",{"id":29,"doc_module":4,"doc_module_name":9,"category_name":30,"show_sort_weight":31,"slug":32},6,"Technology",50,"technology",{"id":34,"doc_module":4,"doc_module_name":9,"category_name":35,"show_sort_weight":36,"slug":37},7,"Healthcare",40,"healthcare",{"id":39,"doc_module":4,"doc_module_name":9,"category_name":40,"show_sort_weight":41,"slug":42},8,"Research & Report",30,"research-report",{"id":44,"doc_module":4,"doc_module_name":9,"category_name":45,"show_sort_weight":46,"slug":47},9,"Religion & Spirituality",20,"religion-spirituality",{"id":46,"doc_module":4,"doc_module_name":9,"category_name":49,"show_sort_weight":46,"slug":50},"World Cup","world-cup",{"id":52,"doc_module":4,"doc_module_name":9,"category_name":53,"show_sort_weight":52,"slug":54},10,"Lifestyle","lifestyle",{"id":56,"doc_module":4,"doc_module_name":9,"category_name":57,"show_sort_weight":24,"slug":58},19,"General","general",{"code":4,"msg":60,"data":61},"ok",{"site_id":62,"language":63,"slug":64,"title":65,"keywords":66,"description":67,"schema_data":68,"social_meta":123,"head_meta":125,"extra_data":127,"updated_unix":129},105,"en","li-fraumeni-syndrome-with-six-primary-tumors-case-report","Li-Fraumeni Syndrome With Six Primary Tumors - Case Report","","Li-Fraumeni syndrome (LFS) is a hereditary cancer predisposition driven by pathogenic germline TP53 mutations and characterized by a broad, diverse clinical spectrum. This case report describes a patient who developed six asynchronous primary tumors by age 33: a phyllode tumor of the breast, pheochromocytoma, rosette-forming glioneuronal tumor (RGNT), adrenocortical carcinoma (ACC), a ductal carcinoma of the breast, and a thymoma. The report highlights that omission of pretest genetic counseling and detailed family tree analysis contributed to overlooking an underlying likely pathogenic TP53 variant (Class 4) with implications for patient management and family risk assessment.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/li-fraumeni-syndrome-with-six-primary-tumors-case-report/337744/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/li-fraumeni-syndrome-with-six-primary-tumors-case-report/337744.png","ImageObject",300,407,{"name":92,"@type":93},"Eliana","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-29","2026-09-21",true,{"@type":102,"interactionType":103,"userInteractionCount":52},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What is Li-Fraumeni syndrome and what causes it?","Question",{"text":112,"@type":113},"Li-Fraumeni syndrome is a hereditary cancer predisposition syndrome associated with high lifetime risk caused by pathogenic germline TP53 mutations.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"What primary tumors were reported in this patient?",{"text":117,"@type":113},"By age 33, the patient developed six asynchronous tumors: a breast phyllode tumor, pheochromocytoma, rosette-forming glioneuronal tumor (RGNT), adrenocortical carcinoma (ACC), a breast ductal carcinoma, and a thymoma.",{"name":119,"@type":110,"acceptedAnswer":120},"How did genetic counseling affect the outcome in this case?",{"text":121,"@type":113},"The report states that omission of pretest genetic counseling and thorough family tree analysis before testing led to oversight of an underlying TP53 likely pathogenic mutation (Class 4), which could affect treatment and family risk considerations.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},337744,1790626274,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":52,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":34,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":144},4398048949847,"https://ap-avatar.wpscdn.com/avatar/400002536579ef2da7f?_k=1778318612642679267","Hindawi  \nCase Reports in Oncological Medicine Volume 2024, Article ID 6699698, 7 pages [https://doi.org/10.1155/2024/6699698](https://doi.org/10.1155/2024/6699698)  \nCase Report  \nLi-Fraumeni Syndrome With Six Primary Tumors—Case Report  \nDejan Stojiljković, 1,2 Ana Cvetković,2,3 Andrej Jokić,3 Dijana Mirčić,3 Sanja Mihajlović, 1 Ana Krivokuća,4 Marija Đorđić Crnogorac,4 and Lazar Glisic 5  \n1Department of Surgery, Surgical Oncology Clinic, Institute for Oncology and Radiology of Serbia, Belgrade, Serbia  \n2Faculty of Medicine, University of Belgrade, Belgrade, Serbia  \n3Department of Anesthesiology With Reanimatology and Intensive Care Unit, Institute for Oncology and Radiology of Serbia, Belgrade, Serbia  \n4Department for Experimental Research and Genetics, Institute for Oncology and Radiology of Serbia, Belgrade, Serbia 5Department of Obstetrics and Gynecology, University Clinic Ulm, Faculty of Medicine, University of Ulm, Ulm, Germany  \nCorrespondence should be addressed to Lazar Glisic; [lazarglisic@hotmail.com](lazarglisic@hotmail.com)  \nReceived 10 November 2023; Revised 6 March 2024; Accepted 23 March 2024; Published 10 May 2024  \nAcademic Editor: Nagaraju Mupparapu  \nCopyright © 2024 Dejan Stojiljković et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.  \nLi-Fraumeni syndrome (LFS) is a cancer predisposition syndrome associated with a high, lifetime risk of a broad spectrum of cancers caused by pathogenic germline TP53 mutations. Numerous different germline TP53 mutations have been associated with LFS, which has an exceptionally diverse clinical spectrum in terms of tumor type and age of onset. Our patient has developed six asynchronous tumors to date: a phyllode tumor of the breast, a pheochromocytoma, a rosette-forming glioneuronal tumor (RGNT), an adrenocortical carcinoma (ACC), a ductal carcinoma of the breast, and a thymoma. The occurrence of such a number of rare tumors is sporadic even among in the population of patients living with cancer predisposition syndromes. In this instance, the omission of pretest genetic counseling and thorough family tree analysis prior to selecting the test led to the oversight of an underlying TP53 likely pathogenic mutation (classified as Class 4) . This emphasizes the necessity for such counseling to prevent overlooking crucial genetic information. Neglecting this step could have had profound implications on the patient’s treatment, particularly considering the early onset and occurrence of multiple tumors, which typically raise suspicion of a hereditary component. The implications for family members must be considered.  \nKeywords: case report; Li-Fraumeni; mitotane; p53  \n1. Introduction  \nMultiple primary neoplasms (MPNs) are defined as two or more primary neoplasms in which each tumor is not an extension, recurrence, or metastasis of the other. They are rare in the general population and are sporadically described in case reports. Our review of the literature found only a few patients with three or more neoplasms [1–3] . In recent years, as the life expectancy of cancer patients has increased due to better diagnostic and therapeutic methods, the incidence of MPNs has been increased. MPNs are most common in syndromes associated with genetic disorders. Li-Fraumeni syndrome (LFS) is extremely rare; it is estimated that itaffects 1 in 5000 to 1 in 20,000 families [3, 4] . The incidence of suspected TP53 mutation ranges from 1 in 3555 to 1 in  \n5476 individuals, but the number is unknown due to a lack of genetic screening [5] . Here, we present our case of a patient with LFS who developed six primary tumors by the age of 33 years.  \n2. Case Description  \nIn September 2005, at the age of 17, the patient started oncology treatment when she was hospitalized for a large left breast tumor and underwent tumorectomy. Phyllod","cbCaiv6vWPQROYkv","https://ap.wps.com/l/cbCaiv6vWPQROYkv","pdf",1628399,"English","# Introduction\n## Multiple primary neoplasms and LFS background\n# Case Description\n## Initial breast tumor and subsequent surgeries\n## Pheochromocytoma and genetic testing (BRCA1)\n## Neurological symptoms and RGNT diagnosis\n## Adrenal findings and ongoing genetic implications","[{\"question\":\"What is Li-Fraumeni syndrome and what causes it?\",\"answer\":\"Li-Fraumeni syndrome is a hereditary cancer predisposition syndrome associated with high lifetime risk caused by pathogenic germline TP53 mutations.\"},{\"question\":\"What primary tumors were reported in this patient?\",\"answer\":\"By age 33, the patient developed six asynchronous tumors: a breast phyllode tumor, pheochromocytoma, rosette-forming glioneuronal tumor (RGNT), adrenocortical carcinoma (ACC), a breast ductal carcinoma, and a thymoma.\"},{\"question\":\"How did genetic counseling affect the outcome in this case?\",\"answer\":\"The report states that omission of pretest genetic counseling and thorough family tree analysis before testing led to oversight of an underlying TP53 likely pathogenic mutation (Class 4), which could affect treatment and family risk considerations.\"}]","Li-Fraumeni Syndrome With Six Primary Tumors - Case Report | PDF",1790020453,18]