{"id":26976,"date":"2021-04-20T12:00:00","date_gmt":"2021-04-20T12:00:00","guid":{"rendered":"https:\/\/www.cancer.gov\/news-events\/cancer-currents-blog\/2021\/nf1-associated-with-more-cancer-types"},"modified":"2024-11-03T15:14:58","modified_gmt":"2024-11-03T15:14:58","slug":"nf1-associated-with-more-cancer-types-than-previously-known","status":"publish","type":"post","link":"https:\/\/ogkologos.com\/en\/nf1-associated-with-more-cancer-types-than-previously-known\/","title":{"rendered":"NF1 Associated with More Cancer Types Than Previously Known"},"content":{"rendered":"

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The inherited condition neurofibromatosis type 1 is caused by changes in a gene called NF1<\/em>, which is found on chromosome 17.<\/p>\n

Credit: DermNetNZ CC BY-NC-ND 3.0 NZ<\/p>\n<\/div>\n<\/figcaption><\/figure>\n<\/div>\n

An inherited condition called neurofibromatosis type 1 (NF1) is linked to the development of more types of cancer than previously realized, according to results from a new study. People with NF1<\/a> also have a greater chance of dying from some cancer types than people without the condition, the study found. These include some rare types of sarcoma<\/a>, as well as ovarian cancer and melanoma.<\/p>\n

The hallmark of NF1 is the development of neurofibromas, which are tumors that develop from the cells and tissues that cover nerves. While neurofibromas are usually benign\u2014that is, they tend not to spread to other places in the body\u2014they can cause serious health problems as they grow, including pain and damage to surrounding tissues.<\/p>\n

People with NF1 are also known to have a higher risk of cancerous tumors, including a sarcoma called malignant peripheral nerve sheath tumor<\/a> (MPNST), brain cancer, and breast cancer, than people without the condition. Researchers have estimated that such malignant cancers decrease the life expectancy of people with NF1 by an average of 10 to 15 years. But because NF1 itself is relatively rare, researchers have struggled to assemble a comprehensive picture of these cancer risks. <\/p>\n

\u201cWe\u2019ve known that people with NF1 are at a greater risk of developing certain cancers, and that they have a genetic predisposition<\/a> to developing these cancers earlier [in life],\u201d said Brigitte Widemann, M.D., chief of NCI\u2019s Pediatric Oncology Branch<\/a>, who was not involved with the study.<\/p>\n

\u201cThis study shows that there are some other cancers that we didn\u2019t know are associated with NF1, and also that the outcomes of these cancers may be worse\u201d than in people without the condition, Dr. Widemann added. \u201cThis is important for patients to know.\u201d<\/p>\n

\u201cThese findings won\u2019t change the way patients with NF1 are treated for any cancers they may develop, but they emphasize the importance of preventive measures and early diagnosis,\u201d said Keila Torres, M.D., Ph.D., of the University of Texas MD Anderson Cancer Center, who led the research. \u201cWe strongly recommend that patients with a diagnosis of NF1 be referred to a neurofibromatosis specialist or neurologist<\/a>\u201d who would know about their increased cancer risk, she added.<\/p>\n

Results from the new study<\/a>, which was funded in part by NCI, were published March 18 in JAMA Network Open<\/em>.<\/p>\n

One Gene, Many Changes<\/h2>\n

NF1 is caused by inherited changes in the NF1<\/em> gene, which provides instructions for making a protein called neurofibromin. The symptoms and the course of NF1 can vary widely. Most people with NF1 are diagnosed within their first few years of life, while others might not know they have the condition until they reach their 20s, 30s, or even later.<\/p>\n

In addition to developing neurofibromas, people with NF1 often develop coffee-colored skin spots and benign skin tumors, freckling of the skin that is not exposed to the sun, and abnormalities in the bones and central nervous system<\/a>.<\/p>\n

Because NF1 is relatively rare\u2014around 1 in 2,500 to 3,000 people are thought to be living with the condition\u2014it is unlikely for any one hospital or clinic to come across more than a handful of individuals with NF1. <\/p>\n

The size of MD Anderson provided an opportunity to analyze data from a large number of people with NF1 who have been treated there over more than 3 decades to provide a clearer picture of the link between NF1 and different cancer types.<\/p>\n

More Cancers Associated with NF1 Than Previously Known <\/h2>\n

Dr. Torres and her colleagues collected data on about 1,600 people with NF1 treated at MD Anderson between 1985 and 2020. They compared cancer diagnoses and disease-specific survival  for each cancer type in people with NF1 with estimates for the general population taken from NCI\u2019s Surveillance, Epidemiology, and End Results (SEER) Program<\/a> database.<\/p>\n

The median age of those with NF1 at their first visit to MD Anderson was 19 years and ranged from 1 month to 83 years. Overall, people with NF1 were almost 10 times more likely to develop any type of cancer during their lifetime than the general population. <\/p>\n

Some of the results from the new study were similar to those from previous studies. For example, about 40% of the people with NF1 developed tumors other than neurofibromas. The most common were glioma, a type of brain tumor, diagnosed in 295 patients (about 18%) and MPNST, a rare sarcoma, diagnosed in 243 (about 15%). One hundred sixteen people (about 7%) developed more than one type of cancer.<\/p>\n

But several cancers found to be associated with NF1 hadn\u2019t been identified in previous studies. These included several rare sarcomas, neuroendocrine tumors<\/a>, ovarian cancer, and melanoma.<\/p>\n

People with NF1 also developed some cancers at an earlier age and were more likely to die from several cancer types compared with the general population. For example, about 33% of people with NF1 who developed melanoma died from that cancer within 5 years of diagnosis, compared with 8% of people diagnosed with melanoma in the general population. <\/p>\n\n\n\n\n
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Cancers that are more common in people with NF1<\/span><\/span><\/span><\/strong><\/p>\n<\/td>\n

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Cancers occurring at a younger age in people with NF1<\/span><\/span><\/span><\/strong><\/p>\n<\/td>\n

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Cancers that appear to be more deadly in people with NF1<\/span><\/span><\/span><\/strong><\/p>\n<\/td>\n<\/tr>\n

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  • Glioma (low- and high-grade)<\/span><\/span><\/span><\/li>\n
  • Sarcoma (many types)<\/span><\/span><\/span><\/li>\n
  • Breast cancer<\/span><\/span><\/span><\/li>\n
  • Endocrine cancers (including pheochromocytoma and neuroendocrine tumors)<\/span><\/span><\/span><\/li>\n
  • Melanoma<\/span><\/span><\/span><\/li>\n
  • Acute lymphoblastic leukemia<\/span><\/span><\/span><\/li>\n
  • Ovarian cancer<\/span><\/span><\/span><\/li>\n
  • Prostate cancer<\/span><\/span><\/span><\/li>\n
  • Meningioma<\/span><\/span><\/span><\/li>\n<\/ul>\n<\/td>\n
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  • Low-grade glioma<\/span><\/span><\/span><\/li>\n
  • High-grade glioma<\/span><\/span><\/span><\/li>\n
  • MPNST<\/span><\/span><\/span><\/li>\n
  • Breast cancer<\/span><\/span><\/span><\/li>\n<\/ul>\n<\/td>\n
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