{"id":2986,"date":"2018-12-24T05:05:01","date_gmt":"2018-12-24T05:05:01","guid":{"rendered":"https:\/\/www.mybiosource.com\/learn\/?p=2986"},"modified":"2024-04-04T07:55:18","modified_gmt":"2024-04-04T07:55:18","slug":"rare-cancers-can-they-be-a-torchbearer-for-common-cancer","status":"publish","type":"post","link":"https:\/\/www.mybiosource.com\/learn\/rare-cancers-can-they-be-a-torchbearer-for-common-cancer\/","title":{"rendered":"Rare Cancers \u2013 Can they be a torchbearer for common cancer?"},"content":{"rendered":"<table style=\"background-color: #c9c7c7;\">\n<tbody>\n<tr>\n<td width=\"623\">\n<h3 style=\"padding-left: 200px;\"><strong><u>Table of Content<\/u><\/strong><\/h3>\n<p><a href=\"#1\"><strong>I. Introduction<\/strong><\/a><br \/>\n<a href=\"#2\"><strong>II. Types of Rare Cancers<\/strong><\/a><br \/>\n\u2022 Head and neck cancer<br \/>\n\u2022 Sarcoma<br \/>\n\u2022 Thyroid cancer<br \/>\n\u2022 Neuroendocrine cancer<br \/>\n\u2022 Brain tumors<br \/>\n\u2022 Lymphoma<br \/>\n\u2022 Pediatric cancer<br \/>\n<a href=\"#3\"><strong>III. Poorer Survival Rates for Rare Cancer Patients<\/strong><\/a><br \/>\n\u2022 Reasons for poorer prognosis<br \/>\n\u2022 Learning from research on rare cancers<br \/>\n<a href=\"#4\"><strong>IV. Improving Diagnosis, Treatment, and Outcome<\/strong><\/a><br \/>\n\u2022 The need for translational research, clinical studies, and population-based research<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p id=\"1\">Normal cells only proliferate when stimulated by an appropriate <span id=\"urn:enhancement-da1b10c6-e582-48be-81ed-4ac55b0e0703\" class=\"textannotation disambiguated wl-thing\">growth factor<\/span> and regulated by inhibitory signals. Cancer cells become increasingly independent of the start and stop signals. The net result is characteristic uncontrolled growth of cancer cells which behave as autonomous individuals rather than as integrated components of the body. They are also characterized by their ability to invade surrounding tissues and <span id=\"urn:enhancement-4ae21d99-9094-4f4e-a347-5a4bd7b98325\" class=\"textannotation disambiguated wl-thing\">metastasize<\/span> throughout the body. The movement of cancer cells are not inhibited by contact with other cells. By now 200 forms of cancer are known including <span id=\"urn:enhancement-fc5ca46f-0de9-4883-baf6-f1442b0da3c4\" class=\"textannotation disambiguated wl-thing\">leukemias<\/span> and lymphomas and among them, some are common, such as breast, bowel, <span id=\"urn:enhancement-8037ea49-68e5-4d3d-a49d-5c58a0bcc3ae\" class=\"textannotation disambiguated wl-thing\">prostate<\/span> and lung cancer, some types are uncommon and some are rare. Rare cancer starts in an unusual place in the body or an unusual type and\u00a0needs special treatment. According to a recent\u00a0analysis by European Medicines Agency (EMA)\u00a0rare cancers are identified as those with an incidence of less than six per 100 000 persons per year. Less common cancers have between six and 12 incidences per year per 100 000 population.\u00a0<em>According to the\u00a0Radboud Institute for Molecular Life Sciences\u00a0(RIMLS), rare cancers represent 22% of all cancers cases. <\/em><\/p>\n<p id=\"2\">Till now following seven types of rare cancers have been identified &#8211;<\/p>\n<ol>\n<li><strong> Head and neck cancer<\/strong> \u2013 They usually begin in the squamous cells that line the <span id=\"urn:enhancement-7f1a6eda-fb62-4382-a683-4f76c9e403f0\" class=\"textannotation disambiguated wl-thing\">mucosal surfaces<\/span> inside the head and neck (e.g. mouth, the nose and throat) and categorized by the area where they begin, e.g. the oral cavity, <span id=\"urn:enhancement-a7789727-4702-4ebd-8fe6-543cec66d5c6\" class=\"textannotation disambiguated wl-thing\">pharynx<\/span>, <span id=\"urn:enhancement-7b569a87-3d05-4230-9500-f2d8980c83b8\" class=\"textannotation disambiguated wl-thing\">larynx<\/span>, paranasal <span id=\"urn:enhancement-48174b78-9340-47be-800d-60c0470d8ff3\" class=\"textannotation disambiguated wl-thing\">sinuses<\/span> and <span id=\"urn:enhancement-1599f81e-c2fc-4205-a6f3-7f273ec1a867\" class=\"textannotation disambiguated wl-thing\">nasal cavity<\/span> and <span id=\"urn:enhancement-2df72add-767b-463f-80bf-f717fc0b3c0e\" class=\"textannotation disambiguated wl-thing\">salivary glands<\/span>.<\/li>\n<li><strong><span id=\"urn:enhancement-c651617e-624f-4810-ad36-ef41accdfd4b\" class=\"textannotation disambiguated wl-thing\">Sarcoma<\/span> &#8211; <\/strong>It arises in the connective tissue of the body, i.e. fat, blood vessels, <span id=\"urn:enhancement-4488d8b5-2f6d-4825-85fb-c69a65bd4c91\" class=\"textannotation disambiguated wl-thing\">nerves<\/span>, bones, muscles, deep skin tissues, and cartilage.<\/li>\n<li><strong><span id=\"urn:enhancement-d8869078-c15f-429a-b0dd-c817843650b3\" class=\"textannotation disambiguated wl-thing\">Thyroid cancer<\/span> \u2013 <\/strong>It affects the <span id=\"urn:enhancement-0e0b5533-30f2-4ba8-8dae-6f6e8aadaec6\" class=\"textannotation disambiguated wl-thing\">thyroid<\/span> gland, a small gland at the base of the neck that produces hormones. Symptoms include a painless lump or swelling in the front of the neck, swollen glands in the neck, unexplained hoarseness, a lingering sore throat and difficulty swallowing.<\/li>\n<li><strong> Neuroendocrine cancer \u2013 <\/strong>This cancer\u00a0begins in the <span id=\"urn:enhancement-e6fde30c-e2b7-4515-9fc4-7790b3abfbed\" class=\"textannotation disambiguated wl-thing\">neuroendocrine<\/span> system, which is the hormone-producing cells of the body. Neuroendocrine cells are found in organs like the lungs and <span id=\"urn:enhancement-490fb55c-bb84-4837-961d-699fda618a3d\" class=\"textannotation disambiguated wl-thing\">gastrointestinal<\/span> tract, including the stomach and intestines.<\/li>\n<li><strong> Brain tumors \u2013 <\/strong>These are\u00a0high-grade tumors that either primary or secondary depending on the location. Tumors starting in the brain are primary tumours whereas spread into the brain from elsewhere are secondary tumors. Symptoms include severe, persistent headaches, <span id=\"urn:enhancement-835f100a-6814-448d-bc30-ecccde70cee4\" class=\"textannotation disambiguated wl-thing\">seizures<\/span>, persistent nausea, vomiting and drowsiness, mental or behavioral changes, vision or speech problems, progressive weakness or paralysis on one side of the body.<\/li>\n<li><strong>Lymphoma &#8211; <\/strong>It occurs when lymphocytes (white blood cells) divide in an abnormal way\u00a0or don\u2019t die when they\u00a0should. Abnormal <span id=\"urn:enhancement-eb7a9122-db56-4b91-9a7a-9eff7d83ae31\" class=\"textannotation disambiguated wl-thing\">lymphocytes<\/span> can collect in <span id=\"urn:enhancement-1ea0face-d39c-4988-9842-ca6d79237dda\" class=\"textannotation disambiguated wl-thing\">lymph nodes<\/span>, often in armpits, neck or groin, almost any part of the body. Symptoms will depend on where the <span id=\"urn:enhancement-dd94d7bb-18d9-48fd-94d3-c8a580edbd9d\" class=\"textannotation disambiguated wl-thing\">lymphoma<\/span> starts and what parts of the body is affected.<\/li>\n<li><b><span id=\"urn:enhancement-80c7f3d8-f0cb-4ce6-88bc-8539d9abffb5\" class=\"textannotation disambiguated wl-thing\">Pediatric<\/span>\u00a0(childhood) <\/b>cancer<b> &#8211; <\/b>The most common cancers in children include <span id=\"urn:enhancement-060c4064-529a-4f9b-9eac-a756677bb01e\" class=\"textannotation disambiguated wl-thing\">leukemia<\/span>, lymphoma, and brain cancer. Things that cause cancer in adults (e.g. smoking or exposure to environmental toxins) are usually not the same as in children. In most cases, childhood cancers come from random <span id=\"urn:enhancement-713051be-05fc-4560-8423-482bac30e55b\" class=\"textannotation disambiguated wl-creative-work\">mutations<\/span> in the genes of growing cells, which means that there\u2019s often no effective way to prevent them.<\/li>\n<\/ol>\n<p id=\"3\">Patients with rare cancer have a 15% poorer overall survival rate than patients with common cancer. This can be attributed to the fact that the level of prognosis achieved for common cancer has not been achieved in rare cancers. Numerous reasons can be cited for the same. Late or incorrect diagnosis due to limited awareness by patients and general practitioners, Lack of access to appropriate therapies and clinical expertise, Relatively small number of clinical trials due to the small number of patients, Market limitations, Few available registries, and tissue banks and lastly low on the political agenda of the international community.<\/p>\n<p id=\"4\">However, we can learn a lot from research on rare diseases and rare cancers. For example, Fanconi anemia research in 2010 has provided insights into disease mechanisms of <span id=\"urn:enhancement-174c32a3-07e1-4ce5-b8d6-363947aac0eb\" class=\"textannotation disambiguated wl-thing\">bone marrow<\/span> failure, cancer, and the resistance to <span id=\"urn:enhancement-0e1e8c11-4bff-4877-8c33-a00483900a35\" class=\"textannotation disambiguated wl-thing\">chemotherapy<\/span>. A rare <span id=\"urn:enhancement-8c1ca529-e870-44a8-b7a4-ba9ca875b52d\" class=\"textannotation disambiguated wl-thing\">pediatric<\/span> cancer, Wilms\u2019 tumor, a has been referred to as a \u201cmodel\u201d for understanding the genetics, epigenetics, and <span id=\"urn:enhancement-fe841dc1-6c71-4d18-8949-ed7441a3de24\" class=\"textannotation disambiguated wl-thing\">molecular biology<\/span> of <span id=\"urn:enhancement-de71983d-fd71-42a6-a749-c8f13893d20c\" class=\"textannotation disambiguated wl-thing\">pediatric<\/span> cancers and cancers generally. IMATINIB for the treatment of <span id=\"urn:enhancement-a5eb579b-44c3-429c-9587-c4e2170b350f\" class=\"textannotation disambiguated wl-thing\">chronic myeloid leukemia<\/span> (CML), <span id=\"urn:enhancement-da65402d-dc99-4c50-b2e0-7b1b94698b43\" class=\"textannotation disambiguated wl-thing\">seminoma<\/span>, and <span id=\"urn:enhancement-94da296a-49b7-4c8f-bbaa-d434a417a2a6\" class=\"textannotation disambiguated wl-thing\">gastrointestinal<\/span> stromal tumor (GIST) has set \u201ca new paradigm for the treatment of cancer with molecularly targeted therapies\u201d. It has also prompted a lot of additional research on tyrosine kinase inhibitors. Researchers at the U.S. National Institutes of Health (<span id=\"urn:enhancement-53db9ece-732c-478e-b5c0-7a11d80fe5e3\" class=\"textannotation disambiguated wl-organization\">NIH<\/span>), who analysed the tissue of patients affected by <span id=\"urn:enhancement-4cb0fc60-a521-4a5e-81e7-47f800a5e34b\" class=\"textannotation disambiguated wl-thing\">paraganglioma<\/span>, a rare <span id=\"urn:enhancement-07487520-e28d-470b-a87a-1e7c88c08dd1\" class=\"textannotation disambiguated wl-creative-work\">endocrine<\/span> tumor, recently discovered a genetic mutation that appears to increase the production of red blood cells in tumors. Such type of findings may have an impact on the understanding and treatment of several other cancers. A large share of research funding goes into the most common cancers (breast, <span id=\"urn:enhancement-aed8a455-c1ba-4199-af7f-9189aefdbca1\" class=\"textannotation disambiguated wl-thing\">colon<\/span>, lung, and <span id=\"urn:enhancement-cbc5dbc6-d1e8-4663-895d-b3a9dec1a0cd\" class=\"textannotation disambiguated wl-thing\">prostate<\/span>) and also more patients are represented in a <span id=\"urn:enhancement-5991e688-4bd1-456e-934e-62c45249b525\" class=\"textannotation disambiguated wl-thing\">clinical trial<\/span> for them. It would, therefore, be rational to anticipate that the most significant therapeutic advances are made in common cancers. However, a recent study reports that novel cancer treatments have their most striking effects in rare cancers, suggesting that \u201c<em>only by stratifying the common tumors, especially when using targeted agents, into the molecular subsets of diseases that compose them are we likely to achieve a substantial effect in these disorders<\/em>.\u201d  To improve diagnosis, treatment and, outcome of rare cancers, <span id=\"urn:enhancement-b47f64ed-f648-466e-aed4-8c4b5b799573\" class=\"textannotation disambiguated wl-thing\">translational research<\/span>, clinical studies, and population-based research are essential. This can be achieved only by collaboration between (inter) national partners and patient advocacy groups.<\/p>\n<p><strong>References\u00a0<\/strong><\/p>\n<ol>\n<li>D&#8217;Andrea AD. Susceptibility pathways in Fanconi&#8217;s anemia and breast cancer. N Engl J Med.2010\u00a0May\u00a020;362(20):1909-19. doi: 10.1056\/NEJMra0809889.<\/li>\n<li>Feinberg AP1,\u00a0Williams BR. Wilms&#8217; tumor as a model for cancer biology. Methods Mol Biol.2003;222:239-48.<\/li>\n<li>Eck MJ1,\u00a0Manley PW. The interplay of structural information and functional studies in kinase drug design: insights from BCR-Abl. Curr Opin Cell Biol.2009\u00a0Apr;21(2):288-95.<\/li>\n<li>M\u00fcller BA Imatinib and its successors&#8211;how modern chemistry has changed drug development. Curr Pharm Des.2009;15(2):120-33.<\/li>\n<li>Braiteh F,\u00a0Kurzrock R. Uncommon tumors and exceptional therapies: paradox or paradigm? Mol Cancer Ther.2007 Apr;6(4):1175-9.<\/li>\n<\/ol>\n","protected":false},"excerpt":{"rendered":"<p>Table of Content I. Introduction II. Types of Rare Cancers \u2022 Head and neck cancer \u2022 Sarcoma \u2022 Thyroid cancer \u2022 Neuroendocrine cancer \u2022 Brain tumors \u2022 Lymphoma \u2022 Pediatric cancer III. Poorer Survival Rates for Rare Cancer Patients \u2022 Reasons for poorer prognosis \u2022 Learning from research on rare cancers IV. Improving Diagnosis, Treatment, [&hellip;]<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_et_pb_use_builder":"off","_et_pb_old_content":"","_et_gb_content_width":"","footnotes":""},"categories":[31],"tags":[85],"class_list":["post-2986","post","type-post","status-publish","format-standard","hentry","category-news","tag-cancers"],"_links":{"self":[{"href":"https:\/\/www.mybiosource.com\/learn\/wp-json\/wp\/v2\/posts\/2986","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.mybiosource.com\/learn\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.mybiosource.com\/learn\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.mybiosource.com\/learn\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/www.mybiosource.com\/learn\/wp-json\/wp\/v2\/comments?post=2986"}],"version-history":[{"count":2,"href":"https:\/\/www.mybiosource.com\/learn\/wp-json\/wp\/v2\/posts\/2986\/revisions"}],"predecessor-version":[{"id":9321,"href":"https:\/\/www.mybiosource.com\/learn\/wp-json\/wp\/v2\/posts\/2986\/revisions\/9321"}],"wp:attachment":[{"href":"https:\/\/www.mybiosource.com\/learn\/wp-json\/wp\/v2\/media?parent=2986"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.mybiosource.com\/learn\/wp-json\/wp\/v2\/categories?post=2986"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.mybiosource.com\/learn\/wp-json\/wp\/v2\/tags?post=2986"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}