{"id":623,"date":"2020-02-14T07:19:16","date_gmt":"2020-02-14T07:19:16","guid":{"rendered":"https:\/\/meddists.com\/learn\/?page_id=623"},"modified":"2020-02-14T07:19:16","modified_gmt":"2020-02-14T07:19:16","slug":"peripheral-nerve-sheath-tumors","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/pre-clinical\/pathology\/soft-tissue-tumors\/peripheral-nerve-sheath-tumors\/","title":{"rendered":"Peripheral nerve sheath tumors"},"content":{"rendered":"<span class=\"block-heading\" id=\"header_1\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Definition<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<p class=\"wp-block-paragraph\">Peripheral nerve sheath tumors (or PNSTs) are a rare type of soft tissue tumors arising from the cells located in the sheath of peripheral nerves.<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Characteristics<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<ul class=\"wp-block-list\"><li>Can be either benign or malignant<\/li><li>In most cases, tumor cells show <strong>Schwann cell&nbsp;<\/strong>differentiation<ul><li>Schwann cells are the cells that produce myelin in the CNS<\/li><\/ul><\/li><li>Associated with&nbsp;<strong>neurofibromatosis<\/strong> (NF) <strong>type&nbsp;I and II<\/strong><ul><li>NF-I is characterized by the loss of the tumor suppressor gene&nbsp;<strong>neurofibromin&nbsp;<\/strong>(chromosome 17)<\/li><li>NF-II is characterized by the loss of&nbsp; tumor suppressor gene <strong>merlin <\/strong>(chromosome 22)<ul><li><strong>Caf\u00e9-au-lait&nbsp;<\/strong>macules are a tell-tale sign of NF-II<\/li><li>Meningiomas appear in about 50% of NF-II cases<sup>1<\/sup><\/li><\/ul><\/li><\/ul><\/li><li>More common in adults<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Benign PNSTs<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Schwannomas<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<ul class=\"wp-block-list\"><li>Benign PNSTs composed of neoplastic Schwann cells<\/li><li>Encapsulated<\/li><li>Occur in soft tissues, internal organs, or nerve roots<ul><li>Most commonly affect the vestibular portion of the 8th cranial nerve (vestibulocochlear)<ul><li>Hearing loss<\/li><\/ul><\/li><\/ul><\/li><li>Most are sporadic, 10% are associated with NF-II<\/li><li>Microscopically, seen as mixtures of dense (<strong>Antoni A<\/strong>) and loose (<strong>Antoni B<\/strong>) regions<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_5\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Neurofibromas<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<ul class=\"wp-block-list\"><li>Benign PNSTs composed of multiple cells and structures:<ul><li>Schwann cells<\/li><li>Fibroblasts<\/li><li>Perineurial cells<\/li><li>Mast cells<\/li><li>Axonal processes<\/li><\/ul><\/li><li>There are three important subtypes to know:<ul><li>Localized cutaneous neurofibromas<ul><li>Limited to a small region<\/li><li>Superficial, polypoid tumors<\/li><\/ul><\/li><li>Plexiform neurofibromas<ul><li>Grows in proximity with peripheral nerves and plexuses<\/li><li>&nbsp;Nearly always associated with NF-I<\/li><li>This subtype carries a small risk for malignant transformation<sup>2<\/sup><\/li><\/ul><\/li><li>Diffuse neurofibromas<ul><li>Spreads in a large area, usually causing disfigurement<\/li><li>Superficially, seen as large flaps of excess tissue<\/li><\/ul><\/li><\/ul><\/li><li>Most are sporadic, 10% are associated with NF-I<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_6\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Malignant PNSTs<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_6\">\n\n\n<p class=\"wp-block-paragraph\">Collectively known as <strong>neurofibrosarcomas<\/strong>.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Closely related to NF-I<\/li><li>May arise from malignant transformation of neurofibromas (plexiform subtype, as mentioned above)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_7\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Traumatic neuroma<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_7\">\n\n\n<p class=\"wp-block-paragraph\">A non-neoplastic proliferation seen as a response to severe trauma of the nerve (transection).<\/p>\n\n\n\n<ol class=\"wp-block-list\"><li>Goutagny S, Kalamarides M. Meningiomas and neurofibromatosis. <em>J Neurooncol<\/em>. 2010;99(3):341-347. <a rel=\"noreferrer noopener\" href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/20714782\" target=\"_blank\">https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/20714782<\/a>.2.Mautner V, Friedrich R, von D, et al. Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma. <em>Neuroradiology<\/em>. 2003;45(9):618-625. <a rel=\"noreferrer noopener\" href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/12898075\" target=\"_blank\">https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/12898075<\/a>. <\/li><\/ol>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Definition<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Characteristics<\/h4><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Benign PNSTs<\/h3><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Schwannomas<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Neurofibromas<\/h4><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Malignant PNSTs<\/h3><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Traumatic neuroma<\/h3><\/div>","protected":false},"excerpt":{"rendered":"<p>Definition Peripheral nerve sheath tumors (or PNSTs) are a rare type of soft tissue tumors arising from the cells located in the sheath of peripheral nerves. Characteristics Can be either benign or malignant In most cases, tumor cells show Schwann cell&nbsp;differentiation Schwann cells are the cells that produce myelin in the CNS Associated with&nbsp;neurofibromatosis (NF) [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":607,"menu_order":6,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-623","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.8 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Peripheral nerve sheath tumors &#8211; Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/meddists.com\/learn\/pre-clinical\/pathology\/soft-tissue-tumors\/peripheral-nerve-sheath-tumors\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"2 minutes\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/meddists.com\\\/learn\\\/pre-clinical\\\/pathology\\\/soft-tissue-tumors\\\/peripheral-nerve-sheath-tumors\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/pre-clinical\\\/pathology\\\/soft-tissue-tumors\\\/peripheral-nerve-sheath-tumors\\\/\",\"name\":\"Peripheral nerve sheath tumors &#8211; 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