{"id":5478,"date":"2021-05-24T18:57:37","date_gmt":"2021-05-24T18:57:37","guid":{"rendered":"https:\/\/www.skincancer.org\/?page_id=5478"},"modified":"2025-05-08T16:56:57","modified_gmt":"2025-05-08T16:56:57","slug":"rare-skin-cancers","status":"publish","type":"page","link":"https:\/\/www.skincancer.org\/skin-cancer-information\/rare-skin-cancers\/","title":{"rendered":"Rare Skin Cancers"},"content":{"rendered":"<div class=\"wpb-content-wrapper\"><p>[vc_section][vc_row][vc_column][vc_column_text]<\/p>\n<h1 style=\"text-align: center;\">Rare Skin Cancers<\/h1>\n<p>[\/vc_column_text][\/vc_column][\/vc_row][vc_row][vc_column offset=&#8221;vc_col-lg-offset-1 vc_col-lg-10&#8243;][vc_custom_heading text=&#8221;What You Need to Know About Uncommon Skin Diseases&#8221; font_container=&#8221;tag:h2|text_align:center&#8221; use_theme_fonts=&#8221;yes&#8221;][\/vc_column][\/vc_row][\/vc_section][vc_section][vc_row][vc_column width=&#8221;9\/12&#8243;][vc_column_text el_class=&#8221;large&#8221;]While <a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/basal-cell-carcinoma\/\">basal cell carcinoma<\/a>, <a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/squamous-cell-carcinoma\/\">squamous cell carcinoma<\/a> and <a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/melanoma\/\">melanoma<\/a> are more widespread, these rare skin cancers are important to know about, because they may be mistaken for noncancerous conditions, delaying diagnosis and treatment.<\/p>\n<h4>Types of Skin Cancer<\/h4>\n<ul>\n<li><a href=\"#alm\">Acral lentiginous melanoma<\/a><\/li>\n<li><a href=\"#tcell\"><span class=\"NormalTextRun SCXW170158526 BCX0\" data-ccp-parastyle=\"Normal (Web)\">Cutaneous T-cell <\/span><span class=\"NormalTextRun SCXW170158526 BCX0\" data-ccp-parastyle=\"Normal (Web)\">l<\/span><span class=\"NormalTextRun SCXW170158526 BCX0\" data-ccp-parastyle=\"Normal (Web)\">ymphoma<\/span><\/a><\/li>\n<li><a href=\"#dfsp\">Dermatofibrosarcoma protuberans (DFSP)<\/a><\/li>\n<li><a href=\"#ks\">Kaposi\u2019s sarcoma<\/a><\/li>\n<li><a href=\"#mac\">Microcystic adnexal carcinoma (MAC)<\/a><\/li>\n<li><a href=\"#mcc\">Merkel cell carcinoma (MCC)<\/a><\/li>\n<li><a href=\"#sc\">Sebaceous carcinoma<\/a><\/li>\n<li><a href=\"#ups\">Undifferentiated pleomorphic sarcoma<\/a><\/li>\n<li><a href=\"#empd\">Extramammary Paget&#8217;s disease (EMPD)<\/a><\/li>\n<\/ul>\n<p>[\/vc_column_text][\/vc_column][vc_column width=&#8221;1\/4&#8243;][vc_column_text el_class=&#8221;page-nav&#8221;]<\/p>\n<ul>\n<li><a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/\">Skin cancer 101<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/early-detection\/\">Skin cancer early detection<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/skin-cancer-prevention\/\">Skin cancer prevention<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/support-resources\/\">Skin cancer support resources<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/support-resources\/dos-and-donts-for-skin-cancer-patients\/\">Do&#8217;s &amp; don&#8217;ts for skin cancer patients<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/get-involved\/skin-cancer-awareness-month\/toolkit\/\" target=\"_blank\" rel=\"noopener noreferrer\">Skin Cancer Awareness Toolkit<\/a><\/li>\n<\/ul>\n<p>[\/vc_column_text][\/vc_column][\/vc_row][\/vc_section][vc_section full_width=&#8221;stretch_row&#8221; content_placement=&#8221;middle&#8221; css=&#8221;.vc_custom_1532456871168{background-color: #323f4a !important;}&#8221;][vc_row][vc_column width=&#8221;1\/3&#8243; el_class=&#8221;subpage-nav&#8221;][vc_btn title=&#8221;Skin Cancer Prevention&#8221; style=&#8221;custom&#8221; custom_background=&#8221;#323f4a&#8221; custom_text=&#8221;#ffcc42&#8243; shape=&#8221;square&#8221; size=&#8221;lg&#8221; align=&#8221;center&#8221; i_align=&#8221;right&#8221; i_icon_fontawesome=&#8221;&#8221; add_icon=&#8221;true&#8221; link=&#8221;url:https%3A%2F%2Fwww.skincancer.org%2Fskin-cancer-prevention%2F|title:Skin%20Cancer%20Prevention&#8221;][\/vc_column][vc_column width=&#8221;1\/3&#8243; el_class=&#8221;subpage-nav&#8221;][vc_btn title=&#8221;Early Detection&#8221; style=&#8221;custom&#8221; custom_background=&#8221;#323f4a&#8221; custom_text=&#8221;#ffcc42&#8243; shape=&#8221;square&#8221; size=&#8221;lg&#8221; align=&#8221;center&#8221; i_align=&#8221;right&#8221; i_icon_fontawesome=&#8221;&#8221; add_icon=&#8221;true&#8221; link=&#8221;url:https%3A%2F%2Fwww.skincancer.org%2Fearly-detection%2F|title:Early%20Detection&#8221;][\/vc_column][vc_column width=&#8221;1\/3&#8243; el_class=&#8221;subpage-nav&#8221;][vc_btn title=&#8221;Facts &amp; Stats&#8221; style=&#8221;custom&#8221; custom_background=&#8221;#323f4a&#8221; custom_text=&#8221;#ffcc42&#8243; shape=&#8221;square&#8221; size=&#8221;lg&#8221; align=&#8221;center&#8221; i_align=&#8221;right&#8221; i_icon_fontawesome=&#8221;&#8221; add_icon=&#8221;true&#8221; link=&#8221;url:https%3A%2F%2Fwww.skincancer.org%2Fskin-cancer-information%2Fskin-cancer-facts%2F|title:Skin%20Cancer%20Facts%20%26%20Statistics&#8221;][\/vc_column][\/vc_row][\/vc_section][vc_section][vc_row el_class=&#8221;content-block&#8221; el_id=&#8221;dfsp&#8221;][vc_column width=&#8221;1\/3&#8243;][vc_custom_heading text=&#8221;Acral lentiginous melanoma&#8221; font_container=&#8221;tag:h3|text_align:left&#8221; use_theme_fonts=&#8221;yes&#8221; el_id=&#8221;alm&#8221;][\/vc_column][vc_column width=&#8221;2\/3&#8243;][vc_column_text css=&#8221;&#8221;]<\/p>\n<p style=\"font-weight: 400;\"><strong>What is it?\u00a0<\/strong>Acral lentiginous melanoma (ALM) is a very rare, aggressive type of skin cancer. While uncommon in general, ALM is the most common type of melanoma found in\u00a0<a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/skin-cancer-skin-of-color\/\">people of colo<\/a>r, including individuals of African ancestry.<\/p>\n<p style=\"font-weight: 400;\"><strong>What does it look like?\u00a0<\/strong>\u00a0ALM may appear as a black or brown area of skin, in hard-to-spot places including under the nails (<a href=\"https:\/\/www.skincancer.org\/blog\/looking-for-clues-the-lesser-known-signs-of-skin-cancer\/\">subungual<\/a>) and on the soles of the feet or palms of the hands.<\/p>\n<p style=\"font-weight: 400;\"><strong>What causes it?\u00a0<\/strong>While most melanomas are caused by exposure to ultraviolet (UV) radiation from the sun or from tanning beds, ALM, which develops on hairless skin, is more likely caused by genetic factors.<\/p>\n<p style=\"font-weight: 400;\"><strong>How is it treated? \u00a0<\/strong><a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/melanoma\/melanoma-treatments\/#surgical\">Excisional surgery<\/a>\u00a0is usually performed to remove the primary tumor and a margin of healthy tissue around it. Depending on the size and location of the tumor, and the overall health of the patient, other treatment options include\u00a0<a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/\">radiation therapy<\/a>, \u00a0<a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/melanoma\/melanoma-treatments\/#immunotherapy\">immunotherapy<\/a>,\u00a0<a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/melanoma\/melanoma-treatments\/#targeted\">targeted therapy<\/a>\u00a0and\u00a0<a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/melanoma\/melanoma-treatments\/#chemotherapy\">chemotherapy<\/a>. The five-year survival rate for African American patients with melanoma is 70 percent. Most deaths from this form of melanoma occur when it is diagnosed late, after it has begun to spread.<\/p>\n<p>[\/vc_column_text][vc_row_inner][vc_column_inner width=&#8221;1\/2&#8243;][vc_column_text]<img loading=\"lazy\" decoding=\"async\" class=\"aligncenter wp-image-7864 size-full\" src=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/Acral-lentiginous-melanoma-340x240-1.jpg\" alt=\"\" width=\"340\" height=\"240\" \/><\/p>\n<div class=\"wpb_column vc_column_container vc_col-sm-6\">\n<div class=\"vc_column-inner\">\n<div class=\"wpb_wrapper\">\n<div class=\"wpb_text_column wpb_content_element \">\n<div class=\"wpb_wrapper\">\n<p><em>Photo credit: Hugh Gloster, MD<\/em><\/p>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n<p>[\/vc_column_text][\/vc_column_inner][vc_column_inner width=&#8221;1\/2&#8243;][vc_column_text]<\/p>\n\n\t\t<style type=\"text\/css\">\n\t\t\t#gallery-1 {\n\t\t\t\tmargin: auto;\n\t\t\t}\n\t\t\t#gallery-1 .gallery-item {\n\t\t\t\tfloat: left;\n\t\t\t\tmargin-top: 10px;\n\t\t\t\ttext-align: center;\n\t\t\t\twidth: 100%;\n\t\t\t}\n\t\t\t#gallery-1 img {\n\t\t\t\tborder: 2px solid #cfcfcf;\n\t\t\t}\n\t\t\t#gallery-1 .gallery-caption {\n\t\t\t\tmargin-left: 0;\n\t\t\t}\n\t\t\t\/* see gallery_shortcode() in wp-includes\/media.php *\/\n\t\t<\/style>\n\t\t<div id='gallery-1' class='gallery galleryid-5478 gallery-columns-1 gallery-size-thumbnail'><dl class='gallery-item'>\n\t\t\t<dt class='gallery-icon landscape'>\n\t\t\t\t<img loading=\"lazy\" decoding=\"async\" width=\"170\" height=\"170\" src=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/figure4a-1.jpg\" class=\"attachment-thumbnail size-thumbnail\" alt=\"\" \/>\n\t\t\t<\/dt><\/dl><br style=\"clear: both\" \/><dl class='gallery-item'>\n\t\t\t<dt class='gallery-icon landscape'>\n\t\t\t\t<img loading=\"lazy\" decoding=\"async\" width=\"170\" height=\"170\" src=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/figure4b-1.jpg\" class=\"attachment-thumbnail size-thumbnail\" alt=\"\" \/>\n\t\t\t<\/dt><\/dl><br style=\"clear: both\" \/>\n\t\t<\/div>\n\n<p>[\/vc_column_text][\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row][vc_row el_class=&#8221;content-block&#8221;][vc_column width=&#8221;1\/3&#8243;][vc_custom_heading text=&#8221;Cutaneous T-cell lymphoma&#8221; font_container=&#8221;tag:h3|text_align:left&#8221; use_theme_fonts=&#8221;yes&#8221; el_id=&#8221;tcell&#8221;][\/vc_column][vc_column width=&#8221;2\/3&#8243;][vc_column_text]<b><span data-contrast=\"none\">What is it?\u202f<\/span><\/b><span data-contrast=\"none\">Cutaneous T-cell lymphoma (CTCL) is a rare type of blood cancer that arises on the skin. CTCL starts in white blood cells called T-lymphocytes that normally fight infection in the body. The most common subtype is known as mycosis fungoides (MF), which is\u202f<\/span><span data-contrast=\"none\">more prevalent among Black people than it is among other racial grou<\/span><span data-contrast=\"auto\">ps. S\u00e9zary syndrome is a less common but more aggressive subtype that may appear as an intense, widespread red rash over the body.\u00a0<\/span><span data-ccp-props=\"{&quot;134233117&quot;:false,&quot;134233118&quot;:false,&quot;201341983&quot;:0,&quot;335559738&quot;:0,&quot;335559739&quot;:240,&quot;335559740&quot;:240}\">\u00a0<\/span><\/p>\n<p><b><span data-contrast=\"none\">What does it look like<\/span><\/b><span data-contrast=\"none\">? T-cell lymphoma most commonly affects the skin, causing rash-like p<\/span><span data-contrast=\"auto\">atches or plaques that grow or spread, itching, lumps on the skin and sometimes even tumors. The disease can also involve the blood, lymph nodes and internal organs. It can be difficult to identify and may require multiple biopsies at different stages of development to get a correct diagnosis.\u00a0<\/span><span data-ccp-props=\"{&quot;134233117&quot;:false,&quot;134233118&quot;:false,&quot;201341983&quot;:0,&quot;335559738&quot;:0,&quot;335559739&quot;:0,&quot;335559740&quot;:240}\">\u00a0<\/span><\/p>\n<p><b><span data-contrast=\"none\">What causes it?\u202f<\/span><\/b><span data-contrast=\"none\">\u202fThe cause of cutaneous T-cell lymphoma is not known. What scientists do know is that the disease begins when T-cells develop changes (mutations) in their DNA causing them to mutate, grow rapidly and attack the skin.\u202f<\/span><span data-ccp-props=\"{&quot;134233117&quot;:false,&quot;134233118&quot;:false,&quot;201341983&quot;:0,&quot;335559738&quot;:0,&quot;335559739&quot;:240,&quot;335559740&quot;:240}\">\u00a0<\/span><\/p>\n<p><b><span data-contrast=\"none\">How is it treated?\u202f<\/span><\/b><span data-contrast=\"none\">While<\/span> <span data-contrast=\"none\">T-cell lymphoma\u202fis not curable, symptoms can be controlled and treated in the following ways:\u202f<\/span><span data-ccp-props=\"{&quot;134233117&quot;:false,&quot;134233118&quot;:false,&quot;201341983&quot;:0,&quot;335559738&quot;:0,&quot;335559739&quot;:0,&quot;335559740&quot;:240}\">\u00a0<\/span><\/p>\n<ul>\n<li><b><span data-contrast=\"none\">Topical medications<\/span><\/b><span data-contrast=\"auto\">, including steroids, retinoids and chemotherapy.\u00a0<\/span><span data-ccp-props=\"{&quot;134233117&quot;:false,&quot;134233118&quot;:false,&quot;201341983&quot;:0,&quot;335559685&quot;:360,&quot;335559738&quot;:0,&quot;335559739&quot;:0,&quot;335559740&quot;:240}\">\u00a0<\/span><\/li>\n<li><b><span data-contrast=\"none\">Phototherapy<\/span><\/b><span data-contrast=\"none\">,<\/span><span data-contrast=\"auto\"> where physicians use special devices to treat the skin with targeted doses of ultraviolet light.\u00a0<\/span><span data-ccp-props=\"{&quot;134233117&quot;:false,&quot;134233118&quot;:false,&quot;201341983&quot;:0,&quot;335559685&quot;:360,&quot;335559738&quot;:0,&quot;335559739&quot;:0,&quot;335559740&quot;:240}\">\u00a0<\/span><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#radiation-therapy\"><b><span data-contrast=\"none\">Radiation therapy<\/span><\/b><\/a><span data-contrast=\"auto\">, specifically electronic beam therapy.<\/span><span data-ccp-props=\"{&quot;134233117&quot;:false,&quot;134233118&quot;:false,&quot;201341983&quot;:0,&quot;335559685&quot;:360,&quot;335559738&quot;:0,&quot;335559739&quot;:0,&quot;335559740&quot;:240}\">\u00a0<\/span><\/li>\n<\/ul>\n<p>[\/vc_column_text][\/vc_column][\/vc_row][vc_row el_class=&#8221;content-block&#8221;][vc_column width=&#8221;1\/3&#8243;][vc_custom_heading text=&#8221;Dermatofibrosarcoma protuberans (DFSP)&#8221; font_container=&#8221;tag:h3|text_align:left&#8221; use_theme_fonts=&#8221;yes&#8221;][\/vc_column][vc_column width=&#8221;2\/3&#8243;][vc_column_text]<strong>What is it?<\/strong> Unlike common skin cancers that arise from cells in the outermost layer of skin, dermatofibrosarcoma protuberans (DFSP) is an extremely rare cancer that arises from cells in the deepest layer of the skin, known as the dermis. While DFSP is slow growing and rarely spreads (metastasizes) to distant areas of the body, it has a high rate of local recurrence.<\/p>\n<p><strong>What does it look like<\/strong>? DFSP can look like an ordinary scar, cyst or bruise. It can develop anywhere on the body but is most commonly found on the arms, legs or torso. Over time, it can grow to become a large lump under the skin. The tumor grows slowly but can penetrate deep into the dermis and infiltrate the fat and surrounding tissue.<\/p>\n<p><strong>What causes it? <\/strong>\u00a0Experts do not fully understand what causes DFSP, but injuries to the affected skin may be a risk factor.[\/vc_column_text][vc_row_inner][vc_column_inner width=&#8221;1\/2&#8243;][vc_column_text]<img loading=\"lazy\" decoding=\"async\" class=\"size-medium wp-image-5767 aligncenter\" src=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/Dermatofibrosarcoma-Protuberans-600x474.png\" alt=\"Dermatofibrosarcoma Protuberans\" width=\"600\" height=\"474\" srcset=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/Dermatofibrosarcoma-Protuberans-600x474.png 600w, https:\/\/www.skincancer.org\/wp-content\/uploads\/Dermatofibrosarcoma-Protuberans-1024x809.png 1024w, https:\/\/www.skincancer.org\/wp-content\/uploads\/Dermatofibrosarcoma-Protuberans-768x607.png 768w, https:\/\/www.skincancer.org\/wp-content\/uploads\/Dermatofibrosarcoma-Protuberans.png 1172w\" sizes=\"auto, (max-width: 600px) 100vw, 600px\" \/><em>Photo credit:<\/em> Robert Egbers, MD, MSc, FACMS <em>via ACMS<\/em>[\/vc_column_text][\/vc_column_inner][vc_column_inner width=&#8221;1\/2&#8243;][vc_column_text css=&#8221;&#8221;]<img loading=\"lazy\" decoding=\"async\" class=\"aligncenter wp-image-5935 size-medium\" src=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/Recurrent-Dermatofibrosarcoma-Protuberans-Gloster-photo-credit-600x480.jpg\" alt=\"recurrent dermatfibrosarcoma protuberans\" width=\"600\" height=\"480\" srcset=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/Recurrent-Dermatofibrosarcoma-Protuberans-Gloster-photo-credit-600x480.jpg 600w, https:\/\/www.skincancer.org\/wp-content\/uploads\/Recurrent-Dermatofibrosarcoma-Protuberans-Gloster-photo-credit-1024x819.jpg 1024w, https:\/\/www.skincancer.org\/wp-content\/uploads\/Recurrent-Dermatofibrosarcoma-Protuberans-Gloster-photo-credit-768x614.jpg 768w, https:\/\/www.skincancer.org\/wp-content\/uploads\/Recurrent-Dermatofibrosarcoma-Protuberans-Gloster-photo-credit.jpg 1080w\" sizes=\"auto, (max-width: 600px) 100vw, 600px\" \/>Recurrent dermatofibrosarcoma protuberans on dark skin. <em>Photo credit:<\/em> Hugh Gloster, MD[\/vc_column_text][\/vc_column_inner][\/vc_row_inner][vc_row_inner][vc_column_inner width=&#8221;1\/2&#8243;][vc_column_text]<img loading=\"lazy\" decoding=\"async\" class=\"wp-image-5769 size-medium aligncenter\" src=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/dermatofibrosarcoma-protuberans-dfsp-e1623694261630-600x481.png\" alt=\"dermatofibrosarcoma protuberans dfsp\" width=\"600\" height=\"481\" \/><em>Photo credit:<\/em> Vitaly Terushkin, MD <em>via ACMS<\/em>[\/vc_column_text][\/vc_column_inner][vc_column_inner width=&#8221;1\/2&#8243;][vc_column_text css=&#8221;&#8221;]<img loading=\"lazy\" decoding=\"async\" class=\"aligncenter wp-image-5936 size-medium\" src=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/Dermatofibrosarcoma-Potuberans-Black-co-Gloster-e1629318315843-600x470.jpg\" alt=\"Dermatofibrosarcoma protuberans\" width=\"600\" height=\"470\" srcset=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/Dermatofibrosarcoma-Potuberans-Black-co-Gloster-e1629318315843-600x470.jpg 600w, https:\/\/www.skincancer.org\/wp-content\/uploads\/Dermatofibrosarcoma-Potuberans-Black-co-Gloster-e1629318315843-1024x802.jpg 1024w, https:\/\/www.skincancer.org\/wp-content\/uploads\/Dermatofibrosarcoma-Potuberans-Black-co-Gloster-e1629318315843-768x601.jpg 768w, https:\/\/www.skincancer.org\/wp-content\/uploads\/Dermatofibrosarcoma-Potuberans-Black-co-Gloster-e1629318315843.jpg 1111w\" sizes=\"auto, (max-width: 600px) 100vw, 600px\" \/>DFSP on dark skin<em>. Photo credit:<\/em> Hugh Gloster, MD[\/vc_column_text][\/vc_column_inner][\/vc_row_inner][vc_row_inner][vc_column_inner][vc_column_text]<strong>How is it treated?<\/strong> DFSP is often treated by <a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#excisional-surgery\">excisional surgery<\/a> to remove the tumor and a margin of healthy skin. Additional treatments include:<\/p>\n<ul>\n<li><strong>Mohs surgery:<\/strong> This specialized surgery involves removing and assessing layers of the tumor until only cancer-free tissue remains. This procedure produces the highest cure rate for DFSP and spares the most amount of healthy tissue. <a href=\"https:\/\/www.skincancer.org\/treatment-resources\/mohs-surgery\/\">More about Mohs surgery<\/a>.<\/li>\n<li><strong>Radiation therapy<\/strong>: <a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/\">Radiation<\/a> destroys cancer cells when surgery is not an option or if DFSP recurs after surgery.<\/li>\n<li><strong>Targeted therapy: <\/strong>Drugs like imatinib (Gleevac) that target and attack certain cells have been approved by the FDA to treat DFSP.<\/li>\n<\/ul>\n<p>[\/vc_column_text][\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row][vc_row el_class=&#8221;content-block&#8221; el_id=&#8221;ks&#8221;][vc_column width=&#8221;1\/3&#8243;][vc_custom_heading text=&#8221;Kaposi\u2019s sarcoma&#8221; font_container=&#8221;tag:h3|text_align:left&#8221; use_theme_fonts=&#8221;yes&#8221;][\/vc_column][vc_column width=&#8221;2\/3&#8243;][vc_column_text]<strong>What is it?<\/strong> Kaposi\u2019s sarcoma is a rare skin cancer that develops from the cells that line the blood vessels or lymph vessels. There are two types of Kaposi&#8217;s sarcoma:<\/p>\n<ol>\n<li>The first type, often referred to as classic Kaposi\u2019s sarcoma (CKS) is often seen in men over age 50 of European, Mediterranean or Middle Eastern descent. This type is slow growing, easier to treat and rarely causes serious problems.<\/li>\n<li>The second type is an aggressive cancer that affects people with weakened immune systems, including cancer patients, organ transplant recipients and people who are HIV positive. While this dangerous, fast-growing cancer is on the decline thanks to antiviral medications, if you think you may be at risk, get prompt medical attention.<\/li>\n<\/ol>\n<p><strong>What does it look like?<\/strong>\u00a0Kaposi&#8217;s sarcoma can present as red, purple or brown lesions on the skin, mucous membranes, lymph nodes and other organs. The lesions may be flat or slightly raised and can grow and spread rapidly.<\/p>\n<p><em><img loading=\"lazy\" decoding=\"async\" class=\"size-full wp-image-1097 alignnone\" src=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/Kaposi-Image-PURCHASED-300x.jpg\" alt=\"Kaposi's sarcoma\" width=\"300\" height=\"300\" \/><br \/>\nPhoto credit: ALAMY<\/em><\/p>\n<p><strong>What causes it?<\/strong>\u00a0The disease is caused by the Kaposi\u2019s sarcoma associated herpesvirus 8 (KSHV). People with healthy immune systems can be exposed to the virus and show little or no signs or symptoms, because of the immune system&#8217;s ability to keep the virus in check. Cancer patients receiving chemotherapy, HIV positive patients and organ transplant patients are all at a high risk of serious infection. The virus is believed to be transmitted by the exchange of saliva.<\/p>\n<p><strong>How is it treated?<\/strong> Kaposi&#8217;s sarcoma can be treated in several different ways depending on the stage of the disease and the overall health of the patient. Treatment options include:<\/p>\n<p><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#excisional-surgery\">Excisional surgery<\/a><br \/>\n<a href=\"https:\/\/www.skincancer.org\/treatment-resources\/mohs-surgery\/\">Mohs surgery<\/a><br \/>\n<a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#cryosurgery\">Cryosurgery<\/a><br \/>\n<a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/\">Chemotherapy<\/a><br \/>\nAntiviral therapy<br \/>\n<a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/\">Immunotherapy<\/a><br \/>\n<a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#radiation-therapy\">Radiation therapy<\/a><\/p>\n<p>The more serious form of Kaposi&#8217;s sarcoma is treated mainly by systemic antiviral therapy to kill the virus that is causing the disease.[\/vc_column_text][\/vc_column][\/vc_row][vc_row el_class=&#8221;content-block&#8221; el_id=&#8221;mac&#8221;][vc_column width=&#8221;1\/3&#8243;][vc_custom_heading text=&#8221;Microcystic adnexal carcinoma&#8221; font_container=&#8221;tag:h3|text_align:left&#8221; use_theme_fonts=&#8221;yes&#8221;][\/vc_column][vc_column width=&#8221;2\/3&#8243;][vc_column_text]<strong>What is it?<\/strong> Microcystic adnexal carcinoma (MAC) is a very rare tumor of the skin that develops in the sweat gland, most often in the head and neck region. This type of tumor is also known as a sclerosis sweat gland carcinoma. Although MACs may occur in other parts of the body, they commonly present on sun exposed areas in the middle of the face. People ages 50 and older are most likely to develop these tumors. While MACs can be locally aggressive, growing in size and into nearby tissues, they rarely spread (metastasize) to more distant parts of the body.<\/p>\n<p><strong>What does it look like:<\/strong> MAC can appear as a yellowish spot, raised patch or smooth bump on the skin.<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-5858 size-full\" src=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/Microcystic-adnexal-carcinoma.jpg\" alt=\"Microcystic adnexal carcinoma\" width=\"387\" height=\"325\" \/><br \/>\n<!-- Photo: https:\/\/ars.els-cdn.com\/content\/image\/1-s2.0-S2352241015000225-gr1.jpg --><\/p>\n<p><strong>What causes it:<\/strong> The cause of microcystic adenexal carcinoma is not yet known. Risk factors include exposure to ultraviolet light (UV) light from the sun, radiation treatment and immunosuppression.<\/p>\n<p><strong>How is it treated?<\/strong>\u00a0Most MACs are treated with\u00a0<a href=\"https:\/\/www.skincancer.org\/treatment-resources\/mohs-surgery\/\">Mohs surgery<\/a>.<\/p>\n<p><!--\u00a0(add graphic model of a 3 dimension layer of skin with arrows ) different layers, squamous cells, basal where the epidermis meets the dermis, merkel, derived incited by sun, virus, a trauma. -->[\/vc_column_text][\/vc_column][\/vc_row][vc_row el_class=&#8221;content-block&#8221; el_id=&#8221;mcc&#8221;][vc_column width=&#8221;1\/3&#8243;][vc_custom_heading text=&#8221;Merkel cell carcinoma (MCC)&#8221; font_container=&#8221;tag:h3|text_align:left&#8221; use_theme_fonts=&#8221;yes&#8221;][\/vc_column][vc_column width=&#8221;2\/3&#8243;][vc_column_text]<strong>What is it?<\/strong> Merkel cell carcinoma (MCC) is a rare, aggressive skin cancer with a high risk for recurring and spreading (metastasizing) to other areas of the body. Merkel cells are located deep in the top layer of skin. Merkel cells are connected to nerves, signaling touch sensation as \u201ctouch receptors.\u201d MCC was named after Merkel cells due to the similar microscopic features; however, recent research suggests that it is unlikely that MCC originates directly from normal Merkel cells.<\/p>\n<p><a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/merkel-cell-carcinoma\/\">Learn more about MCC<\/a><br \/>\n<a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/merkel-cell-carcinoma\/mcc-patient-support\/\">Visit our MCC Patient Support Center<\/a><br \/>\n<a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/merkel-cell-carcinoma\/mcc-warning-signs\/#aeiou\">View photos of MCC<\/a><\/p>\n<p><strong>What causes it?\u00a0<\/strong>Evidence of the Merkel cell polyomavirus can be found in most, but not all, Merkel cell tumors. Since it&#8217;s a common virus and MCC is a rare disease, scientists are not certain about how or why the virus causes MCC in some people. What is clear is that factors such as <a href=\"https:\/\/www.skincancer.org\/risk-factors\/uv-radiation\/\">UV exposure<\/a> and immunosuppression also play a role.<\/p>\n<p><strong>What does it look like? \u00a0<\/strong>These tumors usually appear as firm, painless lesions or nodules on sun-exposed areas of the body, including the head, neck or eyelids. While these\u00a0tumors are not as distinctive as other skin cancers, their rapid rate of growth is often what causes patients and doctors to take notice.<\/p>\n<p><strong>How is it treated?<\/strong> MCC treatment options are determined by the stage of the disease and the overall health of the patient. Treatment includes surgical removal of the primary tumor along with:<\/p>\n<ul>\n<li><a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/merkel-cell-carcinoma\/mcc-treatment\/#radiation\">Radiation<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/merkel-cell-carcinoma\/mcc-treatment\/#immunotherapy\">Immunotherapy<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/skin-cancer-information\/merkel-cell-carcinoma\/mcc-treatment\/#chemotherapy\">Chemotherapy<\/a><\/li>\n<\/ul>\n<p>[\/vc_column_text][\/vc_column][\/vc_row][vc_row el_class=&#8221;content-block&#8221; el_id=&#8221;sc&#8221;][vc_column width=&#8221;1\/3&#8243;][vc_custom_heading text=&#8221;Sebaceous carcinoma&#8221; font_container=&#8221;tag:h3|text_align:left&#8221; use_theme_fonts=&#8221;yes&#8221;][\/vc_column][vc_column width=&#8221;2\/3&#8243;][vc_column_text]<strong>What is it?<\/strong> A sebaceous carcinoma is an uncommon cancer that arises from the unchecked growth of cells that line the oil glands (sebaceous glands or sebum-producing glands) in the skin. It often develops on the face and eyelids, but can occur anywhere on the body. According to the American College of Mohs Surgery, about thirty percent of people with a genetic condition known as Muir-Torre syndrome develop sebaceous carcinomas. Patients with Muir-Torre syndrome can also develop internal malignancies, including colon cancer.<\/p>\n<p><strong>What does it look like?<\/strong> The tumor may arise as a lump or thickening of the skin on the eyelid or on the face that is often painless, and sometimes oozes or bleeds. In other parts of the body, the tumor may look like a yellowish lump which sometimes bleeds.<br \/>\n<img loading=\"lazy\" decoding=\"async\" class=\"wp-image-5771 size-thumbnail alignnone\" src=\"https:\/\/www.skincancer.org\/wp-content\/uploads\/sebaceous-carcinoma-340x240.png\" alt=\"sebaceous carcinoma\" width=\"340\" height=\"240\" \/><br \/>\n<em>Photo credit:<\/em> Ramin Fathi, MD and Stan Tolkachjov, MD, FACMS<em> via ACMS<\/em><\/p>\n<p><strong>How is it treated? <\/strong><\/p>\n<ul>\n<li><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/mohs-surgery\/\">Mohs surgery<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#radiation-therapy\">R<\/a><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#radiation-therapy\">ad<\/a><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#radiation-therapy\">iation therapy<\/a><\/li>\n<\/ul>\n<p>[\/vc_column_text][\/vc_column][\/vc_row][vc_row el_class=&#8221;content-block&#8221; el_id=&#8221;ups&#8221;][vc_column width=&#8221;1\/3&#8243;][vc_custom_heading text=&#8221;Undifferentiated pleomorphic sarcoma&#8221; font_container=&#8221;tag:h3|text_align:left&#8221; use_theme_fonts=&#8221;yes&#8221;][\/vc_column][vc_column width=&#8221;2\/3&#8243;][vc_column_text]<strong>What is it?<\/strong> Undifferentiated pleomorphic sarcomas are rare soft tissue tumors that often arise from cells known as fibroblasts, in the dermis. This type of tumor often occurs in older men. Undifferentiated pleomorphic sarcomas can occur anywhere on the body. They can grow quickly and spread to other parts of the body. Superficial tumors were once known as atypical fibroxanthomas, and deeper, more advanced tumors were called malignant fibrous histiocytomas.<\/p>\n<p><strong>What does it look like? <\/strong>A growing lump or area of swelling on the skin that appears on the arms, legs, abdomen or scalp.<\/p>\n<p><strong>How is it treated? <\/strong>\u00a0Surgery is usually performed to remove the primary tumor. Depending on the size and location of the tumor, and the overall health of the patient, other treatment options include\u00a0<a href=\"https:\/\/www.skincancer.org\/treatment-resources\/mohs-surgery\/\">Mohs surger<\/a>y, <a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/\">radiation therapy<\/a>, chemotherapy and immunotherapy.[\/vc_column_text][\/vc_column][\/vc_row][vc_row el_class=&#8221;content-block&#8221; el_id=&#8221;empd&#8221;][vc_column width=&#8221;1\/3&#8243;][vc_custom_heading text=&#8221;Extramammary Paget&#8217;s disease (EMPD)&#8221; font_container=&#8221;tag:h3|text_align:left&#8221; use_theme_fonts=&#8221;yes&#8221;][\/vc_column][vc_column width=&#8221;2\/3&#8243;][vc_column_text]Extramammary Paget&#8217;s disease (EPMD) is a rare disease that frequently presents in areas where apocrine sweat glands are abundant, in the armpits or around the anus and genitals. The condition is related to Paget&#8217;s disease of the breast. This very slow-growing cancer sometimes takes years to become noticeable.<\/p>\n<p><strong>What does it look like?<\/strong> A persistent, sometimes irritating rash that resembles eczema and does not respond to topical treatments.<\/p>\n<p><strong>How is it treated?<\/strong><\/p>\n<ul>\n<li><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/mohs-surgery\/\">Mohs surgery<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#excisional-surgery\">Excisional surgery<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#5-fluorouracil\">Topical treatments<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#radiation-therapy\">Radiation therapy<\/a><\/li>\n<li><a href=\"https:\/\/www.skincancer.org\/treatment-resources\/treatment-glossary\/#photodynamic-therapy\">Photodynamic therapy<\/a><\/li>\n<\/ul>\n<p>[\/vc_column_text][\/vc_column][\/vc_row][vc_row][vc_column][vc_row_inner][vc_column_inner width=&#8221;1\/3&#8243;][vc_column_text css=&#8221;&#8221;]<em><strong>Reviewed by:<\/strong><br \/>\n<\/em><\/p>\n<p><em>Deborah S. Sarnoff, MD<br \/>\n<\/em>[\/vc_column_text][\/vc_column_inner][vc_column_inner width=&#8221;1\/3&#8243;][\/vc_column_inner][vc_column_inner width=&#8221;1\/3&#8243;][vc_column_text]<\/p>\n<h5 class=\"newsletter-callout\"><a href=\"https:\/\/www.skincancer.org\/newsletter-sign-up\/\">Sign up to receive our newsletter<\/a><\/h5>\n<p>[\/vc_column_text][\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row][vc_row][vc_column][\/vc_column][\/vc_row][\/vc_section]<\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"<p>[vc_section][vc_row][vc_column][vc_column_text] Rare Skin Cancers [\/vc_column_text][\/vc_column][\/vc_row][vc_row][vc_column offset=&#8221;vc_col-lg-offset-1 vc_col-lg-10&#8243;][vc_custom_heading text=&#8221;What You Need to Know About Uncommon Skin Diseases&#8221; font_container=&#8221;tag:h2|text_align:center&#8221; use_theme_fonts=&#8221;yes&#8221;][\/vc_column][\/vc_row][\/vc_section][vc_section][vc_row][vc_column width=&#8221;9\/12&#8243;][vc_column_text el_class=&#8221;large&#8221;]While [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":8,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_acf_changed":false,"footnotes":""},"class_list":["post-5478","page","type-page","status-publish","hentry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v25.3 (Yoast SEO v25.3) - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Rare Skin Cancers - The Skin Cancer Foundation<\/title>\n<meta name=\"description\" content=\"What is rare skin cancer? 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