{"id":12577,"date":"2024-10-11T19:15:57","date_gmt":"2024-10-11T17:15:57","guid":{"rendered":"https:\/\/valintermed.com\/?p=12577"},"modified":"2024-10-11T19:15:57","modified_gmt":"2024-10-11T17:15:57","slug":"plevropulmonalnaya-blastoma","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/","title":{"rendered":"Pleuropulmonary blastoma"},"content":{"rendered":"<div class=\"fpm_start\"><\/div>\n<p>Pleuropulmonary blastoma (PPB) is a rare, malignant tumor that occurs mainly in children and develops from the tissues of the lungs and pleura. This tumor disease is characterized by aggressive growth and metastasis, which creates serious problems for diagnosis and treatment. Pleuropulmonary blastoma is considered one of the most complex and difficult to predict types of childhood cancer. It can manifest itself in various forms of growth and various clinical symptoms, often with a sharp deterioration in the patient&#039;s condition. The pathogenesis of this disease is based on changes in cellular metabolism, which lead to unbalanced cell proliferation and disruption of normal regulation of the cell cycle.<\/p>\n<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_86 counter-flat ez-toc-counter ez-toc-light-blue ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Content<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Toggle Table of Content\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 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href=\"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/#%D0%98%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%B8_%D0%B8%D0%BD%D1%82%D0%B5%D1%80%D0%B5%D1%81%D0%BD%D1%8B%D0%B5_%D0%B8%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D1%8B\" >History of the disease and interesting historical facts<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/#%D0%AD%D0%BF%D0%B8%D0%B4%D0%B5%D0%BC%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%8F\" >Epidemiology<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/#%D0%93%D0%B5%D0%BD%D0%B5%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B0%D1%8F_%D0%BF%D1%80%D0%B5%D0%B4%D1%80%D0%B0%D1%81%D0%BF%D0%BE%D0%BB%D0%BE%D0%B6%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D1%8C_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8E\" >Genetic predisposition to this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/#%D0%A4%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Risk factors for the development of this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/#%D0%94%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Diagnosis of this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/#%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\" >Treatment<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/#%D0%A1%D0%BF%D0%B8%D1%81%D0%BE%D0%BA_%D0%BB%D0%B5%D0%BA%D0%B0%D1%80%D1%81%D1%82%D0%B2_%D0%BF%D1%80%D0%B8%D0%BC%D0%B5%D0%BD%D1%8F%D0%B5%D0%BC%D1%8B%D1%85_%D0%B4%D0%BB%D1%8F_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >List of medications used to treat this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/#%D0%9C%D0%BE%D0%BD%D0%B8%D1%82%D0%BE%D1%80%D0%B8%D0%BD%D0%B3_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Disease monitoring<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/#%D0%92%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D0%B5_%D0%BE%D1%81%D0%BE%D0%B1%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Age-related features of the disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-10\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pleuropulmonary-blastoma\/#%D0%92%D0%BE%D0%BF%D1%80%D0%BE%D1%81%D1%8B_%D0%B8_%D0%BE%D1%82%D0%B2%D0%B5%D1%82%D1%8B\" >Questions and Answers<\/a><\/li><\/ul><\/nav><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%D0%98%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%B8_%D0%B8%D0%BD%D1%82%D0%B5%D1%80%D0%B5%D1%81%D0%BD%D1%8B%D0%B5_%D0%B8%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D1%8B\"><\/span>History of the disease and interesting historical facts<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Pleuropulmonary blastoma was first described in the early 20th century as a rare type of tumor, but its recognition as a separate nosological entity occurred only in the 1970s and 1980s. It is noteworthy that the first cases of pleuropulmonary blastoma descriptions, as a rule, occurred among children under 5 years of age. Initially, researchers believed that this tumor was only one of the atypical forms of neuroblastoma, but further studies established unique morphological and clinical features of pleuropulmonary blastoma. Statistical data of that time, collected on the basis of observations, indicated unstable dynamics of incidence growth, which made the study of this pathology a subject of interest for many generations of oncologists.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%AD%D0%BF%D0%B8%D0%B4%D0%B5%D0%BC%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%8F\"><\/span>Epidemiology<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Epidemiological data on pleuropulmonary blastoma show that this disease is relatively rare. According to statistics, for every 1 million children under 15 years of age, there are about 1-3 cases of pleuropulmonary blastoma per year. The incidence is higher in boys (approximately 2:1 compared to girls), and the age of peak cases varies from 2 to 5 years. Analysis of cases in different regions of the world shows that data on the occurrence of this pathology may vary, but the tendency for the incidence to increase among certain ethnic groups has attracted the attention of researchers, indicating the possible influence of genetic and environmental factors.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%93%D0%B5%D0%BD%D0%B5%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B0%D1%8F_%D0%BF%D1%80%D0%B5%D0%B4%D1%80%D0%B0%D1%81%D0%BF%D0%BE%D0%BB%D0%BE%D0%B6%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D1%8C_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8E\"><\/span>Genetic predisposition to this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Genetic predisposition to pleuropulmonary blastoma is studied in the context of the presence of certain mutations in genes associated with cell proliferation and differentiation. Studies have shown that changes in the TP53 and MYC genes may play a key role in the development of this disease. For example, mutations in the TP53 gene can lead to disruption of cell cycle control mechanisms, leading to abnormal cell division and tumor growth. In addition, some researchers have reported a possible association of pleuropulmonary blastoma with hereditary syndromes such as Li-Fraumeni syndrome, although more research is needed to better understand this relationship.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A4%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Risk factors for the development of this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>There are various risk factors that may contribute to the development of pleuropulmonary blastoma. These include:<\/p>\n<ul>\n<li>Environmental pollutants, including toxic substances found in atmospheric air and water.<\/li>\n<li>Physical factors such as radiation exposure or exposure to strong magnetic fields.<\/li>\n<li>Certain viral infections may be associated with abnormal cell growth, although evidence remains limited.<\/li>\n<li>Having a family history of cancer.<\/li>\n<\/ul>\n<p>In the context of preventing the development of pleuropulmonary blastoma, scientific work is needed to continue studying these factors and their influence on genetic predisposition.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%94%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Diagnosis of this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Diagnosis of pleuropulmonary blastoma includes a set of measures aimed at identifying this disease in the early stages:<\/p>\n<ul>\n<li>Main symptoms may include cough, shortness of breath, chest pain and difficulty breathing.<\/li>\n<li>Laboratory tests include a complete blood count and tumor activity markers such as neuroblastoma antibodies.<\/li>\n<li>Radiological examinations (chest X-ray, CT scan) help visualize the tumor and assess its size and spread.<\/li>\n<li>Other diagnostic tests, such as magnetic resonance imaging, can help determine the location of the tumor.<\/li>\n<li>Differential diagnosis must be made with other types of pulmonary and pleural tumors, such as neuroblastoma or teratomas.<\/li>\n<\/ul>\n<p>The combination of these methods provides the opportunity to choose the optimal strategy for treatment and disease management.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\"><\/span>Treatment<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Treatment of pleuropulmonary blastoma is a multifaceted and individualized process that includes several approaches:<\/p><script data-noptimize=\"\" data-wpfc-render=\"false\">\nfpm_start( \"true\" );\n<\/script>\n\n<ul>\n<li>General treatment may include chemotherapy, which is aimed at killing cancer cells.<\/li>\n<li>Pharmacological treatment includes the use of drugs such as cisplatin and vincristine in various combinations to achieve maximum antitumor activity.<\/li>\n<li>Surgery aims to completely remove the tumor if it is localized and can be a critical step in treatment.<\/li>\n<li>Other treatments may include radiation therapy to shrink the tumor and prevent metastasis.<\/li>\n<\/ul>\n<p>The choice of treatment method depends on the stage of the disease, the behavior of the tumor and the patient&#039;s condition.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A1%D0%BF%D0%B8%D1%81%D0%BE%D0%BA_%D0%BB%D0%B5%D0%BA%D0%B0%D1%80%D1%81%D1%82%D0%B2_%D0%BF%D1%80%D0%B8%D0%BC%D0%B5%D0%BD%D1%8F%D0%B5%D0%BC%D1%8B%D1%85_%D0%B4%D0%BB%D1%8F_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>List of medications used to treat this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Among the drugs used in the treatment of pleuropulmonary blastoma, the following can be distinguished:<\/p>\n<ul>\n<li>Cisplatin<\/li>\n<li>Vincristine<\/li>\n<li>Doxorubicin<\/li>\n<li>Ifosfamide<\/li>\n<li>Methotrexate<\/li>\n<\/ul>\n<p>These drugs are often used in combination to achieve more effective antitumor therapy.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9C%D0%BE%D0%BD%D0%B8%D1%82%D0%BE%D1%80%D0%B8%D0%BD%D0%B3_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Disease monitoring<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Monitoring of patients with pleuropulmonary blastoma includes regular follow-up examinations to assess the effectiveness of treatment and to detect possible signs of recurrence or metastasis. The prognosis of the disease depends on many factors, including the stage at which treatment was started and the overall response to therapy. The main complications are disease recurrence and possible long-term effects of chemotherapy, such as secondary tumors or organ dysfunction.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%92%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D0%B5_%D0%BE%D1%81%D0%BE%D0%B1%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Age-related features of the disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Pleuropulmonary blastoma manifests itself differently depending on the patient&#039;s age. Young children often have more aggressive forms of the tumor, while adolescents may have a more stable condition. In newborns and infants, PPB is usually more difficult to diagnose, as symptoms may be similar to respiratory diseases. In older age (after 15 years), such tumors are much less common, but the presence of the disease requires special attention from doctors.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%92%D0%BE%D0%BF%D1%80%D0%BE%D1%81%D1%8B_%D0%B8_%D0%BE%D1%82%D0%B2%D0%B5%D1%82%D1%8B\"><\/span>Questions and Answers<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<ul>\n<li><strong>What are the main symptoms of pleuropulmonary blastoma?<\/strong> The main symptoms include cough, shortness of breath, chest pain, and possible signs of metastasis such as weight loss and fatigue.<\/li>\n<li><strong>How is pleuropulmonary blastoma diagnosed?<\/strong> Diagnostics includes clinical examination, radiography, computed tomography, urogenital studies, as well as morphological examination of tumor tissue.<\/li>\n<li><strong>What factors can prevent the development of pleuropulmonary blastoma?<\/strong> Risk factors include exposure to environmental pollutants and genetic predispositions.<\/li>\n<li><strong>What is the effectiveness of treatment for pleuropulmonary blastoma?<\/strong> The effectiveness of treatment depends on the time from diagnosis to the choice of optimal therapy, but early diagnosis significantly increases the chances of a favorable outcome.<\/li>\n<li><strong>Are there any age-related risks for pleuropulmonary blastoma?<\/strong> Yes, pleuropulmonary blastoma is most common in young children, although the clinical course and prognosis may vary.<\/li>\n<\/ul>\n<p>This article summarizes current data on pleuropulmonary blastoma and serves as a basis for further research and practice in the field of pediatric oncology.<\/p>\n<div class=\"fpm_end\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Pleuropulmonary blastoma (PPB) is a rare, malignant tumor that occurs mainly in children and develops from the tissues of the lungs and<\/p>","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[298],"tags":[],"class_list":["post-12577","post","type-post","status-publish","format-standard","hentry","category-medlibrary"],"_links":{"self":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12577","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/comments?post=12577"}],"version-history":[{"count":1,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12577\/revisions"}],"predecessor-version":[{"id":14502,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12577\/revisions\/14502"}],"wp:attachment":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media?parent=12577"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/categories?post=12577"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/tags?post=12577"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}