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Mesothelioma is extremely rare in children and adolescents, with only 2% to 5% of patients presenting during the first two decades of life.[1] Fewer than 300 cases in children have been reported.[2] An analysis from the National Cancer Database identified 46 pediatric patients (aged 0–21 years) and 524 young adult patients (aged 22–39 years) with mesothelioma (363 peritoneal and 207 pleural).[3] Patients with peritoneal mesothelioma were more frequently female (63.1%). The mean overall survival was higher in patients with peritoneal mesothelioma (125 months) than in those with pleural mesothelioma (69 months), which remained significant after stratification of pediatric and young adult patients. In adults, increased mesothelioma risk is associated with inherited BAP1 variants, exposures to asbestos, and exposures to radiation therapy during previous cancer treatments. These risk factors are rare in pediatric patients, and there are limited data that address cancer risk in children with asbestos exposures. The amount of radiation exposure required to develop cancer is also unknown.[4,5,6,7,8] Mesothelioma may present in the thoracic/pleural region or in the peritoneum. These presentations have different clinical courses and prognoses. This cancer can involve the membranous coverings of the lung, the heart, or the abdominal organs.[9,10,11]; [12][Level of evidence C1] Mesothelioma can spread onto organ surfaces without invading far into the underlying tissue. This cancer may also spread to regional or distant lymph nodes. Benign and malignant mesotheliomas cannot be differentiated using histological criteria. Benign mesotheliomas are exceedingly rare and often occur in the peritoneal cavity. A poor prognosis is associated with mesotheliomas that are diffuse and invasive or with mesotheliomas that recur. References: Malignant mesotheliomas found in children, adolescents, and young adults are not often associated with asbestos exposures. This differs from most malignant mesotheliomas seen in adults. Recurring ALK gene fusions have been described in children and adolescents with mesothelioma. These fusions occur most often in female patients with peritoneal primary mesotheliomas. ALK gene fusions involve various partner genes, including STRN, TPM1, and EML4.[1] References: In suspicious cases of malignant mesotheliomas, diagnostic thoracoscopy should be considered to confirm the diagnosis.[1] Cross-sectional imaging may suggest the diagnosis of peritoneal mesothelioma, but diagnostic biopsy by laparoscopy or open laparotomy is required. References: Cancer in children and adolescents is rare, although the overall incidence has slowly increased since 1975.[1] Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team approach incorporates the skills of the following pediatric specialists and others to ensure that children receive treatment, supportive care, and rehabilitation to achieve optimal survival and quality of life: For specific information about supportive care for children and adolescents with cancer, see the summaries on Supportive and Palliative Care. The American Academy of Pediatrics has outlined guidelines for pediatric cancer centers and their role in the treatment of children and adolescents with cancer.[2] At these centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity to participate is offered to most patients and their families. Clinical trials for children and adolescents diagnosed with cancer are generally designed to compare potentially better therapy with current standard therapy. Other types of clinical trials test novel therapies when there is no standard therapy for a cancer diagnosis. Most of the progress in identifying curative therapies for childhood cancers has been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI website. Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2020, childhood cancer mortality decreased by more than 50%.[3,4,5] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. For information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors, see Late Effects of Treatment for Childhood Cancer. Childhood cancer is a rare disease, with about 15,000 cases diagnosed annually in the United States in individuals younger than 20 years.[6] The U.S. Rare Diseases Act of 2002 defines a rare disease as one that affects populations smaller than 200,000 people in the United States. Therefore, all pediatric cancers are considered rare. The designation of a rare tumor is not uniform among pediatric and adult groups. In adults, rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people. They account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[7,8] In children and adolescents, the designation of a rare tumor is not uniform among international groups, as follows: Most cancers in subgroup XI are either melanomas or thyroid cancers, with other cancer types accounting for only 2% of the cancers diagnosed in children aged 0 to 14 years and 9.3% of the cancers diagnosed in adolescents aged 15 to 19 years. These rare cancers are extremely challenging to study because of the relatively few patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the small number of clinical trials for adolescents with rare cancers. Information about these tumors may also be found in sources relevant to adults with cancer, such as Malignant Mesothelioma Treatment. References: Treatment options for pediatric patients with malignant pleural mesotheliomas are controversial. Outcomes are often poor in these individuals despite treatment with radical surgical resection, chemotherapy, and radiation therapy. Treatments that use newer chemotherapy agents and immunotherapies are under investigation.[1] Treatment options for childhood malignant mesothelioma include the following: Surgery Radical surgical resection has been attempted in patients with mesotheliomas, with mixed results.[2] In adults, durable responses may be achieved with multimodal therapy that includes extrapleural pneumonectomy and radiation therapy after combination chemotherapy with pemetrexed-cisplatin.[3][Level of evidence B4] However, this approach remains highly controversial.[4] Chemotherapy The European Cooperative Study Group on Pediatric Rare Tumors retrospectively reviewed children, adolescents, and young adults (aged ≤21 years) with mesotheliomas who were treated between 1987 and 2018.[5] Investigators identified 15 male patients and 18 female patients with mesotheliomas. Only one patient had a documented asbestos exposure. In most patients, the primary tumor was located in the peritoneum (23 patients). Tumor histologies were either multicystic mesothelioma of the peritoneum (6 patients) or malignant mesothelioma (27 patients). Surgery and Hyperthermic Compartmental Chemotherapy Hyperthermic intrapleural/intraperitoneal chemotherapy (HIPEC) has been used to treat pleural and intraperitoneal mesotheliomas. HIPEC, in conjunction with radical surgical resection, has been used to treat adults with pleural mesotheliomas. Although results have been encouraging, HIPEC has not been validated in controlled clinical trials because pleural mesotheliomas are rare.[1,6,7] A single-institution study followed seven children with intraperitoneal mesotheliomas who were treated with surgery and HIPEC.[8] At last available follow-up, five of the seven patients were alive and had either minimal disease or no evaluable disease. Radiation Therapy Pain is an infrequent symptom in patients with mesotheliomas. However, if pain occurs, radiation therapy may be used for palliation. Targeted Therapy (Ceritinib) In one case report, a 13-year-old patient with a peritoneal mesothelioma and a STRN::ALK fusion gene responded to ceritinib treatment.[9] For more information, see Malignant Mesothelioma Treatment. References: Information about National Cancer Institute (NCI)–supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, see the ClinicalTrials.gov website. The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above. This summary was comprehensively reviewed. This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages. Purpose of This Summary This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood mesothelioma. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions. Reviewers and Updates This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH). Board members review recently published articles each month to determine whether an article should: Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary. The lead reviewers for Childhood Mesothelioma Treatment are: Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries. Levels of Evidence Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations. Permission to Use This Summary PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary]." The preferred citation for this PDQ summary is: PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Mesothelioma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/mesothelioma/hp/child-mesothelioma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 31593397] Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images. Disclaimer Based on the strength of the available evidence, treatment options may be described as either "standard" or "under clinical evaluation." These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page. Contact Us More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website's Email Us. Last Revised: 2024-09-16 This information does not replace the advice of a doctor. Ignite Healthwise, LLC disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use and Privacy Policy. Learn how we develop our content. Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Ignite Healthwise, LLC.Topic Contents
Childhood Mesothelioma Treatment (PDQ®): Treatment - Health Professional Information [NCI]
Incidence, Risk Factors, and Clinical Presentation
Genomic Alterations
Diagnostic Evaluation
Special Considerations for the Treatment of Children With Cancer
Treatment of Childhood Mesothelioma
Treatment Options Under Clinical Evaluation for Childhood Mesothelioma
Latest Updates to This Summary (09 / 16 / 2024)
About This PDQ Summary
Our Health Library information does not replace the advice of a doctor. Please be advised that this information is made available to assist our patients to learn more about their health. Our providers may not see and/or treat all topics found herein. This information does not replace the advice of a doctor. Ignite Healthwise, LLC disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use and Privacy Policy. Learn how we develop our content. Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Ignite Healthwise, LLC.Childhood Mesothelioma Treatment (PDQ®): Treatment - Health Professional Information [NCI]