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Childhood Mesothelioma Treatment (PDQ®)–Health Professional Version

Incidence, Risk Factors, and Clinical Presentation

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] Mesothelioma may present in the thoracic/pleural region or in the peritoneum, and the two presentations have different clinical courses and prognoses. In adults, these tumors have been associated with exposure to asbestos, exposure to radiation, and inherited mutations of the BAP1 gene. These risk factors are rare in pediatric patients.[3-6]

Mesothelioma may develop after successful treatment of an earlier cancer, especially after treatment with radiation therapy.[3,7,8] The amount of radiation exposure required to develop cancer is unknown. There is no information about the risk in children who are exposed to asbestos.

This tumor can involve the membranous coverings of the lung, the heart, or the abdominal organs.[9-11]; [12][Level of evidence C1] These tumors can spread over the surface of organs, without invading far into the underlying tissue, and may spread to regional or distant lymph nodes.

Biologically, malignant mesothelioma occurring in children, adolescents, and young adults differs from its adult counterpart because there is no relationship to asbestos exposure. Recurrent ALK gene fusions have been described in children and adolescents with mesothelioma, and they more commonly occur in female patients with peritoneal primary tumors. ALK gene fusions involve various partners, including STRN, TPM1, and EML4.[13]

References
  1. Nagata S, Nakanishi R: Malignant pleural mesothelioma with cavity formation in a 16-year-old boy. Chest 127 (2): 655-7, 2005. [PUBMED Abstract]
  2. Rosas-Salazar C, Gunawardena SW, Spahr JE: Malignant pleural mesothelioma in a child with ataxia-telangiectasia. Pediatr Pulmonol 48 (1): 94-7, 2013. [PUBMED Abstract]
  3. Orbach D, André N, Brecht IB, et al.: Mesothelioma in children and adolescents: the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) contribution. Eur J Cancer 140: 63-70, 2020. [PUBMED Abstract]
  4. Tsao AS, Wistuba I, Roth JA, et al.: Malignant pleural mesothelioma. J Clin Oncol 27 (12): 2081-90, 2009. [PUBMED Abstract]
  5. Carbone M, Ferris LK, Baumann F, et al.: BAP1 cancer syndrome: malignant mesothelioma, uveal and cutaneous melanoma, and MBAITs. J Transl Med 10: 179, 2012. [PUBMED Abstract]
  6. Janes SM, Alrifai D, Fennell DA: Perspectives on the Treatment of Malignant Pleural Mesothelioma. N Engl J Med 385 (13): 1207-1218, 2021. [PUBMED Abstract]
  7. Hofmann J, Mintzer D, Warhol MJ: Malignant mesothelioma following radiation therapy. Am J Med 97 (4): 379-82, 1994. [PUBMED Abstract]
  8. Pappo AS, Santana VM, Furman WL, et al.: Post-irradiation malignant mesothelioma. Cancer 79 (1): 192-3, 1997. [PUBMED Abstract]
  9. Kelsey A: Mesothelioma in childhood. Pediatr Hematol Oncol 11 (5): 461-2, 1994 Sep-Oct. [PUBMED Abstract]
  10. Moran CA, Albores-Saavedra J, Suster S: Primary peritoneal mesotheliomas in children: a clinicopathological and immunohistochemical study of eight cases. Histopathology 52 (7): 824-30, 2008. [PUBMED Abstract]
  11. Cioffredi LA, Jänne PA, Jackman DM: Treatment of peritoneal mesothelioma in pediatric patients. Pediatr Blood Cancer 52 (1): 127-9, 2009. [PUBMED Abstract]
  12. Vermersch S, Arnaud A, Orbach D, et al.: Multicystic and diffuse malignant peritoneal mesothelioma in children. Pediatr Blood Cancer 67 (6): e28286, 2020. [PUBMED Abstract]
  13. Argani P, Lian DWQ, Agaimy A, et al.: Pediatric Mesothelioma With ALK Fusions: A Molecular and Pathologic Study of 5 Cases. Am J Surg Pathol 45 (5): 653-661, 2021. [PUBMED Abstract]

Prognosis

Benign and malignant mesotheliomas cannot be differentiated using histological criteria. Benign tumors are exceedingly rare and are more common in the peritoneal cavity. A poor prognosis is associated with lesions that are diffuse and invasive or that recur.

Diagnostic Evaluation

Diagnostic thoracoscopy should be considered in suspicious cases to confirm diagnosis.[1] Cross-sectional imaging may suggest the diagnosis of peritoneal mesothelioma, but diagnostic biopsy by laparoscopy or open laparotomy is required.

References
  1. Nagata S, Nakanishi R: Malignant pleural mesothelioma with cavity formation in a 16-year-old boy. Chest 127 (2): 655-7, 2005. [PUBMED Abstract]

Special Considerations for the Treatment of Children With Cancer

Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975.[1] Referral to medical centers with multidisciplinary teams of cancer specialists experienced in treating cancers that occur in childhood and adolescence should be considered. This multidisciplinary team approach incorporates the skills of the following health care professionals and others to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life:

  • Primary care physicians.
  • Pediatric surgeons.
  • Radiation oncologists.
  • Pediatric medical oncologists/hematologists.
  • Rehabilitation specialists.
  • Pediatric nurse specialists.
  • Social workers.
  • Child-life professionals.
  • Psychologists.

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 pediatric patients with cancer.[2] At these pediatric cancer 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. Most of the progress made in identifying curative therapy 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 2010, childhood cancer mortality decreased by more than 50%.[3] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. For specific 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.[4] The U.S. Rare Diseases Act of 2002 defines a rare disease as one that affects populations smaller than 200,000 people. 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.[5,6] Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:

  • A consensus effort between the European Union Joint Action on Rare Cancers and the European Cooperative Study Group for Rare Pediatric Cancers estimated that 11% of all cancers in patients younger than 20 years could be categorized as very rare. This consensus group defined very rare cancers as those with annual incidences of fewer than 2 cases per 1 million people. However, three additional histologies (thyroid carcinoma, melanoma, and testicular cancer) with incidences of more than 2 cases per 1 million people were also included in the very rare group because there is a lack of knowledge and expertise in the management of these tumors.[7]
  • The Children's Oncology Group defines rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancer, melanoma and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinoma, nasopharyngeal carcinoma, and most adult-type carcinomas such as breast cancer, colorectal cancer, etc.).[8] These diagnoses account for about 4% of cancers diagnosed in children aged 0 to 14 years, compared with about 20% of cancers diagnosed in adolescents aged 15 to 19 years.[9]

    Most cancers in subgroup XI are either melanomas or thyroid cancer, with other types accounting for only 1.3% of cancers in children aged 0 to 14 years and 5.3% of cancers in adolescents aged 15 to 19 years.

These rare cancers are extremely challenging to study because of the low number of patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the lack 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 (Adult).

References
  1. Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010. [PUBMED Abstract]
  2. American Academy of Pediatrics: Standards for pediatric cancer centers. Pediatrics 134 (2): 410-4, 2014. Also available online. Last accessed June 7, 2022.
  3. Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014. [PUBMED Abstract]
  4. Ward E, DeSantis C, Robbins A, et al.: Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 64 (2): 83-103, 2014 Mar-Apr. [PUBMED Abstract]
  5. Gatta G, Capocaccia R, Botta L, et al.: Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 18 (8): 1022-1039, 2017. [PUBMED Abstract]
  6. DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017. [PUBMED Abstract]
  7. Ferrari A, Brecht IB, Gatta G, et al.: Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. Eur J Cancer 110: 120-126, 2019. [PUBMED Abstract]
  8. Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010. [PUBMED Abstract]
  9. Howlader N, Noone AM, Krapcho M, et al., eds.: SEER Cancer Statistics Review, 1975-2012. National Cancer Institute, 2015. Also available online. Last accessed May 25, 2022.

Treatment of Childhood Mesothelioma

Treatment options for childhood malignant mesothelioma include the following:

  1. Surgery.
  2. Chemotherapy.
  3. Radiation therapy.
  4. Surgery and hyperthermic compartmental chemotherapy.

The treatment of patients with malignant pleural mesothelioma is controversial. Outcomes for patients are poor despite treatment with radical surgical resection, chemotherapy, and radiation therapy. Treatment with newer agents and immunotherapy is under investigation.[1]

Radical surgical resection has been attempted with mixed results.[2] In adults, multimodal therapy that includes extrapleural pneumonectomy and radiation therapy after combination chemotherapy with pemetrexed-cisplatin may achieve durable responses.[3][Level of evidence B4] However, this approach remains highly controversial.[4]

Hyperthermic intrapleural chemotherapy, in conjunction with radical surgical resection, has been used to treat adults with pleural mesothelioma. Although results have been encouraging, hyperthermic intrapleural chemotherapy has not been validated in controlled clinical trials because of the rarity of the tumor.[1,5,6] A single-institution experience reported on seven children with intraperitoneal mesothelioma who were treated with surgery and hyperthermic intraperitoneal chemotherapy.[7] At last available follow-up, five of the seven patients were alive with minimal or no evaluable disease.

The European Cooperative Study Group on Pediatric Rare Tumors retrospectively reviewed children, adolescents, and young adults (aged ≤21 years) diagnosed with mesothelial tumors and treated between 1987 and 2018.[8] Investigators identified 15 male patients and 18 female patients. Only one patient had documented exposure to asbestos. The primary tumor was mainly in the peritoneum (23 patients). The histology was either multicystic mesothelioma of the peritoneum (6 patients) or malignant mesothelioma (27 patients). The response rate to treatment with cisplatin-pemetrexed was 50% (6 of 12 cases). After a median follow-up of 6.7 years (range, 0–20 years), the 5-year overall survival rate was 82.3%, and the event-free survival rate was 45.1%. All patients with multicystic mesothelioma were alive after surgery (5 patients) and cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (1 patient).

In one case report, a patient aged 13 years with a peritoneal mesothelioma and a STRN-ALK fusion responded to ceritinib treatment.[9]

Pain is an infrequent symptom. However, if it occurs, radiation therapy may be used for palliation.

For more information, see Malignant Mesothelioma Treatment (Adult).

References
  1. Carbone M, Adusumilli PS, Alexander HR, et al.: Mesothelioma: Scientific clues for prevention, diagnosis, and therapy. CA Cancer J Clin 69 (5): 402-429, 2019. [PUBMED Abstract]
  2. Maziak DE, Gagliardi A, Haynes AE, et al.: Surgical management of malignant pleural mesothelioma: a systematic review and evidence summary. Lung Cancer 48 (2): 157-69, 2005. [PUBMED Abstract]
  3. Krug LM, Pass HI, Rusch VW, et al.: Multicenter phase II trial of neoadjuvant pemetrexed plus cisplatin followed by extrapleural pneumonectomy and radiation for malignant pleural mesothelioma. J Clin Oncol 27 (18): 3007-13, 2009. [PUBMED Abstract]
  4. Treasure T: What is the best approach for surgery of malignant pleural mesothelioma? It is to put our efforts into obtaining trustworthy evidence for practice. J Thorac Cardiovasc Surg 151 (2): 307-9, 2016. [PUBMED Abstract]
  5. Nguyen D, Sugarbaker DJ, Burt BM: Therapeutic R2 resection for pleural mesothelioma. J Thorac Cardiovasc Surg 155 (6): 2734-2735, 2018. [PUBMED Abstract]
  6. Wald O, Sugarbaker DJ: New Concepts in the Treatment of Malignant Pleural Mesothelioma. Annu Rev Med 69: 365-377, 2018. [PUBMED Abstract]
  7. Malekzadeh P, Good M, Hughes MS: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) with cisplatin in pediatric patients with peritoneal mesothelioma: a single institution experience and long term follow up. Int J Hyperthermia 38 (1): 326-331, 2021. [PUBMED Abstract]
  8. Orbach D, André N, Brecht IB, et al.: Mesothelioma in children and adolescents: the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) contribution. Eur J Cancer 140: 63-70, 2020. [PUBMED Abstract]
  9. Rüschoff JH, Gradhand E, Kahraman A, et al.: STRN -ALK Rearranged Malignant Peritoneal Mesothelioma With Dramatic Response Following Ceritinib Treatment. JCO Precis Oncol 3: , 2019. [PUBMED Abstract]

Treatment Options Under Clinical Evaluation for Childhood Mesothelioma

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 following is an example of a national and/or institutional clinical trial that is currently being conducted:

  • APEC1621 (NCT03155620) (Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients with Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders): NCI-COG Pediatric Molecular Analysis for Therapeutic Choice (MATCH), referred to as Pediatric MATCH, will match targeted agents with specific molecular changes identified in a patient's tumor (refractory or recurrent). Children and adolescents aged 1 to 21 years are eligible for the trial.

    Patients with tumors that have molecular variants addressed by treatment arms included in the trial will be offered treatment on Pediatric MATCH. Additional information can be obtained on the NCI website and ClinicalTrials.gov website.

Changes to This Summary (06/08/2022)

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.

Editorial changes were made to this summary.

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® - NCI's Comprehensive Cancer Database pages.

About This PDQ Summary

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:

  • be discussed at a meeting,
  • be cited with text, or
  • replace or update an existing article that is already cited.

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:

  • Denise Adams, MD (Children's Hospital Boston)
  • Karen J. Marcus, MD, FACR (Dana-Farber Cancer Institute/Boston Children's Hospital)
  • Paul A. Meyers, MD (Memorial Sloan-Kettering Cancer Center)
  • Thomas A. Olson, MD (Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta - Egleston Campus)
  • Alberto S. Pappo, MD (St. Jude Children's Research Hospital)
  • Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)
  • Carlos Rodriguez-Galindo, MD (St. Jude Children's Research Hospital)
  • Stephen J. Shochat, MD (St. Jude Children's Research Hospital)

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.

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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>.

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