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医療専門家向け Childhood Mesothelioma Treatment (PDQ®)

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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 who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.

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).

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Incidence, Risk Factors, and Clinical Presentation

Mesothelioma is extremely rare in childhood, 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 develop after successful treatment of an earlier cancer, especially after treatment with radiation.[ 3 ][ 4 ] The amount of exposure required to develop cancer is unknown. In adults, these tumors have been associated with exposure to asbestos, which was used as building insulation.[ 5 ] There is no information about the risk for children exposed to asbestos.

This tumor can involve the membranous coverings of the lung, the heart, or the abdominal organs.[ 6 ][ 7 ][ 8 ]; [ 9 ][Level of evidence: 3iiA] 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.

参考文献
  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. Hofmann J, Mintzer D, Warhol MJ: Malignant mesothelioma following radiation therapy. Am J Med 97 (4): 379-82, 1994.[PUBMED Abstract]
  4. Pappo AS, Santana VM, Furman WL, et al.: Post-irradiation malignant mesothelioma. Cancer 79 (1): 192-3, 1997.[PUBMED Abstract]
  5. Hyers TM, Ohar JM, Crim C: Clinical controversies in asbestos-induced lung diseases. Semin Diagn Pathol 9 (2): 97-101, 1992.[PUBMED Abstract]
  6. Kelsey A: Mesothelioma in childhood. Pediatr Hematol Oncol 11 (5): 461-2, 1994 Sep-Oct.[PUBMED Abstract]
  7. 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]
  8. Cioffredi LA, Jänne PA, Jackman DM: Treatment of peritoneal mesothelioma in pediatric patients. Pediatr Blood Cancer 52 (1): 127-9, 2009.[PUBMED Abstract]
  9. 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]
Prognosis

Benign and malignant mesotheliomas cannot be differentiated using histologic criteria. A poor prognosis is associated with lesions that are diffuse and invasive and with those that recur. In general, the course of the disease is slow, and long-term survival is common.

A French retrospective review identified 14 cases of peritoneal mesothelioma in patients younger than 18 years. A variety of treatments were employed, including surgery, heated intraperitoneal chemotherapy, and systemic chemotherapy. At a median follow up of 7 years, 8 of 14 patients experienced recurrences or disease progression.[ 1 ][Level of evidence: 3iiA]

参考文献
  1. 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]
Diagnostic Evaluation

Diagnostic thoracoscopy should be considered in suspicious cases to confirm diagnosis.[ 1 ]

参考文献
  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]
Treatment of Childhood Mesothelioma

Treatment options for childhood malignant mesothelioma include the following:

  1. Surgery.
  2. Chemotherapy.
  3. Radiation therapy.

Radical surgical resection has been attempted with mixed results.[ 1 ] In adults, a multimodal therapy including extrapleural pneumonectomy and radiation therapy after combination chemotherapy with pemetrexed-cisplatin may achieve durable responses.[ 2 ][Level of evidence: 2A] However, this approach remains highly controversial.[ 3 ] In children, treatment with various chemotherapeutic agents used for carcinomas or sarcomas may result in partial responses.[ 4 ][ 5 ][ 6 ][ 7 ]

Hyperthermic chemotherapy has been used to treat adults with pleural mesothelioma.[ 8 ][ 9 ]

Pain is an infrequent symptom; however, if pain occurs, radiation therapy may be used for palliation.

Papillary serous carcinoma of the peritoneum may be mistaken for mesothelioma.[ 10 ] This tumor generally involves all surfaces lining the abdominal organs, including the surfaces of the ovary. Treatment includes surgical resection whenever possible and use of chemotherapy with agents such as cisplatin, carboplatin, and paclitaxel.

(Refer to the PDQ summary on Malignant Mesothelioma Treatment [Adult] for more information.)

参考文献
  1. 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]
  2. 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]
  3. 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]
  4. Milano E, Pourroy B, Rome A, et al.: Efficacy of a combination of pemetrexed and multiple redo-surgery in an 11-year-old girl with a recurrent multifocal abdominal mesothelioma. Anticancer Drugs 17 (10): 1231-4, 2006.[PUBMED Abstract]
  5. Cioffredi LA, Jänne PA, Jackman DM: Treatment of peritoneal mesothelioma in pediatric patients. Pediatr Blood Cancer 52 (1): 127-9, 2009.[PUBMED Abstract]
  6. Sugalski A, Davis M, Prasannan L, et al.: Clinical, histologic, and genetic features of mesothelioma in a 7-year-old child. Pediatr Blood Cancer 60 (1): 146-8, 2013.[PUBMED Abstract]
  7. Kobayashi S, Waragai T, Sano H, et al.: Malignant peritoneal mesothelioma in a child: chemotherapy with gemcitabine and platinum was effective for the disease unresponsive to other treatments. Anticancer Drugs 25 (9): 1102-5, 2014.[PUBMED Abstract]
  8. Nguyen D, Sugarbaker DJ, Burt BM: Therapeutic R2 resection for pleural mesothelioma. J Thorac Cardiovasc Surg 155 (6): 2734-2735, 2018.[PUBMED Abstract]
  9. Wald O, Sugarbaker DJ: New Concepts in the Treatment of Malignant Pleural Mesothelioma. Annu Rev Med 69: 365-377, 2018.[PUBMED Abstract]
  10. Wall JE, Mandrell BN, Jenkins JJ, et al.: Effectiveness of paclitaxel in treating papillary serous carcinoma of the peritoneum in an adolescent. Am J Obstet Gynecol 172 (3): 1049-52, 1995.[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, refer to the ClinicalTrials.gov website.

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

(Refer to the PDQ summary on Malignant Mesothelioma Treatment [Adult] for more information.)

Special Considerations for the Treatment of Children With Cancer

Cancer in children and adolescents is rare, although the overall incidence of childhood cancer 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 for children and adolescents with cancer. 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:

(Refer to the PDQ Supportive and Palliative Care summaries for specific information about supportive care for children and adolescents with cancer.)

Guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer have been outlined by the American Academy of Pediatrics.[ 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 in these trials 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 therapy that is currently accepted as standard. 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 cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)

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 persons. Therefore, all pediatric cancers are considered rare.

The designation of a rare tumor is not uniform among pediatric and adult groups. Adult rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people, and they are estimated to 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:

These rare cancers are extremely challenging to study because of the low incidence 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 the PDQ summary on Malignant Mesothelioma Treatment (Adult).

参考文献
  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. Corrigan JJ, Feig SA; American Academy of Pediatrics: Guidelines for pediatric cancer centers. Pediatrics 113 (6): 1833-5, 2004.[PUBMED Abstract]
  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, Bisogno G, De Salvo GL, et al.: The challenge of very rare tumours in childhood: the Italian TREP project. Eur J Cancer 43 (4): 654-9, 2007.[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. Bethesda, Md: National Cancer Institute, 2015. Also available online. Last accessed February 20, 2020.[PUBMED Abstract]
Changes to This Summary (06/08/2020)

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.

Incidence, Risk Factors, and Clinical Presentation

Added Vermersch et al. as reference 9 and level of evidence 3iiA.

Prognosis

Added text to state that a French retrospective review identified 14 cases of peritoneal mesothelioma in patients younger than 18 years. A variety of treatments were employed, including surgery, heated intraperitoneal chemotherapy, and systemic chemotherapy. At a median follow up of 7 years, 8 of 14 patients experienced recurrences or disease progression (cited Vermersch et al. as reference 1 and level of evidence 3iiA).

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 who care for cancer 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.

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

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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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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.

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