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Unusual Cancers of Childhood Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Thoracic Cancers

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Clinical presentation and diagnostic evaluation

Patients may be asymptomatic for long periods. Symptoms may include abnormalities of heart rhythm, enlargement of the heart, fluid in the pericardial sac, and congestive heart failure. Some patients present with sudden death.

The utilization of new cardiac MRI techniques can identify the likely tumor type in the majority of children.[94] However, histologic diagnosis remains the standard for diagnosing cardiac tumors.

Treatment

Successful treatment may require surgery, including transplantation, and chemotherapy appropriate for the type of cancer that is present.[95,96,97]; [79][Level of evidence: 3iiA]

Mesothelioma

Incidence and risk factors

Mesothelioma is extremely rare in childhood, with only 2% to 5% of patients presenting during the first two decades of life.[98] Fewer than 300 cases in children have been reported.[99]

This tumor can involve the membranous coverings of the lung, the heart, or the abdominal organs.[100,101,102] 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. Mesothelioma may develop after successful treatment of an earlier cancer, especially after treatment with radiation.[103,104] In adults, these tumors have been associated with exposure to asbestos, which was used as building insulation.[105] The amount of exposure required to develop cancer is unknown, and there is no information about the risk for children exposed to asbestos.

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.

Diagnostic evaluation

Diagnostic thoracoscopy should be considered in suspicious cases to confirm diagnosis.[98]

Treatment

Radical surgical resection has been attempted with mixed results.[106] Treatment with various chemotherapeutic agents used for carcinomas or sarcomas may result in partial responses.[102,107] Pain is an infrequent symptom; however, radiation therapy may be used for palliation of pain.

Papillary serous carcinoma of the peritoneum is sometimes mistaken for mesothelioma.[108] 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 adult Malignant Mesothelioma Treatment for more information.)

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