Breast Cancer
Bronchial Tumors
Pleuropulmonary Blastoma
Esophageal Tumors
Thymoma and Thymic Carcinoma
Tumors of the Heart
Mesothelioma

Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)

Thoracic cancers include breast cancer, bronchial adenomas, bronchial carcinoid tumors, pleuropulmonary blastoma, esophageal tumors, thymomas, thymic carcinomas, tumors of the heart, and mesothelioma. The prognosis, diagnosis, classification, and treatment of these thoracic cancers are discussed below.

The most frequent breast tumor seen in children is a fibroadenoma.[1] These tumors can be observed and many will regress without a need for biopsy. However, rare malignant transformation leading to phyllodes tumors has been reported.[2] Sudden rapid enlargement of a suspected fibroadenoma is an indication for needle biopsy or excision. Phyllodes tumors can be managed by wide local excision without mastectomy.[2]

Carcinomas have been reported in both males and females younger than 21 years.[3-8] There is an increased lifetime risk of breast cancer in female survivors of Hodgkin lymphoma who were treated with radiation to the chest area, however, breast cancer is also seen in patients who were treated for any cancer that was treated with chest irradiation.[7,9-11] (Refer to the PDQ summary on the Late Effects of Treatment for Childhood Cancer for more information about secondary breast cancers.) Carcinomas are more frequent than sarcomas. Mammograms should start at age 25 years or 10 years postexposure to radiation therapy (whichever came last). Treatment options for children and adolescents with breast cancer include radiation, chemotherapy, and surgery. Breast tumors may also occur as metastatic deposits from leukemia, rhabdomyosarcoma, other sarcomas, or lymphoma (particularly in patients who are infected with the human immunodeficiency virus). (Refer to the PDQ summary on adult Breast Cancer Treatment for more information.)

Bronchial tumors are a heterogeneous group of primary endobronchial lesions, and though adenoma implies a benign process, all varieties of bronchial tumors on occasion display a malignant behavior. There are three histologic types. The most frequent type is a carcinoid tumor; this is followed by mucoepidermoid carcinoma and adenoid cystic carcinoma. Carcinoid tumors account for 80% to 85% of all bronchial tumors in children.[12-16] The presenting symptoms are usually because of an incomplete bronchial obstruction with a cough, recurrent pneumonitis, and hemoptysis. Because of difficulties in diagnosis, symptoms are frequently present for months and occasionally children with wheezing have been treated for asthma with delays in diagnosis as long as 4 to 5 years. Metastatic lesions are reported in approximately 6% of cases and recurrences occur in 2% of cases. Atypical carcinoid tumors are rare but more aggressive with 50% of patients presenting with metastatic disease at diagnosis.[17] There is a single report of a child with a carcinoid tumor and metastatic disease who developed the classic carcinoid syndrome.[18] Octreotide nuclear scans may demonstrate uptake of radioactivity by the tumor or lymph nodes, suggesting metastatic spread. Bronchial tumors of all histologic types are associated with an excellent prognosis in children, even in the presence of local invasion.[19,20] The management of bronchial tumors is somewhat controversial because all bronchial tumors are usually visible endoscopically. Biopsy in these lesions may be hazardous because of hemorrhage, and endoscopic resection is not recommended. Bronchography or computed tomography scan may be helpful to determine the degree of bronchiectasis distal to the obstruction since the degree of pulmonary destruction may influence surgical therapy.[21] Epithelial cancers of the lung are rare in children. When they do occur, they tend to be of advanced stage with prognosis dependent on both histology and stage.[17]

Conservative pulmonary resection with the removal of the involved lymphatics is the treatment of choice. Sleeve segmental bronchial resection is possible in children and when feasible, is the treatment of choice.[22,23] Adenoid cystic carcinomas (cylindroma) have a tendency to spread submucosally, and late local recurrence or dissemination has been reported. In addition to en bloc resection with hilar lymphadenectomy, a frozen section examination of the bronchial margins should be carried out in children with this lesion. Neither chemotherapy nor radiation therapy is indicated for bronchial tumors, unless evidence of metastasis is documented.

Pleuropulmonary blastoma is a rare and highly aggressive pulmonary malignancy in children. Pleuropulmonary blastoma appears to progress through stages with the earliest stage (type I) being a purely lung cystic neoplasm with subtle malignant changes, typically occurring in the first 2 years of life with a good prognosis,[24][Level of evidence: 3iiiA] followed by the more aggressive stages: type II (cystic and solid neoplasm) and type III (purely solid neoplasm).[25,26] There have been reports of type I transitioning directly to type III.[27] Cerebral metastasis occurs in up to 50% of patients with type III tumors.[28] An independent group of researchers has established a registry and resource Web site for this rare tumor.[29] An association between congenital lung cysts and pleuropulmonary blastoma has been reported, although cytogenetic and molecular studies can help distinguish the nonneoplastic congenital cystic adenomatoid malformation from pleuropulmonary blastoma.[30-34] Comparative genomic and fluorescent in situ hybridization methods have identified gain of chromosome 8q as the main recurrent chromosomal abnormality in pleuropulmonary blastoma.[[35]][[36]] The tumor is usually located in the lung periphery, but it may be extrapulmonary with involvement of the mediastinum, diaphragm, and/or pleura.[33,37] The tumors may recur or metastasize, in spite of primary resection.[[24]][26] Responses to chemotherapy have been reported with agents similar to those used for the treatment of rhabdomyosarcoma, and adjuvant chemotherapy may benefit patients with type I pleuropulmonary blastoma by reducing the risk of recurrence.[25,38] Achieving total resection of the tumor at any time during treatment is associated with improved prognosis.[33] Chemotherapeutic agents may include vincristine, cyclophosphamide, dactinomycin, and doxorubicin. High-dose chemotherapy with stem cell rescue has been used without success.[39] Radiation, either external beam or P-32, may be used when the tumor cannot be surgically removed. A family history of cancer in close relatives has been noted for many young patients affected by this tumor.[40] In addition, there has been a reported association between pleuropulmonary blastoma and cystic nephroma.[41,42] Data from the International Pleuropulmonary Blastoma Registry suggest that adjuvant chemotherapy may reduce the risk of recurrence.[25]

Esophageal cancer is rare in the pediatric age group, though it is relatively common in older adults.[43] Symptoms are related to difficulty in swallowing and associated weight loss. Most of these tumors are squamous cell carcinomas, though sarcomas can also arise in the esophagus. The most common benign tumor is leiomyoma. Diagnosis is made by histologic examination of biopsy tissue.

Treatment options for esophageal carcinoma include either external-beam intracavitary radiation therapy or chemotherapy agents commonly used to treat carcinomas: platinum derivatives, paclitaxel, and etoposide. Prognosis generally is poor for this cancer, which rarely can be completely resected. (Refer to the PDQ summary on adult Esophageal Cancer Treatment for more information.)

A cancer of the thymus is not considered a thymoma or a thymic carcinoma unless there are neoplastic changes of the epithelial cells that cover the organ.[44,45] The term thymoma is customarily used to describe neoplasms that show no overt atypia of the epithelial component. A thymic epithelial tumor that exhibits clear-cut cytologic atypia and histologic features no longer specific to the thymus is known as thymic carcinoma, also known as type C thymoma. Other tumors that involve the thymus gland include lymphomas, germ cell tumors, carcinomas, carcinoids, and thymomas. Hodgkin lymphoma and non-Hodgkin lymphoma may also involve the thymus and must be differentiated from true thymomas and thymic carcinomas.

Thymoma and thymic carcinomas are rare in adults and children.[46,47] Various diseases and syndromes are associated with thymoma, including myasthenia gravis, polymyositis, systemic lupus erythematosus, rheumatoid arthritis, thyroiditis, Isaacs syndrome or neuromyotonia (continuous muscle stiffness resulting from persistent muscle activity as a consequence of antibodies against voltage-gated potassium channels), and pure red-cell aplasia.[48,49] Endocrine (hormonal) disorders including hyperthyroidism, Addison disease, and panhypopituitarism can also be associated with a diagnosis of thymoma.[50]

These neoplasms are usually located in the front part of the chest and are usually discovered during a routine chest x-ray. Symptoms can include cough, difficulty with swallowing, tightness of the chest, chest pain, and shortness of breath, though nonspecific symptoms may occur. These tumors generally are slow growing but are potentially invasive, with metastases to distant organs or lymph nodes. Staging is related to invasiveness. Surgery is performed with the goal of a complete resection.

Radiation therapy is necessary for patients with invasive thymoma or thymic carcinoma, even with a complete resection.[50] Chemotherapy is usually reserved for patients with advanced-stage disease who have not responded to radiation therapy or corticosteroids. Agents that have been effective include doxorubicin, cisplatin, and paclitaxel.[50-52] The prognosis for patients with invasive thymoma or thymic carcinoma usually is poor, though significantly higher rates of survival have been reported for patients with tumors that are not locally invasive. (Refer to the PDQ summary on adult Thymoma and Thymic Carcinoma Treatment for more information.)

Researchers have described a group of young patients with midline carcinomas with a very poor prognosis. The tumors arise in midline epithelial structures including the thymus, mediastinum, airway structures, and bladder. They exhibit squamous differentiation. Tumors from 8 of 11 patients exhibited a balanced chromosomal translocation t(15;19) involving the BRD4 and NUT genes. These patients had no response to chemotherapy and died very quickly. Tumors from the remaining three patients had a chromosomal break in the NUT gene on chromosome 15 but had normal chromosome 19. These patients were older and had a slightly longer survival than the eight patients exhibiting t(15;19).[53]

The most common tumors of the heart are benign and include myxomas, rhabdomyomas, and neurofibromas (i.e., tumors of the nerves that innervate the muscles).[54-56] Primary tumors of the heart may include benign and malignant teratomas, rhabdomyosarcomas, hemangiomas, and chondrosarcomas. Multiple cardiac tumors noted in the fetal or neonatal period are highly associated with a diagnosis of tuberous sclerosis.[54] In a retrospective review of 94 patients with cardiac tumors detected by prenatal or neonatal echocardiography, 68% of the patients exhibited features of tuberous sclerosis.[57] In another study, 79% (15out of 19) of patients with rhabdomyomas discovered prenatally had tuberous sclerosis, while 96% of those diagnosed postnatally had tuberous sclerosis. Most rhabdomyomas, whether diagnosed prenatally or postnatally, will spontaneously regress.[58] Other tumors of the heart can include metastatic spread of rhabdomyosarcoma, melanoma, leukemia, and carcinoma of other sites. Symptoms include abnormalities of heart rhythm, enlargement of the heart, fluid in the pericardial sac, and congestive heart failure. Successful treatment may require surgery, which may include transplantation, and chemotherapy appropriate for the type of cancer that is present.[59,60]

Mesothelioma is extremely rare in childhood with only 2% to 5% of patients presenting during the first two decades of life.[61]

This tumor can involve the membranous coverings of the lung, the heart, or the abdominal organs.[62] 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.[63,64] In adults, these tumors have been associated with exposure to asbestos, which was used as building insulation.[65] The amount of exposure required to develop cancer is unknown, and there is no information about the risk for children exposed to asbestos.

Benign and malignant mesotheliomas cannot be differentiated using histologic criteria. A poor prognosis is associated with lesions that are diffuse and invasive or for those that recur. In general, the course of the disease is slow, and long-term survival is common. Diagnostic thoracoscopy should be considered in suspicious cases to confirm diagnosis.[61] Radical surgical resection has been attempted with mixed results.[66] Treatment with various chemotherapeutic agents used for carcinomas or sarcomas may result in partial responses.[67] 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.[68] 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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