Radiation, either external beam or P-32, may be used when the tumor cannot be surgically removed.
High-dose chemotherapy with stem cell rescue has been used without success.
There are no standard treatment options. Current treatment regimens have been informed by consensus conferences. The rare occurrence of these tumors makes recommending treatment difficult. Some general treatment considerations from the Pleuropulmonary Blastoma Registry include the following:
- Type I: Surgery alone for select cases; adjuvant chemotherapy may decrease recurrences.[40,58] Evidence suggests a close histologic relationship between a Type 4 cystic adenomatoid malformation and a Type I pleuropulmonary blastoma.[59,60] Complete surgical lobectomy is adequate treatment for these patients, but close observation is recommended.
- Type II and Type III: Surgery followed by chemotherapy.
An independent group of researchers has established a registry and resource Web site for this rare tumor.
Incidence and histology
Esophageal cancer is rare in the pediatric age group, although it is relatively common in older adults.[61,62] Most of these tumors are squamous cell carcinomas, although sarcomas can also arise in the esophagus. The most common benign tumor is leiomyoma.
Clinical presentation and diagnostic evaluation
Symptoms are related to difficulty in swallowing and associated weight loss. 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 is generally poor for this cancer, which rarely can be completely resected.
(Refer to the PDQ summary on adult Esophageal Cancer Treatment for more information.)
Thymoma and Thymic Carcinoma
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.[63,64,65] The term thymoma is customarily used to describe neoplasms that show no overt atypia of the epithelial component. Thymic carcinomas have a higher incidence of capsular invasion and metastases. 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.
Incidence and risk factors
Thymoma and thymic carcinomas are very rare in children.[66,67,68] In the Tumori Rari in Età Pediatrica (TREP) registry, only eight cases were identified over a 9-year period.
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.[70,71] Endocrine (hormonal) disorders including hyperthyroidism, Addison disease, and panhypopituitarism can also be associated with a diagnosis of thymoma.