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Unusual Cancers of Childhood Treatment (PDQ®)

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Changes to This Summary (04/06/2015)

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.

General Information About Unusual Cancers of Childhood

Added Corrigan et al. as reference 2.

Head and Neck Cancers

Added text to state that a French registry analysis found similar outcomes in children and young adults who developed papillary thyroid carcinoma after previous radiation therapy compared with children and young adults who developed spontaneous papillary thyroid carcinoma; patients with previous thyroid irradiation for benign disease, however, presented with more invasive tumors and lymph node involvement (cited Sassolas et al. as reference 68).

Added text to state that the median duration of therapy for the entire cohort was 27 months, with a range of 2 to 52 months.

Added Treatment options under clinical evaluation for medullary thyroid carcinoma as a new subsection.

Revised text to state that the most common malignant salivary gland tumor in children is mucoepidermoid carcinoma, followed by acinic cell carcinoma, and adenoid cystic carcinoma; less common malignancies include rhabdomyosarcoma, adenocarcinoma, and undifferentiated carcinoma (cited Aro et al. as reference 141).

Thoracic Cancers

Added text to state that a review of 48 published cases of thymoma in patients younger than 18 years, excluding thymic carcinoma, found an association between stage of disease and survival; it also suggested guidelines for treatment. The overall 2-year survival in this series was 71% (cited Fonseca et al. as reference 72).

Added text to state that most children present with low-stage disease.

Added text to state that rarely, synovial sarcoma may arise in the heart or pericardium (cited Wang et al. as reference 97).

Abdominal Cancers

Added text to state that the likelihood of a TP53 germline mutation is highest in the first years of life and diminishes with age (cited Wasserman et al. as reference 14).

Added text to state that the Italian Rare Tumors in Pediatric Age project enrolled 133 children younger than 18 years with appendiceal neuroendocrine tumors between 2000 and 2013; they found no relapses or deaths in this cohort after appendectomy for incompletely resected tumors (cited Virgone et al. as reference 120 and level of evidence 3iiiA).

Genital/Urinary Tumors

Added Di Carlo et al. as reference 4.

Other Rare Childhood Cancers

Added text about a retrospective analysis from the European-American-Pheochromocytoma-Paraganglioma-Registry that identified 177 patients with paraganglial tumors who were diagnosed before age 18 years. Also added text about germline mutation incidence, prevalence of second tumors, hereditary disease, and life expectancy of these patients (cited Bausch et al. as reference 52 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.

  • Updated: April 6, 2015