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Unusual Cancers of Childhood Treatment (PDQ®)

  • Last Modified: 12/23/2011

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Changes to This Summary (12/23/2011)

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.

Head and Neck Cancers

Added text to state that nasopharyngeal carcinoma (NPC) is overrepresented in black children in the United States when compared with other malignancies (cited Cheuk et al. as reference 6).

Revised text to state that the overall survival of children and adolescents with NPC has improved over the last four decades and about 80% are cured; although the outcome has improved with the use of chemotherapy and radiation therapy, significant morbidities do occur.

Added text to state that investigators at St. Jude Children's Research Hospital performed a retrospective review of 59 young patients with NPC; they reported better outcome for patients whose treatment included cisplatin and for patients who received more than 50 Gy of radiation to the primary tumor and to the regional lymph nodes; these treatments were associated with a high probability of hearing loss, hypothyroidism, trismus, xerostomia, dental problems, and chronic sinusitis or otitis.

Added text to state that routine neck dissection and nodal exploration are not indicated in the absence of clinical or radiological evidence of disease; management of cervical lymph node metastases has been addressed in a review article (cited Zanation et al. as reference 30).

Added text to state that thyroid nodules in children are five times more likely to be malignant than those in adults (cited Waguespack et al. as reference 42).

Added text to state that the management of differentiated thyroid cancer in children has been reviewed in detail.

Added Raval et al. as reference 62.

Revised text to state that for patients with a small (<1 cm) unifocal nodule, treatment may involve only a lobectomy.

Added Morris et al. as reference 86.

Added text to state that review of the Surveillance Epidemiology and End Results database identified 54 patients younger than 20 years with oral cavity squamous cell carcinoma (OCSCC) between 1973 and 2006. Pediatric patients with OCSCC had better survival than adult patients; however, when differences in patient, tumor, and treatment-related characteristics are adjusted for, the two groups experienced equivalent survival (added level of evidence 3iA).

Revised text to state that the most common malignant lesion is mucoepidermoid carcinoma; less common malignancies include acinic cell carcinoma, rhabdomyosarcoma, adenocarcinoma, adenoid cystic carcinoma, and undifferentiated carcinoma (cited Kupferman et al. as reference 109).

Revised text to state that the tumors (midline carcinomas) arise in midline epithelial structures including the thymus, mediastinum, airway structures, pancreas, liver, and bladder (cited Shehata et al. as reference 127).

Thoracic Cancers

Added text to state that cerebral metastasis may occur in 11% of patients with Type II pleuropulmonary blastoma (PPB).

Revised text to state that the tumor is usually located in the lung periphery, but it may be extrapulmonary with involvement of the heart/great vessels, mediastinum, diaphragm, and/or pleura.

Added text to state that the International Pleuropulmonary Blastoma Registry identified 11 cases of Type II and Type III PPB with tumor extension into the thoracic great vessels or the heart; radiographic evaluation of the central circulation should be performed in children with suspected or diagnosed PPB to identify potentially fatal embolic complications (cited Priest et al. as reference 46). Also added that the cerebral parenchyma is the most common metastatic site.

Added Bruce as reference 70.

Revised text to state that primary malignant pediatric heart tumors are rare but may include malignant teratomas, rhabdomyosarcomas, chondrosarcomas, infantile fibrosarcoma, and other sarcomas (cited Kogon et al. as reference 78).

Abdominal Cancers

Added text to state that other constitutional syndromes with predisposition to develop adrenocortical tumors include MEN1, familial adenomatous polyposis, and Carney complex (cited Ribeiro et al. as reference 10).

Added Hatzipantelis et al. as reference 79.

Genital/Urinary Tumors

Added Paner et al. and Lerena et al. as references 2 and 6, respectively.

Added Cecchetto et al. as reference 17 and level of evidence 3iiiDii.

Other Rare Childhood Cancers

Added National Comprehensive Cancer Network as reference 17.