Unusual Cancers of Childhood Treatment (PDQ®)–Health Professional Version

Changes to This Summary (01/31/2019)

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

The Histology and Molecular Features subsection of the Esthesioneuroblastoma section was extensively revised.

Added Spinelli et al. as reference 89.

Added text to state that in one review, it was estimated that 12% of mammary analog secretory carcinoma (MASC) cases occurred in the pediatric population (cited Khalele as reference 145).

Added text to state that MASC is characterized by an ETV6-NTRK3 fusion (cited Skálová et al. as reference 148).

Added text to state that objective responses have been observed in all reported patients with recurrent NTRK fusion–positive MASCs who were treated with entrectinib or larotrectinib (cited 2017 Drilon et al. and 2018 Drilon et al. as references 155 and 156, respectively).

Revised text to state that reports with small numbers of patients have documented that in selected cases, the administration of a quadrivalent human papillomavirus (HPV) vaccine can be associated with a complete remission and an increase in the intersurgical interval (cited Mészner et al. as reference 176). Also added text to state that in contrast, other reports have not documented a therapeutic effect of the quadrivalent HPV vaccine (cited Katsuta et al. as reference 177).

Abdominal Cancers

Added text about the results of a retrospective review of the Italian Pediatric Rare Tumor Registry that identified 43 pediatric patients diagnosed with solid pseudopapillary tumor of the pancreas between 2000 and 2018 (cited Crocoli et al. as reference 61 and level of evidence 3iiA).

Added text about the two well-described forms of hereditary colorectal cancer.

Added text to state that other colorectal cancer syndromes and their associated genes include oligopolyposis (POLE, POLD1), familial adenomatous polyposis 3 (NTHL1), juvenile polyposis syndrome (BMPR1A, SMAD4), Cowden syndrome (PTEN), and Peutz-Jeghers syndrome (STK11).

Added Ambe et al. as reference 139.

Other Rare Childhood Cancers

Added text to state that treatment options for childhood paraganglioma and pheochromocytoma include surgery, chemotherapy, high-dose iodine I 131-labeled metaiodobenzylguanidine, and tyrosine kinase inhibitor therapy (sunitinib).

Added text about the results of a prospective study that monitored 60 families who had more than three members with melanoma (cited Goldstein et al. as reference 100).

Added text to state that treatment options for childhood melanoma include surgery and immune checkpoint inhibitors or BRAF/MEK inhibitors.

The Immune Checkpoint Inhibitors or BRAF/MEK Inhibitors subsection was renamed from Adjuvant Therapy.

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.

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