Neuroblastoma in Adolescents and Children Older than 10 Years: Unusual Clinicopathologic and Biological Features.

Authors:
Katherine Chastain
Katherine Chastain
Children's Mercy Hospital
Eugenio Taboada
Eugenio Taboada
The Children's Mercy Hospitals and Clinics
Robert Garola
Robert Garola
The Children's Mercy Hospital
United States
John Herriges
John Herriges
Laboratory of Genetics
Linda D Cooley
Linda D Cooley
Children's Mercy Hospital
Atif A Ahmed
Atif A Ahmed
Children's Mercy Hospital
United States

J Pediatr Hematol Oncol 2019 Apr 9. Epub 2019 Apr 9.

Department of Pathology, Children's Mercy Hospital/University of Missouri Kansas City School of Medicine, Kansas City, MO.

Neuroblastoma (NB) in children older than 10 years is rare. We reviewed our archives for patients with NB aged 10 to 18 years and summarized their clinicopathologic/genetic records. Of 96 patients, 4 patients were identified in this age group. Four tumors were abdominal; 1 patient had 2 tumors at diagnosis, one of which was presacral. Tumor sizes ranged from 3 to 20 cm. All tumors were high risk at clinical stages 3 and 4, with metastasis to bone marrow and other areas. Four tumors were poorly differentiated with unfavorable histology and one patient with bilateral adrenal disease had an intermixed ganglioneuroblastoma on one side. Another tumor exhibited pheochromocytoma-like morphology. MYCN amplification was present in bone marrow metastasis in one case. Complex chromosomal gains and 19p deletions were common. Exome sequencing revealed ALK variants in 2 cases and previously unreported MAGI2, RUNX1, and MLL mutations. All patients received standard chemotherapy and 2 patients received ALK-targeted trial therapy. Three patients died of disease, ranging 18 to 23 months after diagnosis. One patient has active disease and is receiving trial therapy. In conclusion, NB in children older than 10 years may exhibit unusual clinicopathologic and genetic features with large tumors, bilateral adrenal disease, rare morphologic features, complex DNA microarray findings and novel mutations. Patients often have grim prognoses despite genomic profiling-guided targeted therapy.

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Source
http://dx.doi.org/10.1097/MPH.0000000000001485DOI Listing
April 2019
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