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sgp_warrior_mom Posted: 15 Jul 2009 5:50 PM

My youngest grandson was diagnosed with neuroblastoma. He has an older brother who seems to be healthy. Should we have the older brother tested to make sure he's not likely to develop neuroblastoma too? Thank you for your help.

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Mark Dungan replied on 16 Jul 2009 2:55 PM

I think everyone of us that has had a child or grandchild with neuroblastoma has wondered about this risk.  Some of us ad nauseam.  The good news is that familial neuroblastoma is extremely rare (1-2% of cases)  Many parents, in order to sleep at night, have had their other children test with an HVA/VMA urine test just in case.  If you are losing sleep it may be worthwhile to discuss with your physician. There have been several journal articles on the subject and Dr. John Maris (from CHOP) is one of the experts in this area. 

The full text article can be found here if you are interested:

http://cancerres.aacrjournals.org/cgi/content/full/62/22/6651

Here is an excerpt from this recent article:

"Hereditary neuroblastoma is rare, and a family history of the disease is present in only 1–2% of cases (2 , 12, 13, 14) . Similar to retinoblastoma, patients with familial neuroblastoma are characterized by an earlier median age at diagnosis and a higher frequency of multifocal primary tumors (2 , 12 , 15) . However, patients with hereditary neuroblastoma show the same clinical heterogeneity seen in sporadic cases, including cases as disparate as spontaneous regression and relentless progression within individual families (12 , 15) . Thus, the rarity of familial neuroblastoma may be explained, at least in part, both by the incomplete penetrance and by the lethality of the phenotype. ^"

1. Knudson A. G., Jr., Strong L. C. Mutation and cancer: neuroblastoma and pheochromocytoma. Am. J. Hum. Genet., 24: 514-532, 1972.[Medline]
2. Kushner B. H., Gilbert F., Helson L. Familial neuroblastoma. Case reports, literature review, and etiologic considerations. Cancer (Phila.), 57: 1887-1893, 1986.[Medline]
3. Maris J. M., Kyemba S. M., Rebbeck T. R., White P. S., Sulman E. P., Jensen S. J., Allen C., Biegel J. A., Brodeur G. M. Molecular genetic analysis of familial neuroblastoma. Eur. J. Cancer, 33: 1923-1928, 1997.
4. Chompret A., de Vathaire F., Brugieres L., Abel A., Raquin M. A., Hartmann O., Feunteun J., Bonaiti-Pellie C. Excess of cancers in relatives of patients with neuroblastoma. Med. Pediatr. Oncol., 31: 211 1998.
5. Maris J. M., Tonini G. P. Genetics of familial neuroblastoma Brodeur G. M. Sawada T. Tsuchida Y. Voute P. A. eds. . Neuroblastoma, 125-136, Elsevier Science B. V. Amsterdam 2000.