Familial neuroblastoma: Case reports, literature review, and etiologic considerations Journal Article


Authors: Kushner, B. H.; Gilbert, F.; Helson, L.
Article Title: Familial neuroblastoma: Case reports, literature review, and etiologic considerations
Abstract: The phenomenon of familial neuroblastoma is discussed in the context of case reports describing disseminated neuroblastoma in two of three half‐brothers who share a common unaffected mother and who each have a different father. This family's cytogenetics proved to be unremarkable; also, the mother's peripheral blood DNA did not show tumorigenic capacities in transfection‐nude mice experiments. An analysis of reported cases permits an updated examination of the clinical features of this entity and defines the limits of genetic counseling of families of all neuroblastoma patients. Multiple primaries are a hallmark of familial neuroblastoma. Most diagnoses are made in the first 18 months of life and at ages that fall within 12 months of the age of diagnosis of the other affected family member. Difficulties in determining the incidence and penetrance of an inherited susceptibility to neuroblastoma derive from undiagnosed tumors that have undergone regression or spontaneous maturation to benign ganglioneuroma, as well as from early deaths or long‐term treatment complications that preclude reproduction and multigenerational pedigrees. Nevertheless, the risk of neuroblastoma in siblings or offspring of the large majority of persons with neuroblastoma appears to be less than 6%. Recent observations concerning chromosomal aberrations and oncogenes in embryonal malignancies are presented in an integrated model of tumorigenesis that corresponds to clinical experience. Copyright © 1986 American Cancer Society
Keywords: child; preschool child; bone neoplasms; case report; comparative study; lymphatic metastasis; animal; mice; heredity; cell line; age factors; age; risk; cancer genetics; chromosome aberration; oncogene; dna; mice, nude; infant; neuroblastoma; diagnosis; sibling; genetic risk; lymphocytes; multiple cancer; nervous system; survey; endocrine system; etiology; abdominal neoplasms; autonomic nervous system; disease susceptibility; chromosome banding; family study; human; male; priority journal
Journal Title: Cancer
Volume: 57
Issue: 9
ISSN: 0008-543X
Publisher: Wiley Blackwell  
Date Published: 1986-05-01
Start Page: 1887
End Page: 1893
Language: English
DOI: 10.1002/1097-0142(19860501)57:9<1887::Aid-cncr2820570931>3.0.Co;2-7
PUBMED: 3955526
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 18 August 2021 -- Source: Scopus
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  1. Brian Kushner
    312 Kushner
  2. Lawrence Helson
    26 Helson