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Cited by 618Neurofibromatosis and Schwannoma CasesNeuroblastoma Research and TreatmentsHereditary Neurological Disorders

The DOI matches its Crossref record; 22 of the 22 references it cites were found in a registry or an index.

Long-Term Follow-up of von Recklinghausen Neurofibromatosis

Sørensen, Sven Asger, Mulvihill, John J., Nielsen, Arne·1986·New England Journal of Medicine
DOI 10.1056/nejm198604173141603
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Abstract

To document the natural history of von Recklinghausen neurofibromatosis, we followed up a nationwide cohort of 212 affected patients and families identified in Denmark 42 years ago. We obtained follow-up information on 99 percent. Because all 76 probands were identified through hospitals, they may include a disproportionate number of severe cases of neurofibromatosis. To diminish this effect of selection bias, we distinguished between the probands and their affected relatives. In a comparison with the general population, survival rates were significantly impaired in relatives with neurofibromatosis, worse in probands, and worst in female probands. Malignant neoplasms or benign central nervous system tumors occurred in 45 percent of the probands, giving a relative risk of 4.0 (95 percent confidence limits, 2.8 to 5.6) as compared with expected numbers. Multiple primary neoplasms were found in 15 probands, but only 1 relative. Compared with the general population, male relatives with neurofibromatosis had the same rate of neoplasms, whereas female relatives had a nearly twofold higher rate (relative risk, 1.9; 1.1 to 3.1). Nervous system tumors were disproportionately represented. We conclude that patients with severe neurofibromatosis requiring hospitalization often have a poor prognosis, but incidentally diagnosed relatives may have a considerably better outcome.

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Crossref · 10.1056/nejm198604173141603

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22 of 22 references verified0 citation errors · 0 not checkable

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Citations

Cites

Show all 21 references

Research works printed but not linked: 9

  1. Hope, 1981, Adv Neurol, 29, 33
  2. Borberg, 1951, Acta Psychiatr Neurol, 71, 1
  3. Armitage P. Statistical methods in medical research. Oxford: Blackwell, 1971.
  4. Le Lievre, 1978, J Embryol Exp Morphol, 47, 17
  5. Storm, 1985, Natl Cancer Inst Mongr, 68, 411
  6. Reich, 1976, Am J Dis Child, 130, 888
  7. Manuelidis EE, Solitare GB. Glioblastoma multiforme. In: Minckler J, ed. Pathology of the nervous system. New York: McGraw-Hill, 1971:2049–52.
  8. Boudin, 1970, Presse Med, 78, 1427
  9. Friedman, 1982, JNCI, 69, 1289

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Metadata

Authors
Sørensen, S. A., Mulvihill, J. J., & Nielsen, A.
ORCID
Mulvihill, John J.
Venue
New England Journal of Medicine
Publisher
Massachusetts Medical Society
Published
1986-04-17
Volume
314
Issue
16
Pages
1010-1015
ISSN
  • 0028-4793 (ISSN-L)
  • 1533-4406
DOI
10.1056/nejm198604173141603
OpenAlex
W2330798445
Cited by
617
References
21
Citations by year
2026: 2 · 2025: 5 · 2024: 11 · 2023: 12 · 2022: 13 · 2021: 7 · 2020: 16 · 2019: 16 · 2018: 9 · 2017: 13 · 2016: 17 · 2015: 11 · 2014: 18 · 2013: 18 · 2012: 31
FWCI
17.09
Type
journal article
Language
en
Open access
closed
Locations
  • New England Journal of Medicine (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Clinical Neurology › Neurofibromatosis and Schwannoma Cases
  • Health Sciences › Medicine › Oncology › Neuroblastoma Research and Treatments
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
Keywords
von Recklinghausen neurofibromatosis, neurofibromatosis type 1, natural history (disease), survival, cancer, central nervous system tumors, multiple primary neoplasms, sex differences, familial neurofibromatosis, prognosis, long-term follow-up
MeSH terms
Brain Neoplasms, Denmark, Female, Follow-Up Studies, Glioma, Humans, Male, Neoplasms, Multiple Primary, Neurofibromatosis 1, Prognosis, Risk, Sex Factors, Skin Neoplasms

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