Pleuropulmonary blastoma: A marker for familial disease

J. R. Priest, J. Watterson, L. Strong, V. Huff, W. G. Woods, R. L. Byrd, S. H. Friend, I. Newsham, M. D. Amylon, A. Pappo, D. H. Mahoney, C. Langston, R. Heyn, G. Kohut, D. R. Freyer, B. Bostrom, M. S. Richardson, Julio Barredo, L. P. Dehner

Research output: Contribution to journalArticle

164 Citations (Scopus)

Abstract

Objective: To catalog and evaluate patterns at disease in families of children with pleuropulmonary blastoma (PPB). Methods: Data have been collected since 1988 on 45 children with PPB and their families. All pathologic materials were centrally reviewed. Preliminary molecular genetic analyses were performed when possible. Results: In 12 of 45 patients, an association was found between PPB and other dysplasias, neoplasias, or malignancies in the patients with or in their young relatives. The diseases found to be associated with PPB include other cases of PPB, pulmonary cysts, cystic nephromas, sarcomas, medulloblastomas, thyroid dysplasias and neoplasias, malignant germ cell tumors, Hodgkin disease, leukemia, and Langerhans cell histiocytosis. Abnormalities of the p53 tumor suppressor gene, Wilms tumor suppressor gene (WT1), and the putative second genetic locus for Wilms tumor (WT2) were not found in preliminary investigations. Conclusions: The occurrence of PPB appears to herald a constitutional and heritable predisposition to dysplastic or neoplastic disease in approximately 25% of cases. All patients with PPB and their families should be investigated carefully. Further research of this new family cancer syndrome may provide insight into the genetic basis of these diseases.

Original languageEnglish
Pages (from-to)220-224
Number of pages5
JournalJournal of Pediatrics
Volume128
Issue number2
DOIs
StatePublished - Feb 28 1996
Externally publishedYes

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Tumor Suppressor Genes
Neoplasms
Wilms' Tumor Genes
Langerhans Cell Histiocytosis
Inborn Genetic Diseases
Medulloblastoma
Genetic Loci
Wilms Tumor
Germ Cell and Embryonal Neoplasms
Pleuropulmonary blastoma
Hodgkin Disease
Sarcoma
Cysts
Molecular Biology
Thyroid Gland
Leukemia
Lung

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Priest, J. R., Watterson, J., Strong, L., Huff, V., Woods, W. G., Byrd, R. L., ... Dehner, L. P. (1996). Pleuropulmonary blastoma: A marker for familial disease. Journal of Pediatrics, 128(2), 220-224. https://doi.org/10.1016/S0022-3476(96)70393-1

Pleuropulmonary blastoma : A marker for familial disease. / Priest, J. R.; Watterson, J.; Strong, L.; Huff, V.; Woods, W. G.; Byrd, R. L.; Friend, S. H.; Newsham, I.; Amylon, M. D.; Pappo, A.; Mahoney, D. H.; Langston, C.; Heyn, R.; Kohut, G.; Freyer, D. R.; Bostrom, B.; Richardson, M. S.; Barredo, Julio; Dehner, L. P.

In: Journal of Pediatrics, Vol. 128, No. 2, 28.02.1996, p. 220-224.

Research output: Contribution to journalArticle

Priest, JR, Watterson, J, Strong, L, Huff, V, Woods, WG, Byrd, RL, Friend, SH, Newsham, I, Amylon, MD, Pappo, A, Mahoney, DH, Langston, C, Heyn, R, Kohut, G, Freyer, DR, Bostrom, B, Richardson, MS, Barredo, J & Dehner, LP 1996, 'Pleuropulmonary blastoma: A marker for familial disease', Journal of Pediatrics, vol. 128, no. 2, pp. 220-224. https://doi.org/10.1016/S0022-3476(96)70393-1
Priest JR, Watterson J, Strong L, Huff V, Woods WG, Byrd RL et al. Pleuropulmonary blastoma: A marker for familial disease. Journal of Pediatrics. 1996 Feb 28;128(2):220-224. https://doi.org/10.1016/S0022-3476(96)70393-1
Priest, J. R. ; Watterson, J. ; Strong, L. ; Huff, V. ; Woods, W. G. ; Byrd, R. L. ; Friend, S. H. ; Newsham, I. ; Amylon, M. D. ; Pappo, A. ; Mahoney, D. H. ; Langston, C. ; Heyn, R. ; Kohut, G. ; Freyer, D. R. ; Bostrom, B. ; Richardson, M. S. ; Barredo, Julio ; Dehner, L. P. / Pleuropulmonary blastoma : A marker for familial disease. In: Journal of Pediatrics. 1996 ; Vol. 128, No. 2. pp. 220-224.
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