Article
1 October 1969

Soft-Tissue Sarcomas, Breast Cancer, and Other Neoplasms: A Familial Syndrome?

Publication: Annals of Internal Medicine
Volume 71, Number 4

Abstract

Four families were identified in which a pair of children had soft-tissue sarcomas: three sets of sibs and one set of cousins. One parent of each affected child developed cancer; carcinoma of the breast occurred in three mothers under 30 years of age. Other young adults in these families had a high frequency of cancer, with no evidence of underlying genetic disorders known to carry a high risk of neoplasia. The increased familial susceptibility to cancer was manifested not only by the large number of members affected but by a seeming excess of multiple primary neoplasms. These findings suggest a new "familial" syndrome of neoplastic diseases in which heredity or oncogenic agents, or both, may have a causal role.

Get full access to this article

View all available purchase options and get full access to this article.

References

1.
FRAUMENI JF and THOMAS LB: Malignant bladder tumors in a man and his three sons. JAMA 201: 507, 1967.
2.
HEATH CW and MOLONEY WC: Familial leukemia; five cases of acute leukemia in three generations. New Eng. J. Med. 272: 882, 1965.
3.
FRAUMENI JFVOGEL CL, and EASTON JM: Sarcomas and multiple polyposis in a kindred. A genetic variety of hereditary polyposis? Arch. Intern. Med. (Chicago) 121: 57, 1968.
4.
SCHIMKE RN and HARTMANN WH: Familial amyloid-producing medullary thyroid carcinoma and pheochromocytoma. A distinct genetic entity. Ann. Intern. Med. 63: 1027, 1965.
5.
RIGBY PGPRATT PTROSENLOF RC, and LEMON HM: Genetic relationships in familial leukemia and lymphoma. Arch. Intern. Med. (Chicago) 121: 67, 1968.
6.
LYNCH HTKRUSH AJ, and LARSEN AL: Heredity and multiple primary malignant neoplasms: six cancer families. Amer. J. Med. Sci. 254: 322, 1967.
7.
LI FP and FRAUMENI JF: Rhabdomyosarcoma in children: epidemiologic study and identification of a familial cancer syndrome. In preparation.
8.
MILLER RW: Deaths from childhood cancer in sibs. New Eng. J. Med. 279: 122, 1968.
9.
PAPADRIANOS EHAAGENSEN CD, and COOLEY E: Cancer of the breast as a familial disease. Ann. Surg. 165: 10, 1967.
10.
REMZI D and KENDI S: Rhabdomyosarcoma of the prostate in childhood. Turk. J. Pediat. 8: 143, 1966.
11.
BOTTOMLEY RH and CONDIT PT: Cancer families. Cancer Bull. 20: 22, 1968.
12.
HOWARD GM and CASTEN VG: Rhabdomyosarcoma of the orbit in brothers. Arch. Ophthal. (Chicago) 70: 319, 1963.
13.
REED WBNICKEL WR, and CAMPION G: Internal manifestations of tuberous sclerosis. Arch. Derm. (Chicago) 87: 715, 1963.
14.
HEARD G: Malignant disease in von Recklinghausen's neurofibromatosis. Proc. Roy. Soc. Med. 56: 502, 1963.
15.
SCHWEISGUTH OGERARD-MARCHANT R, and LEMERLE J: Naevomatose baso-cellulaire association à un rhabdomyosarcome congenital. Arch. Franc. Pediat. 25: 1083, 1968.
16.
EPSTEIN CJMARTIN GMSCHULTZ AL, and MOTULSKY AG: Werner's syndrome. A review of its symptomatology, natural history, pathologic features, genetics and relationship to the natural aging process. Medicine (Balt.) 45: 177, 1966.
17.
DE HAAS EB and HOUTSMULLER AJ: Fuchs' heterochromia in a patient with Recklinghausen's disease. Ophthalmologic (Basel) 144: 409, 1962.
18.
SLOANE JA and HUBBELL MM: Soft-tissue sarcomas in children associated with congenital anomalies. Cancer 23: 175, 1969.
19.
FRAUMENI JF and LI FP: Hodgkin's disease in childhood: an epidemiologic study. J. Nat. Cancer Inst. 42: 681, 1969.
20.
FRAUMENI JFMILLER RW, and HILL JA: Primary carcinoma of the liver in childhood: an epidemiologic study. J. Nat. Cancer Inst. 40: 1087, 1968.
21.
MILLER RWFRAUMENI JF, and MANNING MD: Association of Wilms's tumor with aniridia, hemihypertrophy and other congenital malformations. New Eng. J. Med. 270: 922, 1964.
22.
FRAUMENI JF and MILLER RW: Adrenocortical neoplasms with hemihypertrophy, brain tumors, and other disorders. J. Pediat. 70: 129, 1967.
23.
MOLONEY JB: A virus-induced rhabdomyosarcoma of mice. Nat. Cancer Inst. Monogr. 22: 139, 1966.
24.
BITTNER JJ: Experimental aspects of mammary cancer in mice, in Breast Cancer and Its Diagnosis and Treatment, edited by LEWISON, E. F., The Williams & Wilkins Co., Baltimore, 1955, p. 75.

Information & Authors

Information

Published In

cover image Annals of Internal Medicine
Annals of Internal Medicine
Volume 71Number 41 October 1969
Pages: 747 - 752

History

Published in issue: 1 October 1969
Published online: 1 December 2008

Keywords

Authors

Affiliations

FREDERICK P. LI, M.D.
JOSEPH F. FRAUMENI JR., M.D., F.A.C.P.
From the Epidemiology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Md.
Requests for reprints should be addressed to Joseph F. Fraumeni, Jr., M.D., 410 Wiscon Bldg., National Institutes of Health, Bethesda, Md. 20014

Metrics & Citations

Metrics

Citations

If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. For an editable text file, please select Medlars format which will download as a .txt file. Simply select your manager software from the list below and click Download.

For more information or tips please see 'Downloading to a citation manager' in the Help menu.

Format





Download article citation data for:
FREDERICK P. LI, JOSEPH F. FRAUMENI. Soft-Tissue Sarcomas, Breast Cancer, and Other Neoplasms: A Familial Syndrome?. Ann Intern Med.1969;71:747-752. doi:10.7326/0003-4819-71-4-747

View More

Get Access

Login Options:
Purchase

You will be redirected to acponline.org to sign-in to Annals to complete your purchase.

Create your Free Account

You will be redirected to acponline.org to create an account that will provide access to Annals.

View options

PDF/ePub

View PDF/ePub

Related in ACP Journals

Media

Figures

Other

Tables

Share

Share

Copy the content Link

Share on social media