Cancer in Fanconi anemia, 1927-2001

Blanche P. Alter

Research output: Contribution to journalArticle

Abstract

BACKGROUND. Fanconi anemia (FA) is an autosomal recessive disease associated with an abnormal response to DNA damage. Although FA is well known for the association of aplastic anemia and characteristic birth defects, leukemia and solid tumors also occur at a high rate in this group of patients. A review of all reported cases is informative with regard to the specific types of cancer, the ages at which they occur, and the cumulative probability of their development. METHODS. Medline and bibliographies of publications were searched for articles containing "Fanconi's anemia" or "aplastic anemia" and all cases of FA from 1927 through 2001 were included in the database. Cancer cases were identified within these reports. Descriptive statistical analyses were performed using Stata7 software. RESULTS. One thousand three hundred cases of FA were identified. Nine percent had leukemia (primarily acute myeloid leukemia), 7% had myelodysplastic syndrome, 5% had solid tumors, and 3% had liver tumors. Patients with cancer were older than the cancer-free patients at the time of diagnosis of FA. The median age for cancer (including leukemia) was 16, compared with 68 in the general population. The most frequent solid tumors were aerodigestive and gynecological carcinomas. In approximately 25% of patients with cancer, the malignancy preceded the diagnosis of FA. CONCLUSIONS. If the competing risks of aplastic anemia and leukemia could be removed, the estimated cumulative probability of development of a solid tumor in FA patients is 76% by the age of 45 years. Carcinogenic pathways and cancer prevention, surveillance, and treatment can be studied to advantage in this genetic model of human cancer.

Original languageEnglish (US)
Pages (from-to)425-440
Number of pages16
JournalCancer
Volume97
Issue number2
DOIs
StatePublished - Jan 15 2003
Externally publishedYes

Fingerprint

Fanconi Anemia
Neoplasms
Aplastic Anemia
Leukemia
Genetic Models
Myelodysplastic Syndromes
Bibliography
Acute Myeloid Leukemia

Keywords

  • Aplastic anemia
  • Bone marrow transplant
  • Cancer
  • Fanconi anemia
  • Leukemia
  • Liver tumor
  • Malignancy
  • Myelodysplastic syndrome

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Cancer in Fanconi anemia, 1927-2001. / Alter, Blanche P.

In: Cancer, Vol. 97, No. 2, 15.01.2003, p. 425-440.

Research output: Contribution to journalArticle

Alter, BP 2003, 'Cancer in Fanconi anemia, 1927-2001', Cancer, vol. 97, no. 2, pp. 425-440. https://doi.org/10.1002/cncr.11046
Alter, Blanche P. / Cancer in Fanconi anemia, 1927-2001. In: Cancer. 2003 ; Vol. 97, No. 2. pp. 425-440.
@article{f456d95f050b4c7289e2a8aeb12f3bfb,
title = "Cancer in Fanconi anemia, 1927-2001",
abstract = "BACKGROUND. Fanconi anemia (FA) is an autosomal recessive disease associated with an abnormal response to DNA damage. Although FA is well known for the association of aplastic anemia and characteristic birth defects, leukemia and solid tumors also occur at a high rate in this group of patients. A review of all reported cases is informative with regard to the specific types of cancer, the ages at which they occur, and the cumulative probability of their development. METHODS. Medline and bibliographies of publications were searched for articles containing {"}Fanconi's anemia{"} or {"}aplastic anemia{"} and all cases of FA from 1927 through 2001 were included in the database. Cancer cases were identified within these reports. Descriptive statistical analyses were performed using Stata7 software. RESULTS. One thousand three hundred cases of FA were identified. Nine percent had leukemia (primarily acute myeloid leukemia), 7{\%} had myelodysplastic syndrome, 5{\%} had solid tumors, and 3{\%} had liver tumors. Patients with cancer were older than the cancer-free patients at the time of diagnosis of FA. The median age for cancer (including leukemia) was 16, compared with 68 in the general population. The most frequent solid tumors were aerodigestive and gynecological carcinomas. In approximately 25{\%} of patients with cancer, the malignancy preceded the diagnosis of FA. CONCLUSIONS. If the competing risks of aplastic anemia and leukemia could be removed, the estimated cumulative probability of development of a solid tumor in FA patients is 76{\%} by the age of 45 years. Carcinogenic pathways and cancer prevention, surveillance, and treatment can be studied to advantage in this genetic model of human cancer.",
keywords = "Aplastic anemia, Bone marrow transplant, Cancer, Fanconi anemia, Leukemia, Liver tumor, Malignancy, Myelodysplastic syndrome",
author = "Alter, {Blanche P.}",
year = "2003",
month = "1",
day = "15",
doi = "10.1002/cncr.11046",
language = "English (US)",
volume = "97",
pages = "425--440",
journal = "Cancer",
issn = "0008-543X",
publisher = "John Wiley and Sons Inc.",
number = "2",

}

TY - JOUR

T1 - Cancer in Fanconi anemia, 1927-2001

AU - Alter, Blanche P.

PY - 2003/1/15

Y1 - 2003/1/15

N2 - BACKGROUND. Fanconi anemia (FA) is an autosomal recessive disease associated with an abnormal response to DNA damage. Although FA is well known for the association of aplastic anemia and characteristic birth defects, leukemia and solid tumors also occur at a high rate in this group of patients. A review of all reported cases is informative with regard to the specific types of cancer, the ages at which they occur, and the cumulative probability of their development. METHODS. Medline and bibliographies of publications were searched for articles containing "Fanconi's anemia" or "aplastic anemia" and all cases of FA from 1927 through 2001 were included in the database. Cancer cases were identified within these reports. Descriptive statistical analyses were performed using Stata7 software. RESULTS. One thousand three hundred cases of FA were identified. Nine percent had leukemia (primarily acute myeloid leukemia), 7% had myelodysplastic syndrome, 5% had solid tumors, and 3% had liver tumors. Patients with cancer were older than the cancer-free patients at the time of diagnosis of FA. The median age for cancer (including leukemia) was 16, compared with 68 in the general population. The most frequent solid tumors were aerodigestive and gynecological carcinomas. In approximately 25% of patients with cancer, the malignancy preceded the diagnosis of FA. CONCLUSIONS. If the competing risks of aplastic anemia and leukemia could be removed, the estimated cumulative probability of development of a solid tumor in FA patients is 76% by the age of 45 years. Carcinogenic pathways and cancer prevention, surveillance, and treatment can be studied to advantage in this genetic model of human cancer.

AB - BACKGROUND. Fanconi anemia (FA) is an autosomal recessive disease associated with an abnormal response to DNA damage. Although FA is well known for the association of aplastic anemia and characteristic birth defects, leukemia and solid tumors also occur at a high rate in this group of patients. A review of all reported cases is informative with regard to the specific types of cancer, the ages at which they occur, and the cumulative probability of their development. METHODS. Medline and bibliographies of publications were searched for articles containing "Fanconi's anemia" or "aplastic anemia" and all cases of FA from 1927 through 2001 were included in the database. Cancer cases were identified within these reports. Descriptive statistical analyses were performed using Stata7 software. RESULTS. One thousand three hundred cases of FA were identified. Nine percent had leukemia (primarily acute myeloid leukemia), 7% had myelodysplastic syndrome, 5% had solid tumors, and 3% had liver tumors. Patients with cancer were older than the cancer-free patients at the time of diagnosis of FA. The median age for cancer (including leukemia) was 16, compared with 68 in the general population. The most frequent solid tumors were aerodigestive and gynecological carcinomas. In approximately 25% of patients with cancer, the malignancy preceded the diagnosis of FA. CONCLUSIONS. If the competing risks of aplastic anemia and leukemia could be removed, the estimated cumulative probability of development of a solid tumor in FA patients is 76% by the age of 45 years. Carcinogenic pathways and cancer prevention, surveillance, and treatment can be studied to advantage in this genetic model of human cancer.

KW - Aplastic anemia

KW - Bone marrow transplant

KW - Cancer

KW - Fanconi anemia

KW - Leukemia

KW - Liver tumor

KW - Malignancy

KW - Myelodysplastic syndrome

UR - http://www.scopus.com/inward/record.url?scp=0037439356&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0037439356&partnerID=8YFLogxK

U2 - 10.1002/cncr.11046

DO - 10.1002/cncr.11046

M3 - Article

C2 - 12518367

AN - SCOPUS:0037439356

VL - 97

SP - 425

EP - 440

JO - Cancer

JF - Cancer

SN - 0008-543X

IS - 2

ER -