Tumors in children with birth defects. Current data from the Czech Republic

Authors: Šípek 1,2;  J. Mališ 3,4;  J. Štěrba 5,6;  J. Mužík 7;  V. Gregor 1,8;  Z. Štembera 9;  J. Horáček 1,10;  V. Bajčiová 5,6;  T. Kepák 5,6;  A. Šípek jr. 11;  P. Langhammer 12;  L. Petržílková 12;  E. Vanková 12;  J. Wiesnerová 12
Authors‘ workplace: Oddělení lékařské genetiky, Fakultní Thomayerova nemocnice, Praha, ředitel MUDr. K. Filip, CSc., MBA 1;  Sanatorium PRONATAL, odborný vedoucí doc. MUDr. T. Mardešić, CSc. 2;  Klinika dětské hematologie a onkologie, 2. LF UK, Praha, děkan doc. MUDr. O. Hrušák, Ph. D. 3;  Klinika dětské hematologie a onkologie, Fakultní nemocnice Motol, Praha, ředitel JUDr. ing. M. Ludvík, MBA 4;  Klinika dětské onkologie, Fakultní nemocnice, Brno, ředitel MUDr. R. Kraus, MBA 5;  Klinika dětské onkologie, LF MU, Brno, děkan prof. MUDr. J. Žaloudík, CSc. 6;  Institut biostatistiky a analýz, Masarykova univerzita, ředitel doc. RNDr. L. Dušek, Ph. D. 7;  Institut postgraduálního vzdělávání ve zdravotnictví, Praha, Katedra lékařské genetiky, ředitel MUDr. Z. Hadra 8;  Ústav pro péči o matku a dítě, ředitel doc. MUDr. J. Feyereisl, CSc. 9;  Gennet, Praha, vedoucí MUDr. D. Stejskal 10;  1. LF UK, Praha, děkan prof. MUDr. T. Zima, DrSc., MBA 11;  Ústav zdravotnických informací a statistiky České republiky, ředitelka Mgr. V. Mazánková 12
Published in: Ceska Gynekol 2009; 74(2): 105-117
Category: Original Article


Aim of study:
An analysis of birth defects incidences in a co-incidence with children age tumors in the Czech Republic in 1994 – 2005. Some bio-social factors (maternal age, birth weight, gestational week at birth) and their roles were studied as well.

Typ of study:
Retrospective demografic-epidemiological analysis of birth defects and children age tumors incidences in children born in the Czech Republic during 1994 – 2005.

Material and methods:
Data from the National Birth Defects Register and National Oncological Register (both run in the Institute for Health Information and Statistics) in the Czech Republic were used along with some additional data from the Register of newborns and Register of mothers at childbirth. Out of these data, a group of children with both birth defect and tumor was analyzed according to particular diagnoses and to some selected bio-social factors. Out of the total number 1707 children with tumor (934 (54.7%) boys and 773 (45.3%) girls) were 1572 children without birth defect and 135 with both tumor and birth defect. Total number of children with birth defect were 39 197 (39 059 live births and 138 stillbirths), 22 741 (58.1%) boys and 16 435 (41.9%) girls (in 21 cases the sex was not specified). In these children totally 53 539 birth defect diagnoses were registered (30 739 in boys, 22 781 in girls and 19 in children with unspecified sex).

In 1572 children without birth defect and with tumor, a mean age at time of tumor diagnosis was 3.6 years, in 135 children with both tumor and birth defect was 2.2 years, which is significantly lower (p<0.001, Mann-Whitney U test). No statistically significant difference was found in birthweight and birthlenght and gestational week and maternal age at time of delivery. An increased frequency of tumors in the group of children with birth defect was found in groups mesothelial tumors (C45 – C49), tumors of urinary tract (C64 – C68) and tumors of head and neck (C00 – C14, C30 – C31). On the other hand, a decreased tumor frequency in the group of children with birth defect was found in groups of lymfoid and haematopoietic tumors ((C81 – C96) and tumors of eye and brain (C69 – C72). As a risk factor of tumorigenesis in in children with birth defect was a birth defect from groups of defects of cardiovascular system, uropoietic system, chromosal aberrations and other unspecified defects. In children with both birth defect and tumor a decreased survival rate (p=0.0437, Log-rank test) was found. A decreased survival rate was also confirmed after tumor diagnosis, although this decrease was not statistically significant (p=0.2021, Log-rank test). There is also a highly statistically significant difference (p<0.001, Log-rank test) in survival between groups with and without a birth defect prior to tumor diagnosis.

A higher risk of tumorigenesis in children with birth defect (compared to children without birth defect) was confirmed. There was also a lower survival in a group of children with tumor and birth defect compared to those with tumor and without birth defects. A higher risk of tumorigenesis in some types of birt defects was also found.

Key words:
birth defect, incidence, tumor, Czech Republic.


1. Agha, MA., Williams, JI., Marrett, L., et al. Congenital abnormalities and childhood cancer. A cohort linkage study. Willey InterScience - Published on line 15. March 2005, p. 11939-1948.

2. Altmann, AE., Halliday, JL, Giles, GG. Association between congenital malformations and childhood cancer. A register based case-control study. Br J Cancer, 1998, 78, p. 1244–1249.

3. Blot, WJ., Stiller, CA., Kinnier-Wilson, LM. Oral clefts and childhood cancer. Lancet, 1980, 1, p. 722.

4. Donnai, D., Winter, RM. Congenital malformations syndromes. Chapman and Hall Medical.1995.

5. Evans, G., Burnell, L., Campbell, R., et al. Congenital anomalies and genetic syndromes in 173 cases of medulloblastoma Med Pediatr Oncol, 1993, 21, p. 433-434.

6. Fong, C., Brodeur, GM. Down’s syndrome and leukemia: epidemiology, genetics, cytogenetics and mechanisms of leukemogenesis. Cancer Genet Cytogenet, 1987, 28, p. 55–76.

7. Foulkes, WD., Buu, PN., Filiatrault, D., et al. Excess of congenital abnormalities in French-Canadian children with neuroblastoma: a case series study from Montreal. Med Pediatr Oncol, 1997, 29, p. 272–279.

8. Friedman, JM. Genetics and Epidemiology, Congenital Anomalies and Cancor. Am J Hum Genet, 1997, 60, p. 469-473.

9. Kobayashi, N., Furukawa, T., Takatsu, T. Congenital anomalies in children with malignancy. Paediatr Univ Tokyo, 1968, 16, p. 1–7.

10. Leck, I. Congenital malformations and childhood neoplasm. J Med Genet, 1997, 14, p. 321-326.

11. Mann, JR., Dodd, HE., Draper, GJ., et al. Congenital abnormalities in children with cancer and their relatives: results from a case-control study (IRESCC). Br J Cancer, 1993, p. 357-363.

12. Mehes, K., Signer, E., Pluss, HJ., et al. Increased prevalence of minor anomalies in childhood malignancy. Eur J Pediatr, 1985, 144, p. 243-249.

13. Mertens, AC., Wen, W., Davies, SM., et al. Congenital abnormalities in children with acute leukemia: a report from the Children’s Cancer Group. J Pediatr, 1998, 133, p. 617–623.

14. Mili, F., Khoury, MJ., Flanders, DW., et al. Risk of childhood cancer for infants with birth defects: a record linkage study. Am J Epidemiol, 1993, 137, p. 629–638.

15. Narod, SA., Hawkins, MM., Robertson, CM., et al. Congenital anomalies and childhood cancer in Great Britain. Am J Hum Genet, 1997, 60, p. 474–485.

16. Narod, SA., Stiller, C., Lenoir, GM. An estimate of the heritable fraction of childhood cancer. Br J Cancer, 1991, 63, p. 993–999.

17. Robison, LL., Swanson, T., Day, D., et al. Renal anomalies in childhood acute lymphoblastic leukemia. N Engl J Med, 1982, 307, p. 1086-1087.

18. Shashidhar Pasi, G., Lewandowski, Jr. RC., Borgaonkar, DS. Handbook of chromosomal syndromes. Willey-Liss, 2003.

19. Stiller, CA., Allen, MB., Eatock, EM. Childhood cancer in Britain: the National Registry of Childhood Tumour and incidence rates, 1978-1987. Eur J Cancer, 1995, Suppl 31A, p. 2028-2034.

20. Stiller, CA., Lennox, EL., Kinnier-Wilson, LM. Incidence of cardiac septal defects in children with Wilms’ tumour and other malignant disease. Carcinogenesis, 1987, 8, p. 129-132.

21. Windham, GC., Bjerkedal, T., Langmark, F. A population-based study of cancer incidence in twins and in children with congenital malformation or low birth weight, Norway, 1967-1980. Am J Epidemiol, 1985, 121, p. 49–56.

22. Windham, GC., Bjerkedal, T., Langmark, F. A populationbased study of cancer incidence in twins and in children with congenital malformations or low birth weight, Norway, 1967-1980. Am J Epidemiol, 1985, 121, p. 49-56.

Paediatric gynaecology Gynaecology and obstetrics Reproduction medicine

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