Inguinal hernia in patients with Ewing sarcoma: a clue to etiology.
ABSTRACT Various congenital anomalies have been associated with childhood cancer, but as yet no anomaly has been consistently found with Ewing sarcoma (ES). Recently a large case-control study of ES patients reported a greater number of hernias in both cases and their sibling controls than in population controls. Most of these hernias were inguinal. Because these anomalies were also reported previously in two case series, we looked for inguinal hernias in a different population of ES patients.
We abstracted medical records for 306 pathologically confirmed ES/primitive neuroectodermal tumor (PNET) patients seen at NIH between 1960 and 1992. Epidemiological data on demographics and medical conditions were analyzed. The frequency of anomalies was compared to expected rates to calculate relative risk and confidence intervals.
Anomalies were present in 67 (22%) cases. A particular anomaly, inguinal hernia, was reported for 13 (5%) NIH cases. Compared to population estimates for white children, the relative risk of inguinal hernia among white NIH cases was 13.3 (95% CI 3.60-34.1) for females and 6.67 (95% CI 2.67-13.7) for males.
The findings of inguinal hernias in some patients with ES suggest that a disruption in normal embryological development occurred. This may provide an important clue to the etiology of ES. We hypothesize that these hernias may relate to an in utero exposure or indicate an underlying genetic disorder. Future studies should carefully evaluate ES families for genetic disease and explore environmental factors. Med. Pediatr. Oncol. 34:195-199, 2000. Published 2000 Wiley-Liss, Inc.
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ABSTRACT: A case-control study of Ewing's sarcoma (ES) was conducted to search for occupational exposures associated with ES. The study consisted of 196 cases and 196 random-digit controls matched on geographical region, gender, ethnic origin and birth date. A questionnaire was administered to mothers of participants to obtain information on medical conditions, medications, and parental occupations during and after the index pregnancy. An occupational exposure expert coded jobs and industries for possible and probable exposure to selected occupational hazards. Risk of ES was increased with probable parental exposure to wood dusts during their usual occupation post pregnancy (odds ration [OR] = 3.2; 95% confidence interval [CI] = 1.1-9.2). Other exposures, including a priori suspected risk factors such as exposure to pesticides and farm animals, were not significantly associated with ES. A history of household pesticide extermination was associated with ES among boys aged 15 or younger (OR = 3.0; 95% CI = 1.1-8.1), but not among girls or older boys. Our results suggest that earlier reports of associations of ES with parental farm employment may have been describing risks associated with organic dusts encountered when working on a farm, rather than agricultural exposures or other farming related exposures.International Journal of Cancer 05/2005; 114(3):472-8. · 6.20 Impact Factor
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ABSTRACT: Ewing's sarcoma family of tumours has been associated with a history of hernia and with a parental occupation of farming. However, the causes of these tumours remain unknown. We therefore aimed to investigate the association between hernia and Ewing's sarcoma family of tumours. We did a pooled analysis of two case-control studies and a meta-analysis of three case-control studies of Ewing's sarcoma family of tumours that had adequate information on history of hernia. The primary endpoint was development of a tumour from the Ewing's sarcoma family. 138 patients with such a tumour and 574 controls were included in the pooled analysis, and 357 patients with these tumours and 745 controls were included in the meta-analysis. Risk was assessed by an odds ratio (OR) and 95% CI by use of multivariate analysis with unconditional logistic regression for the pooled analysis and random effects model for the meta-analysis. Pooled analysis showed that children with Ewing's sarcoma family of tumours were more likely to have had an umbilical hernia than were controls (odds ratio [OR] 3.3 [95% CI 1.3-8.0]). Meta-analysis showed that children with Ewing's sarcoma family of tumours were more likely to have had a hernia (3.2 [1.9-5.7]). Ewing's sarcoma family of tumours and hernias (particularly inguinal hernias) have common embryological pathways of neuroectodermal origin, and environmental factors, such as farming, might link the two entities.The Lancet Oncology 08/2005; 6(7):485-90. · 25.12 Impact Factor
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ABSTRACT: Limited population-based epidemiologic information is available on Ewing's sarcoma family of tumours (ESFT), a rare group of neoplasms. Several associations have been noted on a few studies but results were not consistent, except for exposure to farming among cases and their parents. Here we present the non-farm findings of a nationwide case-control study of ESFT in children and young adults in Australia. The analysis included 106 persons with confirmed ESFT and 344 population-based controls selected randomly via telephone. Information was collected by interview (84% face to face). We found a strong and significant association of ESFT with hernias, in particular hernia repaired in hospital (OR = 5.6, 95% Cl 1.3-6.4). Among other factors, there was a near doubling of risk for males, and male cases had their pubertal signs earlier (started shaving earlier) than male controls. There was also an increased risk of ESFT at higher levels of self-assessed exercise, but no other factor really stood out. For pregnancy-related factors, there was a tripling of risk for glandular fever, a doubling of risk for urinary tract infection and a near doubling of risk for X-rays during or just before pregnancy, but these estimates were not significant. In addition, there was a large number of inverse associations with medical conditions (specifically bone disorders), case exposure to medications, vaccinations and X-rays, with ultrasound during the pregnancy having the most certain effects. We conclude that, although the aetiology of ESFT remains obscure, overall there is strong evidence of an association with inguinal hernia; this can now be added to the farm-associated risk reported by others and us. The other associations reported here await replication and refinement in future studies. (C) 2003 Wiley-Liss, Inc.International Journal of Cancer 08/2003; · 6.20 Impact Factor