Cancer risk in second degree relatives of children with soft tissue sarcoma.

The risk of cancer in the second degree relatives of a population-based series of children with soft tissue sarcoma was studied in relation to (i) various characteristics in these relatives, (ii) certain clinical features in the index children previously identified as risk factors for cancer in their first degree relatives. Overall there was a non-significant deficit of cancers in the second degree relatives (RR = 0.88) and cancer risk was unrelated to type or site of cancer, type of relative, or to risk factors in the index case. The findings indicate that although the families investigated may include a proportion with the Li-Fraumeni cancer family syndrome, the increased cancer risk already reported in the first degree relatives does not extent to second degree relatives in general.