# GENETIC EPIDEMIOLOGY OF CHILDHOOD SARCOMA

> **NIH NIH R01** · UNIVERSITY OF TX MD ANDERSON CAN CTR · 1990 · $117,629

## Abstract

Through study of a cohort of 3 year survivors of childhood
sarcoma, we have confirmed a rare familial cancer syndrome
involving childhood sarcoma, breast cancer, and an array of other
tumors in 6-7% of cases and demonstrated that it is most likely
attributable to a rare autosomal dominant gene with high
penetrance.  We have demonstrated that, among survivors of
childhood soft tissue sarcoma, gene carriers have a significantly
increased risk of a second malignant neoplasm as compared with
non gene carriers, and that the risk is increased by radiation in
both groups.  We now propose to extend the genetic epidemiologic
family studies to include a cohort of 400 childhood bone sarcoma
patients, (a) to determine the extent to which childhood bone
sarcomas are involved in different familial cancer syndromes, (b)
to define parameters for models that characterize these
syndromes and which can be used for genetic counseling/linkage
investigation, (c) to determine whether certain clinical-
epidemiologic features discriminate between hereditary and non-
hereditary cases and (d) to identify specific kindreds for
collaborative investigation to localize gene(s) predisposing to
cancer and to test for genetic heterogenity.  The primary
statistical tool that will be used to address these questions is
segregation analysis under a generalized mixed model.

A second primary objective will be to determine the risk of
developing second tumors to the 400 childhood bone sarcoma
patients.  The study will consider the impact of heredity and
cancer treatment (radiotherapy and chemotherapy) in explaining
variation in risk to second tumors.

## Key facts

- **NIH application ID:** 3177408
- **Project number:** 5R01CA038929-06
- **Recipient organization:** UNIVERSITY OF TX MD ANDERSON CAN CTR
- **SAM.gov UEI:** S3GMKS8ELA16
- **Principal Investigator:** LOUISE C STRONG
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 1990
- **Award amount:** $117,629
- **Award type:** 5
- **Project period:** 1984-12-01 → 1992-11-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/3177408

## Citation

> US National Institutes of Health, RePORTER application 3177408, GENETIC EPIDEMIOLOGY OF CHILDHOOD SARCOMA (5R01CA038929-06). Retrieved via MORVS 2026-10-25 from https://api.morvs.ai/grant/nih/3177408. Licensed CC0.

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