An unusual dysplasia-malformation-cancer syndrome in two patients
- 1 January 1978
- journal article
- case report
- Published by Wiley in American Journal of Medical Genetics
- Vol. 1 (3) , 279-289
- https://doi.org/10.1002/ajmg.1320010304
Abstract
We report two patients with a similar syndrome of gross malformation of a lower limb and contiguous structures due to involvement with dysplastic, teratomatous tissue. This dysplasia seems to have arisen in a paramedian position in the embryonic hindquarter at the time of lower limb-bud differentiation. Malignant degeneration at 5--7 months led to metastases and death in both cases around 1 year of age. The behavior of the dysplastic/oncoplastic tissue suggests a 2-"mutational" causal model. This is an apparently previously undescribed formal genesis syndrome.Keywords
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