Melanoma and soft tissue sarcoma in seven patients

Article Abstract:

Soft tissue sarcomas account for less than 1 percent of the cancers in the US, and melanomas about 3 present, although the incidence of melanoma is on the rise. Therefore, it seemed remarkable that over a four-year period seven cases were seen at one institution in which the patients had both soft tissue sarcoma and melanoma. Three of these patients had other tumors as well. Although chance cannot be ruled out, the occurrence of these two uncommon cancers together suggest a possible relationship. For this reason, patient records in the Connecticut Tumor Registry were examined; the frequency of sarcoma occurring after melanoma was roughly seven times the expected rate, and the occurrence of melanoma after sarcoma was roughly twice the expected value. While it must be emphasized that the small number of patients in whom both cancers occurred prevents establishing a relationship with any statistical reliability, the results suggest that some common etiologic factor may be involved. While the nature of the possible relationship between the two cancers is not known, two possibilities seem most reasonable. One is that both cancers may result from exposure to a single environmental carcinogen. The other possibility is that some common genetic factor may predispose patients to both cancers. This possible explanation is appealing because of the appearance of additional tumors in three of the seven patients. In addition, sarcomas occur in several cancer syndromes known to be of genetic origin, including breast cancer-sarcoma syndrome and hereditary retinoblastoma. The authors hope that when clinicians become aware of this possible association new cases will be brought to the attention of researchers for further study. (Consumer Summary produced by Reliance Medical Information, Inc.)

Melanoma

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Cancer in relatives of survivors of childhood sarcoma

Article Abstract:

In 1969, four families were described in which the young relatives of children with sarcomas suffered an excess of breast cancers and other tumors. This syndrome, which is now called with Li-Fraumeni syndrome, has now been detected in more than 50 families, and it appears that the tendency to cancer development is inherited as an autosomal dominant trait. There has been some suggestion that the mothers and young relatives of children with sarcomas should be considered to be at increased risk for developing breast cancer, and that mammographic screening be initiated accordingly. However, not all children with sarcomas are necessarily members of a cancer-prone family, and it is not certain to what degree early screening would accomplish the desired objectives if instituted for all such cases. To ascertain the level of overall risk for the relatives of children with sarcomas, independent of any demonstrated familial cancer tendency, the medical records of 402 relatives of 88 long-term survivors of childhood sarcoma were retrospectively examined. Twenty-six cancers were noted in the relatives; in a group of this size and age a distribution 24 cancers would be expected. Likewise, four cases of breast cancer were observed in the mothers of the patients; in a comparable general population three would be expected. The results indicate that although there may be a significantly increased risk of cancer for a small subset of subjects with an inherited cancer syndrome, the overall rate of cancer among the relatives of children with sarcomas may not be much different from normal. The authors suggest that more research is necessary before special screening measures are justified for the relatives of such children. (Consumer Summary produced by Reliance Medical Information, Inc.)

Statistics, Li-Fraumeni syndrome

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Cancer in the families of children with soft tissue sarcoma

Article Abstract:

Previous research has identified families with a higher-than-average risk of cancer. These families were first identified on the basis of soft-tissue sarcomas occurring in the children. Soft tissue sarcomas include entities such as rhabdomyosarcoma and fibrosarcoma. To extend the knowledge of such cancer-prone families, a study was undertaken of 754 first-degree relatives of 177 children with soft-tissue sarcoma. A total of 40 cancers were recorded among the relatives, in contrast to the 25 which might be expected in a population of this size. There was no significant excess among the fathers of the index cases, and only a borderline excess among the mothers. Siblings of the index cases, however, had a roughly five-fold increase in the risk of cancer. Breast cancers were the most common among the 40 cancers identified in the relatives; carcinomas of the trachea and lung, and colon and rectum were quite common in childhood. These results suggest that soft tissue sarcomas in children are likely to have a strong genetic component. Furthermore, the occurrence of more common cancers in the families of children with relatively uncommon soft tissue sarcomas may provide insight into some of the fundamental mechanisms of cancer development. (Consumer Summary produced by Reliance Medical Information, Inc.)

Research, Genetic disorders

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