Trichoepithelioma papulosum multiplex.

Trichoepithelioma papulosum multiplex is an uncommon autosomal dominant disorder in which multiple trichoepitheliomas are seen. Its cause may be a defective tumor suppressor gene. Studies have mapped this gene to the 9p21 locus. However, there is a parallel or identical syndrome of multiple trichoepitheliomas and cylindromas. Within a given family, some members may have cyclindromas whereas others may have trichoepitheliomas or a combination of both. Although preliminary evidence suggests a different gene is responsible, it is possible that TPM may be caused by more than two independent genes, with some cases of TPM due to impairment of the gene for cylindromatosis. This entity, as well as other disorders with multiple appendageal tumors, may require clarification and distinction from TPM.

[A century of progress in the knowledge and treatment of nephroblastoma]. / Un siècle de progrès dans la connaissance et le traitement du néphroblastome.

Since its initial description by Max Wilms over a century ago, nephroblastoma has benefited from considerable improvements both in terms of basic knowledge about it and management of it. Today, the majority of these very young patients can expect a long-term survival in excess of 90% at the price of a light therapy that combines surgical resection, chemotherapy based on ill-toxic agents, and in selected cases, radiotherapy of remarkably low toxicity. The contribution of large international studies will be emphasized here.

Multiple primary cancers in Connecticut, 1935-82.

Recently, the National Cancer Institute published a comprehensive monograph on multiple primary cancers in Connecticut and Denmark. This paper summarizes some of the observations made on the Connecticut population. Data compiled by the Connecticut Tumor Registry have extended our knowledge about the patterns of multiple primary cancers, especially among long-term survivors of cancer and among patients with relatively rare tumors about which little information currently exists. When compared with the general Connecticut population, cancer patients had a 31 percent (RR = 1.31) increased risk of developing a second cancer and a 23 percent (RR = 1.23) elevated risk of second cancer at a different site from the first. Common environmental exposures seemed responsible for the excess occurrence of many second cancers, particularly those related to cigarette smoking, alcohol consumption, or both. For example, persons with epithelial cancers of the lung, larynx, esophagus, buccal cavity, and pharynx were particularly prone to develop new cancers in the same or contiguous tissue throughout their lifetimes. Cancers of the colon, uterine corpus, breast, and ovary frequently occurred together, suggesting underlying hormonal or dietary influences. Only patients with prostate cancer were at significantly low risk for second cancer development; this might be an artifact of case finding, since advanced age at initial diagnosis was generally associated with an underascertainment of second cancers. Radiotherapy may have caused rectal and other cancer among patients with cancers of the female genital tract, and leukemia among patients with uterine corpus cancer. Chemotherapy with alkylating agents probably contributed to the excess of acute nonlymphocytic leukemia following multiple myeloma or cancers of the breast and ovary. Genetic susceptibility seemed to explain some tumor complexes, such as the multiple occurrences of cutaneous melanoma and the excess of bone cancer following retinoblastoma. Research into multiple cancer syndromes should enhance our understanding of carcinogenic factors and mechanisms and the development of strategies for cancer prevention and control.

Multiple primary malignant neoplasms. The Connecticut experience, 1935-1964.

Results for non-simultaneously diagnosed malignant tumors from Connecticut indicate that individuals with one malignant neoplasm have 1.29 times the risk of developing a new independent primary tumor when compared to individuals who never had cancer (P less than 0.01). However, the increased risk of multiple primary tumors is highly selective on a site-specific basis. Table 135 presents Connecticut Registry data indicating the risk of a subsequent primary malignancy by anatomic site of the later primary in patients with a first primary cancer. Tables 136 and 137 present tabulations for anatomic sites with statistically significant excesses and deficiencies, with an analysis by time interval between the two malignancies. Finally, Table 138 presents figures showing histologic confirmation for site-group paris with significant excesses of observed-over-expected later primary malignant neoplasms. The reader should bear in mind that just as the risk of subsequent primaries varies with the anatomic site of the subsequent primary (Table 135), the risk is also highly dependent upon the anatomic site of the first primary cancer (Chapters 6-12).