Abstract
A girl with hemihypertrophy and hamartomas, now 14 years old, had Wilms' tumor and subsequently developed adrenocortical carcinoma. The occurrence of the two tumors with the signs of an inborn defect of growth control supports the hypothesis that both tumors can be caused by the same etiologic factors, which are also teratogenic. An alternative explanation of induction of the second tumor by previous radio- and chemotherapy is discussed. Possible relationships between our case and the syndrome of Wiedemann and Beckwith are pointed out.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Aron, B. S.: Wilms' tumor — A clinical study of eighty-one patients. Cancer 33, 637–646 (1974)
Beckwith, J. B.: Extreme cytomegaly of the adrenal fetal cortex, omphalocele, hyperplasia of kidneys and pancreas, and Leydig-cell hyperplasia — another syndrome? Presented at Annual Meeting of Western Society for Pediatric Research, Los Angeles, California, Nov. 1963
Beckwith, J. B.: Macroglossia, omphalocele, adrenal cytomegaly, gigantism, and hyperplastic visceromegaly. Proceedings of the First Conference on The Clinical Delineation of Birth Defects. Baltimore, Maryland, 1968. Birth Defects: Original Article Series Vol. V, No. 2, 188–196 (1969)
Belpomme, D., Carde, P., Oldham, R. K., Mathe, G., Jacquillat, C., Chelloul, N., Weil, M., Auclerc, G., Weisgerber, C., Tanzer, T., Bernard, J.: Malignancies possibly secondary to anticancer therapy. Recent Results in Cancer Research 49, 115–123 (1974)
Benaily, M., Schweisguth, O., Job, J. C.: Les tumeurs cortico-surrenales de l'enfant. Arch. Franç. Ped. 32, 441–453 (1975)
Filippi, G., McKurick, V. A.: The Beckwith-Wiedemann syndrome. Medicine 49, 279–298 (1970)
Fraumeni, J. F. jr., Geiser, C. F., Manning, M. D.: Wilms' tumor and congenital hemihypertrophy: Report of five new cases and review of the literature. Pediatrics 40, 886–889 (1967)
Fraumeni, J. F. jr., Miller, R. W.: Adrenocortical neoplasms with hemihypertrophy, brain tumors and other disorders. J. Pediat. 70, 129–137 (1967)
Fraumeni, J. F. jr., Miller, R. W., Hill, J. A.: Primary carcinoma of the liver in childhood: An epidemiologic study. J. Nat. Cancer Inst. 40, 1087–1099 (1968)
Geiser, C. F., Baez, A., Schindler, A. M., Shih, V. E.: Epithelial hepatoblastoma associated with congenital hemihypertrophy and cystathionuria: Presentation of a case. Pediatrics 46, 66–73 (1970)
Haicken, B. N., Schulman, N. H., Schneider, M. D.: Adrenocortical carcinoma and congenital hemihypertrophy. J. Pediatrics 83, 284–285 (1973)
Ishak, K. G., Glunz, P. R.: Hepatoblastoma and hepatocarcinoma in infancy and childhood: Report of 47 cases. Cancer 20, 396–422 (1967)
Knudson, A. G., Strong, L. C.: Mutation and Cancer: A model for Wilms' tumor of the kidney. J. Nat. Cancer Inst. 48, 313–324 (1972)
Knudson, A. G.: The genetics of childhood cancer. Cancer 35, 1022–1026 (1975)
Kosseff, A. L., Herrman, J., Gilbert, E. F., Viseskul, Ch., Lubinsky, M., Opitz, J. M.: Studies of malformation syndromes of man XXIX: The Wiedemann-Beckwith syndrome. Europ. J. Pediat. 123, 139–165 (1976)
Leck, I., Record, R. G., McKeown, T., Edwards, J. H.: The incidence of malformations in Birmingham, England, 1950–1959. Teratology 1, 263–280 (1968)
Li, F. P., Cassady, J. R., Jaffe, N.: Risk of second tumors in survivors of childhood cancer. Cancer 35, 1230–1235 (1975)
Meier, J. G., Harshaw, W. G.: Treatment and Prognosis in Wilms' tumor. A study of 51 cases with special reference to the role of actinomycin D. Cancer 20, 96–102 (1967)
Meadows, A. T., Lichtenfeld, J. L., Koop, C. E.: Wilms' tumor in three children of a woman with congenital hemihypertrophy. New Engl. J. Med. 291, 23–24 (1974)
Miller, R. W., Fraumeni, J. F. jr., Manning, M. D.: Association of Wilms' tumor with aniridia, hemihypertrophy, and other congenital malformations. New Engl. J. Med. 270, 922–927 (1964)
Miller, R. W.: Relation between cancer and congenital defects in man. New Engl. J. Med. 275, 87–93 (1966)
Miller, R. W.: Relation between cancer and congenital defects: an epidemiologic evaluation. J. Nat. Cancer Inst. 40, 1079–1085 (1968)
Miller, R. W.: Radiation-induced cancer. J. Nat. Cancer Inst. 49, 1221–1227 (1972)
Parker, D. A., Shalko, R. G.: Congenital asymmetry: report of 10 cases with associated developmental abnormalities. Pediatrics 44, 584–589 (19697)
Pendergrass, Th. W.: Congenital anomalies in children with Wilms' tumor. Cancer 37, 403–408 (1976)
Pfister, R. C., Weber, A. L., Smith, E. H., Wilkinson, R. H., May, D. A.: Congenital asymmetry (hemihypertrophy) and abdominal disease. Radiology 116, 685–691 (1975)
Reddy, I. K., Schimke, R. N., Chang, C. H. J., Svoboda, D. J., Slaven, J., Therou, L.: Beckwith-Wiedemann syndrome — Wilms' tumor, cardiac hamartomas, persistent visceromegaly, and glomeruloneogenesis in a 2-year old boy. Arch. Path. 94, 523–532 (1972)
Riedel, H. A.: Adrenogenital syndrome in a male child due to adrenocortical tumor. Report of a case with hemihypertrophy and subsequent development of embryoma (Wilms' tumor). Pediatrics 10, 19–27 (1952)
Ringrose, R. E., Jabbour, J. T., Keele, D. K.: Hemihypertrophy. Pediatrics 36, 434–448 (1965)
Schwartz, A. D., Lee, H., Baum, E. S.: Leucemia in children with Wilms' tumor. J. Pediatrics 87, No. 3, 374–376 (1975)
Simon, F. A., Drutz, J. E., Corriere, J. N.: Wilms' tumor and pigmented naevi. J. Pediatrics 90, 840 (1977)
Sotelo-Avila, C., Gooch, W. M.: Neoplasms associated with Beckwith-Wiedemann syndrome. Persp. Pediat. Path. 3, 255–272 (1976)
Valdes-Dapena, M., Arly, J. B.: Multiple (4) primary neoplasms in a child with aniridia. Am. J. Pathol. 62, 22A (1971) (abstr.)
Wiedemann, H. R.: Complexe malformatif familial avec hernie ombilicale et macroglossie- un “syndrome nouveau”? J. Genet. Hum. 13, 223–233 (1964)
Wiedemann, H. R.: E.M.G. syndrome. Lancet 1973a II, 626–627 a
Wiedemann, H. R.: Exomphalos-Makroglossie-Gigantismus-Syndrom, Berardinelli-Seip-Syndrom and Sotos-Syndrom — eine vergleichende Betrachtung unter ausgewählten Aspekten. Z. Kinderheilkunde 115, 193–207 (1973b)
Wolff, J. A.: Advances in the treatment of Wilms' tumor. Cancer 35, 901–904 (1975)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Müller, S., Gadner, H., Weber, B. et al. Wilms' tumor and adrenocortical carcinoma with hemihypertrophy and hamartomas. Eur J Pediatr 127, 219–226 (1978). https://doi.org/10.1007/BF00442064
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00442064