Childhood cancer is relatively rare in all Western and developed countries. In the United States, approximately 4000 new cases of malignant solid tumors are diagnosed each year , while data from cancer registries in Europe indicate that the fraction of all pediatric malignancies (i.e., diagnosed between 0 and 14 years of age) is in the order of 0.8% of all registered tumors . Despite the relative rarity, there are several reasons for interest in these tumors. First, for many cancers of childhood a genetic nature has been suspected, and then documented, by observing the striking familial aggregation and vertical transmission of uncommon tumors; retinoblastoma (Rb) , Wilms' tumor (WT) , and Li-Fraumeni syndrome are examples of well defined hereditary neoplastic diseases of infancy. Second, some of these neoplasms (Rb and WT, in particular) have become a paradigm for interpreting tumor development as being due to the sequential inactivation of the two alleles in a tumor suppressor gene (see the previous chapter) . This model , initially suggested for Rb , is applicable to many other clinical conditions which are at present under active investigation (adenomatosis coli, hereditary bre ast and colonic cancer). Third, the distribution of childhood cancer is rather peculiar and markedly different from that of adult malignant tumors, as shown in Fig. 1. In fact, tumors of the Iymphohematopoietic system represent 50% of all malignancies developed before the age of 15, brain tumors 22%, and sarcoma 11%; all together, carcinomas do not reach 6% of tot al pediatric tumors. Cancer distribution is entirely different in adults , where carcinomas represent the large majority (89% of total), and altogether the most frequent neoplasms of childhood do not reach 12% of all registered tumors. The reasons for this striking difference in cancer distribution are unclear, though rather intuitively one may suspect that genetic factors affect pediatric tumors more than the adult types, which in turn should be more closely dependent on the cumulative effect of environmental factors.
Childhood Cancer Mesoblastic Nephroma Hereditary Case Adrenal Medullary Hormone Painless Abdominal Mass
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Call MK, Glaser T, Ito CY et al. (1990) Isolation and characterization of a zinc finger polypeptide on the human chromosome 11 Wilms’ tumor locus. Cell 60:509–520PubMedCrossRefGoogle Scholar
Riccardi VM, Sujansky E, Smith AC et al. (1978) Chromosomal imbalance in the aniridia-Wilms’ tumor association: 11p interstitial deletion. Pediatrics 61:604–610PubMedGoogle Scholar
Fearon ER, Vogelstein B, Feinberg A (1984) Somatic deletion and duplication of genes on chromosome 11 in Wilms’ tumours. Nature 309:176–178PubMedCrossRefGoogle Scholar
Weissman BE, Saxon PJ, Pasquale SR et al. (1987) Introduction of a normal human chromosome 11 into a Wilms’ tumor cell line controls its tumorigenic expression. Science 236:175–180PubMedCrossRefGoogle Scholar
Baird PN, Groves N, Haber DA et al. (1992) Identification of mutations in the WT-1 gene in tumours from patients with WAGR syndrome. Oncogene 7:2141–2149PubMedGoogle Scholar
Gessler M, Konig A, Moore J et al. (1993) Homozygous inactivation of WT-1 in a Wilms’ tumor associated with WAGR syndrome. Genes Chromos Cancer 7:131–136PubMedCrossRefGoogle Scholar
Dowdy SF, Fasching CL, Araujo D et al. (1991) Suppression of tumorigenicity in Wilms’ tumor by the pl5.5-pl4 region of chromosome 11. Science 254:293–295PubMedCrossRefGoogle Scholar
Reeve AE, Sa S, Raizis AM et al. (1989) Loss of allelic heterozygosity at a second locus on chromosome 11 in sporadic Wilms’ tumor. Mol Cell Biol 9:1799–1803PubMedGoogle Scholar
Cowell JK, Groves N, Baird P (1993) Loss of heterozygosity at Ilpl3 in Wilms’ tumours does not necessarily involve mutations in the WT-1 gene. Br J Cancer 67:1259–1261PubMedCrossRefGoogle Scholar
Wiedemann HR (1964) Complex malformatif familial avec hernie ombilicale et macroglossie. Un syndrome noveau? J Genet Hum 13:223–232PubMedGoogle Scholar
Sotelo-Avila C, Crooch WM (1976) Neoplasms associated with the Beckwith-Wiedemann syndrome. Perspect Pediatr Pathol 3:255–272PubMedGoogle Scholar
Wiedemann HR (1983) Tumor and hemihypertrophy associated with Wiedemann-Beckwith’s syndrome. Eur J Pediatr 141:129–131CrossRefGoogle Scholar
Ping AJ, Reeve AE, Low DJ et al. (1989) Genetic linkage of Wiedemann-Beckwith syndrome to 11pl5. Am J Hum Genet 44:720–723PubMedGoogle Scholar
Nikawa N, Ishikariyama S, Takahashi S et al. (1986) The Wiedemann-Beckwith syndrome. Pedigree studies of 5 families with evidence for autosomal dominant inheritance with variable expressivity. Am J Med Genet 24:41–55CrossRefGoogle Scholar
Smith EI, Krous HF, Tunell WP et al. (1980) The impact of chemotherapy and radiation therapy on secondary operations for neuroblastoma. Ann Surg 191:561–569PubMedCrossRefGoogle Scholar
Rosen EM, Cassady JR, Frantz CN et al. (1984) Neuroblastoma: the joint center for radiation therapy/Dana Farber cancer Institute/Children hospital experience Neuroblastoma: the joint center for radiation therapy/Dana Farber cancer Institute/Children hospital experience. J Clin Oncol 2:719–732PubMedGoogle Scholar
Schwab M, Alitalo K, Klempnauer KH et al. (1983) Amplified DNA with limited homology to myc cellular oncogene is shared by human neuroblastoma cell lines and a neuroblastoma tumor. Nature 305:245–248PubMedCrossRefGoogle Scholar
Brodeur GM, Azar C, Brother M et al. (1992) Neuroblastoma: effect of genetic factors on prognosis and treatment. Cancer 70:1685–1694PubMedCrossRefGoogle Scholar
Nakagawara A, Arima-Nakagawara M, Scavarda NJ et al. (1993) Association between high levels of expression of the TRK gene and favorable outcome in human neuroblastoma. N Engl J Med 328:847–854PubMedCrossRefGoogle Scholar
Leone A, Seeger RC, Hong CM et al. (1993) Evidence for nm23 RNA overex-pression, DNA amplification and mutation in aggressive childhood neuroblastoma. Oncogene 8:855–865PubMedGoogle Scholar