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a Relative proportions are based on data from POG 9047 for whom risk group was known b The EFS and S rates are based on all patients from POG 9047 for whom risk group was known

The primary site in infants and children who have widespread disease is the retroperitoneum in at least 70-80% of cases,whereas the primary site is extraabdominal (mainly thorax and pelvis) in up to 50 % of children with stage-3, favorable biology tumors (Matthay et al. 1998). Infants with widespread neu-roblastoma typically come to medical attention because of abdominal distention from large liver tumors or because of periorbital ecchymoses from metastatic involvement of cranial bones. Subcutaneous tumor nodules are another characteristic, though less common, presenting sign in infants. The distribution of distant sites of disease - bone, bone marrow, liver, skin - differs greatly between infants and older patients, as evidenced in a large Children's Cancer Group (CCG) study of 648 patients (DuBois et al. 1999). In stage-4S patients («=81), bone marrow, liver, and skin were involved in 34.6,80.2, and 13.6% of cases, respectively. With stage-4 disease, os-teomedullary involvement was significantly less frequent in infants («=133) than in older patients («=434): 48.9 vs 68.2% for bone, and 57.1 vs 81.3% for bone marrow. Conversely, liver involvement with stage 4 was significantly more common in infants than in older patients: 53.4 vs 12.9%. Thus, some infants who are classified as having stage 4 by virtue of large primary tumors may actually have a pattern of distant disease that more closely resembles stage 4S.

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