Neuroblastoma by Unknown

Author:Unknown
Language: eng
Format: epub
ISBN: 9783030183967
Publisher: Springer International Publishing

10.8.4 Stage 4S Disease

Stage 4s is a “special” group of patients who have a special pattern of diffuse involvement and carry a good prognosis [38]. Infants younger than 1 year with dissemination limited to the skin or liver or less than 10% of neuroblasts in the bone marrow, without bone metastases, are considered to have a stage 4s disease. Gigliotti et al. reported 45 of 134 newborns (33%) having stage 4s disease [4]. Although these patients have extensive metastatic disease, these tumors generally undergo spontaneous regression even without treatment. Therefore, patients are generally treated with supportive therapy only and have excellent outcome [39]. However, this disease can cause significant life-threatening complications, particularly in the neonatal period [40]. The signs and symptoms of organ distress caused by hepatomegaly can occur in the lungs, the kidneys, the gastrointestinal tract, the inferior vena cava, and the liver [41]. A scoring scale reflecting organ compromise was developed, the scores ranging from 0 to 10. Scores were derived for 32 of 35 patients; 13 were neonates when first seen, and 19 were infants aged 1–12 months. Neonates were more likely than infants to develop increasing symptomatology (50% versus 25%) and were more likely to die when a score of 2 or more developed. None of the six neonates who did so survived despite treatment, compared with three of four infants. Early intervention is recommended for 4s neonates who develop a score of 1.

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