Systemic @entity3 from XXXX : report of two cases and review of the literature.

@entity1 @entity5 @entity158 @entity3 @entity2946
The clinical and pathologic data from two cases of @entity2946 with systemic @entity3 , and 101 previously reported cases were evaluated to define better the clinical presentation and natural history. @entity1 ranged in age from six months to 48 years, with a mean of 13 years; two thirds of the @entity1 were male. Ventricular shunts had been inserted in 20% of the @entity1 . Systemic @entity3 occurred on an average of two years after the diagnosis of the @entity5 in @entity1 without shunts, but only 1.3 years in @entity1 with shunts. Fifty-nine percent of the @entity1 were known to have experienced recurrence or spread of @entity2946 within the central nervous system by the time systemic @entity3 appeared. Ninety percent showed radiologic evidence of bone @entity3 , of which 60% were osteoblastic. Bones most frequently involved were pelvis, femur and vertebrae; @entity158 was the most common initial symptom. At autopsy, lymph node @entity3 were found in 65% and liver @entity3 were found in 28% of all cases in addition to bone @entity3 in 82%. Lung @entity3 occurred in 9% of the @entity1 without shunts, compared with 30% of @entity1 with shunts. The average survival was seven months after the appearance of systemic @entity3 for @entity1 both with and without shunts. Approximately 5% of @entity1 with @entity2946 may be expected to develop systemic @entity3 . This development is associated with increased morbidity and a shortened life expectancy.
