Congenital disseminated extrarenal XXXX and @entity5606 .

@entity1 @entity2741 @entity5 @entity5606 @entity600 @entity6195
UNASSIGNED: Soft tissue @entity5 arising in association with genetic or @entity600 have been increasingly reported. @entity5606 ( @entity5606 ) is a highly aggressive @entity5 of infancy and young childhood, characterized by typical morphology and biallelic inactivation of the @entity6195 ( @entity6195 /SMARCB5/ @entity6195 ) gene on chromosome 22q.2. @entity5606 represents a unique clinicopathologic presentation of this @entity5 . We report a case occurring in a female neonate which presented at birth a voluminous left thigh mass. Surgical biopsy performed at day 9 showed morphology and immunoprofile of @entity5606 . Staging evaluation identified @entity2741 and distant nodules. The mass showed rapid growth. Despite chemotherapy, the @entity5 progressed with exteriorization through the biopsy scar. Chemotherapy was discontinued and treatment limited to palliative care and the @entity1 died on day 51. The @entity5 was homozygous for the @entity6195 deletion with apparent de novo heterozygous germ line deletion in the @entity1 , not identified in the parents.
