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Originally Considered "Rhabdomyosarcomatoid"
Variant
of Wilms Tumor (abundant eosinophilic cytoplasm)
- Lack of Myofilaments (Cross-striations)
- Lack of Myofibroblastic Differentiation
- Lack of Myoglobin, Actin, Desmin, Muscle Markers
Uncertain Etiology
Association with Primitive Neuroepithelial
Tumors of
Posterior and Middle Cranial Fossa in 15%
Median Age 11 months
90% of Cases <3 years of age
Oldest Case 8.5 years of age
Gender Ratio 1.5M : 1.0F
Hypercalcemia with Elevated Parathormone
Levels
No Associated Syndromes
Short, Unrelenting Clinical Course:
Resistant to Chemotherapy and Radiotherapy
Usually, Die of Disease Within 1 year
of Diagnosis
Metastasize Widely via Hematogenous
and Lymphatic Routes |
