CEREBRAL METASTASES OF ALVEOLAR RHABDOMYOSARCOMA IN AN INFANT WITH MULTIPLE SKIN NODULES

Purpose: This report describes extremely rare cases of infantile rhabd omyosarcoma with multiple skin nodules. They are of interest not only for their anatomic sites, but also for subsequent cerebral metastases with sudden cranial hypertension. Patients: Two infants had multiple s kin nodules and excisional biopsy revealed alveolar type rhabdomyosarc omas. The patients were treated with tumor resection and combined chem otherapy without any clinical progression for 9 and 16 months, respect ively. Results: Evidence of cerebral metastases developed with sudden vomiting and convulsion as the first manifestation. In one patient, ur gent radiographic examinations failed to reveal lesions except for dil ated cerebral ventricles. Seven weeks after the onset of the neurologi c symptoms, only Gd-DPTA-enhanced magnetic resonance imaging (MRI) rev ealed multiple punctate metastatic lesions hyperintense to the surroun ding cerebral tissue. Despite appropriate chemotherapy, both patients had disease progression and died of central nervous system metastases. Conclusions: The authors emphasize the need to recognize the multiple cutaneous presentation of infantile rhabdomyosarcoma and the associat ion of cerebral metastases as a potential and fatal complication. The diagnosis is facilitated by Gd-DPTA-enhanced MRI, particularly when ce rebral computed tomography scans fail to disclose metastatic lesions.