Ito F, Watanabe Y, Harada T, Horibe K
Department of Pediatric Surgery and Pediatrics, Nagoya University School of Medicine, Japan.
J Pediatr Hematol Oncol. 1997 Sep-Oct;19(5):466-9. doi: 10.1097/00043426-199709000-00012.
This report describes extremely rare cases of infantile rhabdomyosarcoma with multiple skin nodules. They are of interest not only for their anatomic sites, but also for subsequent cerebral metastases with sudden cranial hypertension.
Two infants had multiple skin nodules and excisional biopsy revealed alveolar type rhabdomyosarcomas. The patients were treated with tumor resection and combined chemotherapy without any clinical progression for 9 and 16 months, respectively.
Evidence of cerebral metastases developed with sudden vomiting and convulsion as the first manifestation. In one patient, urgent radiographic examinations failed to reveal lesions except for dilated cerebral ventricles. Seven weeks after the onset of the neurologic symptoms, only Gd-DPTA-enhanced magnetic resonance imaging (MRI) revealed multiple punctate metastatic lesions hyperintense to the surrounding cerebral tissue. Despite appropriate chemotherapy, both patients had disease progression and died of central nervous system metastases.
The authors emphasize the need to recognize the multiple cutaneous presentation of infantile rhabdomyosarcoma and the association of cerebral metastases as a potential and fatal complication. The diagnosis is facilitated by Gd-DPTA-enhanced MRI, particularly when cerebral computed tomography scans fail to disclose metastatic lesions.
