Ewing’s Sarcoma of the Skull in an Infant

Abstract

A 1-year-old female infant presented with a large 7–8 cm Ewing’s sarcoma of the left parietal calvarium. It deeply indented the subjacent hemisphere. Wide surgical excision was followed by chemotherapy but not radiation. The patient was clinically free of tumor when she expired 9 months later of sepsis. Though primary Ewing’s sarcoma of the skull is said to be relatively rare, we have tabulated 37 cases of this disorder, including the present one (we excluded mandibular tumors (>100 cases) since they are not usually treated by neurosurgeons). The mean age was 11 years and of those available to follow-up, three quarters were tumor-free 6 months to 7 years postsurgery. The remainder survived an average of 21 months. Ewing’s tumors of the calvarium generally grant a favorable prognosis following surgery and/or radiation and chemotherapy. Those at the base of the brain may be more difficult to eradicate. In addition to primary Ewing’s tumors, metastases to the skull and the brain from other primary sources in the skeleton have been documented in several reports.

Mass in the Forehead of a Three-Year-Old Girl

“…Ewing sarcoma has a predilection for the first two decades of life. Primary calvarial Ewing sarcoma is exceedingly rare, with fewer than 50 reported cases [21]. Rhabdomyosarcomas may affect children, presenting as a mass at the orbit, nasopharynx or paranasal sinuses [22].…”

Tumores ósseos raros da calota craniana: sarcoma de Ewing e tumor de Triton

“…O sarcoma de Ewing se desenvolve primariamente das células reticulares imaturas do mesênquima primitivo da cavidade medular. A localização primária, usual desses tumores, é nos ossos longos (2,3,5) . A maioria dos pacientes apresenta dor e edema no local da lesão, encontrando-se, na área acometida, calor e massa palpável (2,3) .…”

Primary Ewing’s Sarcoma of the Skull in Children

“…When originating in these sites, ES has a marked propensity for dissemination and metastasis. By way of contrast, primary ES of the cranium is rare [9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19]accounting for approximately 6% of all cases. Within the cranium, ES occurs most frequently at the cranial base [9, 10, 15], but several cases of ES affecting the cranial vault have also been described [12, 15, 17].…”

“…Within the cranium, ES occurs most frequently at the cranial base [9, 10, 15], but several cases of ES affecting the cranial vault have also been described [12, 15, 17]. Primary ES of the skull typically grows as an extradural mass lesion which rarely breaches the dura, and as such, can grow to an extremely large size before detection [13]. Recently, a pPNET originating from the tentorium was described [20].…”

“…Recently, a pPNET originating from the tentorium was described [20]. Unlike ES arising from the extremities or pelvis, primary cranial ES rarely metastasizes [13]. …”