Primary Ewing's Sarcoma of the Cranium

Abstract

Although primary Ewing's sarcoma of the cranium is a malignant bone tumor, it is associated with a good prognosis when treated with radical surgery, aggressive multidrug chemotherapy, and radiotherapy.

Primary Ewing's Sarcoma of the Occipital Bone. Case Report.

“…1,8,27) The location is frequently the frontal and parietal convexities, 19,27) and less often the squama and the mastoid process of the temporal bone, 5,6,11,30) the ethmoid bone, 12) the orbit, 1,26,31) the petrous bone, 6,8,17,22,27) and the occipital bone. 6,9,32) Central nervous system involvement by secondary deposits from primary extracranial Ewing's sarcoma occurs in 10% to 33% of cases. 4,6,19) Early diagnosis is extremely important to obtain long-term survival because metastasis to the lungs and/or to other bones may occur in the very early stages of tumor development.…”

“…6,9,32) Central nervous system involvement by secondary deposits from primary extracranial Ewing's sarcoma occurs in 10% to 33% of cases. 4,6,19) Early diagnosis is extremely important to obtain long-term survival because metastasis to the lungs and/or to other bones may occur in the very early stages of tumor development. 7,23) Metastases to the cranium were found in 32% of cases and cerebral manifestations are common and occur within 2 years in 90% of cases.…”

“…15) In contrast to the frequent metastases from primary Ewing's sarcomas in general, metastases from primary Ewing's sarcoma of the cranium have not been reported. 6) Cranial CT or radiography of Ewing's sarcoma reveals lytic lesions with poor margination and often a`moth-eaten' appearance. The`onion-skin' appearance due to the periosteal reaction is more common in the long bones than in the spine.…”

“…Only 22 cases of primary cranial Ewing's sarcomas have been reported [1][2][3]5,[8][9][10][11][12]14,[16][17][18][19]21,22,[31][32][33] in addition to a series of 14 patients with primary cranial Ewing's sarcoma. 6) Ewing's sarcoma usually occurs in the long bones of the lower limbs, generally in patients less than 15 years of age. The tumor metastasizes early, and about 4% of cases are found to have skull involvement.…”

Giant Calvarial Ewing’s Sarcoma

“…Prognosis in Ewing's sarcoma has improved following the introduction of multidrug chemotherapy, and 5-year survival is 57.1% when radical surgery is followed by combination chemotherapy and radiotherapy [5].…”