Primary pericranial Ewing′s sarcoma on the temporal bone: A case report

Abstract

Background:Primary Ewing's sarcoma originating in the pericranium is an extremely rare disease entity.Case Description:A 9-year-old female patient was admitted to our department due to a left temporal subcutaneous mass. The mass was localized under the left temporal muscle and attached to the surface of the temporal bone. Head computed tomography revealed a mass with bony spicule formation on the temporal bone, however, it did not show bone destruction or intracranial invasion. F-18 fluorodeoxyglucose positron emission tomography showed no lesions other than the mass on the temporal bone. Magnetic resonance imaging showed that the mass was located between the temporal bone and the pericranium. The mass was completely resected with the underlying temporal bone and the overlying deep layer of temporal muscle, and was diagnosed as primary Ewing's sarcoma. Because the tumor was located in the subpericranium, we created a new classification, “pericranial Ewing's sarcoma,” and diagnosed the present tumor as pericranial Ewing's sarcoma.Conclusion:We herein present an extremely rare case of primary pericranial Ewing's sarcoma that developed on the temporal bone.

Giant Calvarial Ewing's Sarcoma: A Case Report

“… 6 Skull infiltration is rare with an incidence of 1 to 2% of EWS. 4 EWS mostly infiltrates frontal, parietal, temporal bones and skull base with the most common involvement being parieto-occipital region. 5 Squamous and petrous part of temporal, ethmoid, and mastoid bone are less frequently observed localizations of the tumor.…”

“…7 Although intracranial involvement was seen in the majority of calvarial EWSs; extracranial extension was observed in only eight cases in the literature. 4 …”

“…On F-18 FDG PET/CT, the tumor seems strongly labeled. 4 Peritumoral edema may be an indicator of high malignancy 9 . “Dural tail sign” can be seen with EWS and considered as a benign response but mostly it is an atypical tail because of the aggressive nature of the tumor.…”

“…3 In the literature, majority of papers on calvarial EWS present cases with intracranial involvement; extracranial extension is reported in only eight cases. 4 …”

Sechenov First Moscow State Medical University (Sechenov University)

“…Реже поражаются позвонки и ребра (12%), лопатки, кости черепа (6-9%) и мелкие кости стопы и кисти [2,16]. Локализация в области головы и шеи составляет от 1 до 4% случаев [11,13,21], с более частым поражением лобной или теменной костей.…”