- Department of Neurosurgery. Shiga University of Medical Science, Setatsukinowa-cho, Shiga, Japan
- Department of Clinical Laboratory Medicine and Division of Diagnostic Pathology, Shiga University of Medical Science, Setatsukinowa-cho, Shiga, Japan
Department of Neurosurgery. Shiga University of Medical Science, Setatsukinowa-cho, Shiga, Japan
DOI:10.4103/2152-7806.183545Copyright: © 2016 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Kawano H, Nitta N, Ishida M, Fukami T, Nozaki K. Primary pericranial Ewing's sarcoma on the temporal bone: A case report. Surg Neurol Int 03-Jun-2016;7:
How to cite this URL: Kawano H, Nitta N, Ishida M, Fukami T, Nozaki K. Primary pericranial Ewing's sarcoma on the temporal bone: A case report. Surg Neurol Int 03-Jun-2016;7:. Available from: http://surgicalneurologyint.com/surgicalint_articles/primary-pericranial-ewings-sarcoma-on-the-temporal-bone-a-case-report/
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.
Keywords: Ewing's sarcoma, pericranium, skull, temporal bone
Ewing's sarcoma, a small round cell tumor that was first described by James Ewing in 1921, is most commonly diagnosed in the second decade of life.[
A 9-year-old girl had been well until 2 months before admission when she noticed a small subcutaneous mass in the left temporal region. The mass thereafter rapidly became larger. A skull X-ray at the first hospital showed no bone defects or expansion; however, a skull X-ray with dynamic range compression modification showed a sunburst appearance on the left temporal bone [
Preoperative images. A skull X-ray with dynamic range compression (a), axial computed tomography of the skull (b), F-18 fluorodeoxyglucose positron emission tomography (c), axial T2-weighted imaging (d), and postcontrast T1-weighted imaging of the axial plane (e) and coronal plane (f). Arrows and arrowheads indicate the enhanced outer and inner membranous structures, respectively
The histopathological features of the tumor. Hematoxylin and eosin staining of the tumor (a) and the outer surface of the temporal bone (b-d). The tumor infiltrated some of the marrow cavities (black arrows in [b]), but not others (white arrow in [b]). The tumor invaded the compact bone (c) and infiltrated the marrow cavities under the thin compact bone (d). The black arrowheads in (b-d) show the surface of the temporal bone. Scale bars: 50 μm (a, c, d); 200 μm (b)
In the present case, Ewing's sarcoma was located between the pericranium and the temporal bone. The main imaging and histopathological findings were as follows. First, the skull X-ray and CT showed a sunburst appearance as a periosteal reaction, without the presence of a bone defect. Second, MRI showed two enhanced membranous structures that surrounded the tumor and were connected to the pericranium. Third, although the tumor was mainly located outside the temporal bone, the histopathological analysis revealed that it had invaded some of the marrow cavities in the temporal bone. These findings suggested pericranial Ewing's sarcoma with mild invasion into the membranous bone.
Ewing's sarcoma, a small round cell tumor, is most often diagnosed in the second decade of life and is the second most common form of primary bone cancer in childhood.[
The periosteal reaction is a rare radiographic appearance in primary Ewing's sarcoma cases that involve the bones of the head and neck.[
Periosteal Ewing's sarcoma is a rare form of Ewing's sarcoma that particularly affects the femur.[
Then, what were the cells of origin in the present case? Ewing's sarcoma expresses the EWS-FLI-1 fusion gene, which is generated by chromosomal translocation, whereas EWS-FLI-1 induces cell cycle arrest in normal murine and human fibroblasts because of EWS-FLI-1-mediated cytotoxicity.[
We herein reported the first known case of primary pericranial Ewing's sarcoma which occurred in a 9-year-old girl.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
We are grateful to Akira Saito, Takashi Taga, and Kenji Takagi for fruitful discussions and helpful comments. We report no conflicts of interest.
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