Primary intraosseous desmoplastic small round cell tumor of the calvarium: Case report and review of the literature  Vadim Khachaturov, MD, Roth W. Christopher, Jacob R. Hodge, MD, John Joseph Doyle, MD, Darren J. Leitao, MD, Camelia Stefanovici, MD, Karen Fritchie, MD  Human Pathology: Case Reports  Volume 2, Issue 1, Pages 9-15 (March 2015) DOI: 10.1016/j.ehpc.2014.11.011 Copyright © 2015 Terms and Conditions

Fig. 1 Non-contrast axial (A) and reformatted coronal (B) CT images of the calvarium showing an aggressive intraosseous lesion which is extensively involving the right sphenoid and temporal bones. There is dense patchy sclerosis with osseous expansion and a prominent "sunburst" periosteal reaction. The lesion extends into the orbit and middle ear. Human Pathology: Case Reports 2015 2, 9-15DOI: (10.1016/j.ehpc.2014.11.011) Copyright © 2015 Terms and Conditions

Fig. 2 A. Intraoperative gross photograph of the right ear canal demonstrating an exophytic, lobulated pink-tan mass. B. Intraoperative photograph of the patient's right side showing a pre-auricular facelift incision, exposing a glistening white-tan pre-auricular tumor. The temporalis muscle and ear canal are labeled for orientation. Human Pathology: Case Reports 2015 2, 9-15DOI: (10.1016/j.ehpc.2014.11.011) Copyright © 2015 Terms and Conditions

Fig. 3 A. Microscopically the tumor is composed of diffuse sheets of neoplastic cells, some in vague nests with associated dense desmoplasia and scattered mature bony trabeculae. The tumor consists of round, hyperchromatic cells with inconspicuous nucleoli and prominent perinuclear cytoplasmic clearing (hematoxylin–eosin, original magnification ×100 and ×400 [inset]). B. The tumor cells are diffusely reactive for desmin with a distinct perinuclear dot-like configuration (immunohistochemistry, original magnification ×200 and ×400 [inset]). Human Pathology: Case Reports 2015 2, 9-15DOI: (10.1016/j.ehpc.2014.11.011) Copyright © 2015 Terms and Conditions

Fig. 4 Sanger sequencing of the EWSR1-WT1 fusion transcript with a rare in-frame junction of EWSR1 exon 10 to WT1 exon 8. Human Pathology: Case Reports 2015 2, 9-15DOI: (10.1016/j.ehpc.2014.11.011) Copyright © 2015 Terms and Conditions

Fig. 5 Non-contrast axial CT image five months post chemotherapy and radiotherapy showing diffuse and prominent tumor calcification with minimum change in tumor size. Human Pathology: Case Reports 2015 2, 9-15DOI: (10.1016/j.ehpc.2014.11.011) Copyright © 2015 Terms and Conditions