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Research Letter |

Prenatal Presentation of Fronto-orbital Congenital Infantile Fibrosarcoma:  A Clinicopathologic Report

Hughie H. Tsang, MBBS1,5; Peter J. Dolman, MD, FRCSC1,6; Douglas J. Courtemanche, MD, MS, FRCSC3,7; Shahrad Rod Rassekh, MD, MHSc, FRCPC4,8; Christof Senger, MD, FRCPC2,9; Christopher J. Lyons, MBBS, FRCSC1,5
[+] Author Affiliations
1Department of Ophthalmology, BC Children’s Hospital, Vancouver, British Columbia, Canada
2Department of Anatomical Pathology, BC Children’s Hospital, Vancouver, British Columbia, Canada
3Division of Plastic Surgery, BC Children’s Hospital, Vancouver, British Columbia, Canada
4Division of Pediatric Hematology/Oncology/Blood and Marrow Transplantation, BC Children’s Hospital, Vancouver, British Columbia, Canada
5Division of Pediatric Ophthalmology, Department of Ophthalmology and Visual Sciences, University of British Columbia, Vancouver, British Columbia, Canada
6Division of Oculoplastics/Orbit, Department of Ophthalmology and Visual Sciences, University of British Columbia, Vancouver, British Columbia, Canada
7Division of Plastic Surgery, Department of Surgery, University of British Columbia, Vancouver, British Columbia, Canada
8Division of Hematology/Oncology/Blood and Marrow Transplant, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada
9Department of Pathology, University of British Columbia, Vancouver, British Columbia, Canada
JAMA Ophthalmol. 2013;131(7):965-967. doi:10.1001/jamaophthalmol.2013.1934.
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Extract

Congenital infantile fibrosarcoma (CIFS) is a mesenchymal tumor that occurs in the first year of life and rarely involves the orbit. We describe a patient with a prenatal presentation of orbital and forehead CIFS.

Article InformationCorresponding Author: Dr Lyons, Room A136, Department of Ophthalmology, BC Children’s Hospital, 4480 Oak St, Vancouver, BC V6H 3V4, Canada (clyons@cw.bc.ca).

Published Online: April 4, 2013. doi:10.1001/jamaophthalmol.2013.1934

Conflict of Interest Disclosures: None reported.

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Figures

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Figure 1.
Ultrasonographic, Clinical, Computed Tomographic, and Magnetic Resonance Images

A, The 4.5 × 4.2 × 3.0-cm mass on a prenatal ultrasonographic scan at 37 weeks’ gestation. B, Clinical image from day 1 of life. C, Sagittal computed tomographic image showing globe displacement and compression by the mass. D, Postnatal sagittal magnetic resonance image showing a large variegated lesion with patchy intralesional hyperattenuations consistent with hemorrhage.

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Figure 2.
Histopathologic Findings and Clinical Image

A, The tumor is cellular and composed of plump and spindle-shaped fibroblast-type stromal cells with a moderate amount of slightly eosinophilic cytoplasm (hematoxylin-eosin, original magnification ×400). B, The fibroblast-type cells stained for vimentin only. Fluorescence in situ hybridization confirmed the ETV6-NTRK3 translocation specific for congenital infantile fibrosarcoma. Other markers, including CD99, actin, desmin, myogenin, anaplastic lymphoma kinase 1, myeloperoxidase, CD117, S-100 protein, and BAF47, were negative or nonsupportive of other mesenchymal tumors (vimentin, original magnification ×400). C, Immunohistochemical staining for CD163 highlights a prominent reactive inflammatory component, mostly histiocytes (CD163, original magnification ×400). D, Clinical image at age 10 months.

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