Extraskeletal osteosarcoma of the orbit: A clinicopathologic case report and review of literature.
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Date
2016-09
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Abstract
Primary extraskeletal osteosarcoma (EOS) is an extremely rare
malignancy. In this report, the clinical course of a 32-yearold
man presenting with proptoses is described. Medical
history included Hirschsprung disease (HD), horseshoe
kidney, azoospermia, and vertebral anomalies. Imaging of
the orbit showed an oval, well-defined heterogeneous mass
adjacent to the lateral wall of the orbit. The patient underwent
a lateral orbitotomy and complete excision of the mass. The
mass was not attached to the bone. Histopathologic and
immunohistochemical examination confirmed the diagnosis of
an EOS. The patient received chemotherapy and radiotherapy
and is free of the disease 3 years after the diagnosis. Genetic
screening showed no mutations for both the RET protooncogene
for HD and the p53 tumor suppressor gene for
osteosarcoma.
Description
Keywords
Extraskeletal osteosarcoma, Hirschsprung disease, p53 tumor suppressor gene, RET proto-oncogene
Citation
de Maeyer V M D S, Kestelyn P A F A, Shah Akash D, Broecke C M Van Den, Denys H G N, Decock C E. Extraskeletal osteosarcoma of the orbit: A clinicopathologic case report and review of literature. Indian Journal of Ophthalmology. 2016 Sept; 64(9): 687-689.