Open AccessExtraskeletal osteosarcoma of the orbit: A clinicopathologic case report and review of literature
Author(s) -
V M D S de Maeyer,
Philippe Kestelyn,
Akash Shah,
C. Van den Broecke,
Hannelore Denys,
CE Decock
Publication year - 2016
Publication title -
indian journal of ophthalmology/indian journal of ophthalmology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 51
eISSN - 1998-3689
pISSN - 0301-4738
DOI - 10.4103/0301-4738.97555
Subject(s) - medicine , orbit (dynamics) , osteosarcoma , ret proto oncogene , malignancy , horseshoe kidney , radiology , pathology , kidney , mutation , biochemistry , chemistry , germline mutation , engineering , gene , aerospace engineering
Primary extraskeletal osteosarcoma (EOS) is an extremely rare malignancy. In this report, the clinical course of a 32-year-old 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 proto-oncogene for HD and the p53 tumor suppressor gene for osteosarcoma.