Orbital Proptosis as the Sentinel Sign of Metastatic Desmoplastic Small Round Cell Tumor: A Rare Ophthalmic Gateway to Systemic Malignancy.
Vishakha T Vatkar, Yashi Yadava, Himani Yadav et al.
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Case in brief
A 56-year-old man presented with 15 days of rapidly progressive, painless left proptosis. Examination showed axial proptosis and peripapillary flame-shaped hemorrhages. MRI demonstrated a lobulated extraconal orbital mass extending contiguously into the anterior temporal fossa without bone erosion. Systemic evaluation identified a retroperitoneal mass and multiple hepatic lesions; CT-guided biopsy confirmed desmoplastic small round cell tumor (DSRCT) by histology and immunohistochemistry. The orbital mass was excised by lateral orbitotomy, and he received imatinib with multidisciplinary oncologic care.
What made this case unusual
Proptosis was the first clinical manifestation of occult metastatic DSRCT. Imaging also showed extra-axial intracranial extension without associated bone erosion.
Diagnostic / clinical pearl
Progressive unexplained proptosis can be the presenting sign of systemic malignancy; orbital imaging and systemic evaluation may reveal disease beyond the orbit.