Orbital Proptosis as the Sentinel Sign of Metastatic Desmoplastic Small Round Cell Tumor: A Rare Ophthalmic Gateway to Systemic Malignancy.

Ann Afr MedJul 15, 2026 (epub)
Case ReportOphthalmologyOncology (Medical)Radiology (Diagnostic)Pathology (Anatomic & Clinical)

Vishakha T Vatkar, Yashi Yadava, Himani Yadav et al.

✦ AI-curated · Sources linked

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.

Source

PubMed · PMID 42458992

Published in Ann Afr Med. This summary was written by xxcode from the publication's abstract and metadata. It is not peer reviewed and is not a substitute for the original article. For clinical decisions, review the original publication.

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