Abstract ID: 26-507
Giant Cell Tumor of Soft Tissue of the Orbit in an 11-Year-Old Girl: A Case Report and Review of the Literature
Author: Ángela Molina Berenguer Base Hospital / Institution: Puerta del Mar University Hospital
Presentation Type: ePoster Presentation
Purpose
To report an exceptionally rare case of periocular giant cell tumor of soft tissue (GCT-ST) in a pediatric patient and to systematically review all published head and neck GCT-ST cases.
Methods
We conducted a retrospective clinical chart review for the present case. Concurrently, we performed a systematic literature search following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) guidelines to extract demographic, clinical, and histopathological data for all published cases of GCT-ST in head and neck and periocular locations.
Results
An 11-year-old girl presented with a painless mass near the medial canthus mimicking a dermoid cyst. Orbital CT showed a solid lesion with minimal bone remodeling and higher density than typical for a cyst. Complete macroscopic excision via a left inferior anterior orbitotomy yielded microscopic margins in contact with the lesion (R1). Histopathology and immunohistochemistry (CD68+, SMA+, H3.3 G34W negative) confirmed GCT-ST. Given its expected low malignant potential, the patient’s young age, and the risk to critical adjacent structures, no further resection or adjuvant therapy was performed. At 37 months, surveillance imaging confirms she remains disease-free. The systematic review identified 20 head and neck cases (median age, 47 years). The periocular subgroup (comprising 3 cases, including ours) was exclusively female with a younger median age (11 years) and smaller tumors compared to the global cohort.
Conclusion
Periocular GCT-ST is exceptionally rare. The present case exhibits the longest disease-free interval reported to date. This supports that, although aggressive variants may occur, conservative surgical management combined with close long-term surveillance can yield durable control in pediatric patients, despite microscopically positive margins.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Hugo | González Valdivia | Sant Joan de Déu Hospital |