Abstract ID: 26-380
A Dreadful Dystopia : Primary Orbital Mesenchymal Chondrosarcoma Unmasked
Author: JAYAGAYATHRI RAJAGOPALAN Base Hospital / Institution: Aravind Eye Hospital, Pondicherry
Presentation Type: ePoster Presentation
Purpose
To report a case of relatively sudden onset progressive proptosis in a middle aged female
Methods
Prospective case study of a 51-year-old female with a two-week history of painful left eye proptosis and restricted extraocular movements.
Results
MRI revealed a 2×1.5×2 cm superior extraconal mass localised near the superior rectus muscle and involving it. She presented with inferior dystopia and abaxial proptosis. Initial management with oral steroids for suspected inflammatory disease failed, resulting in clinical worsening. An incision biopsy revealed the pathognomonic biphasic histological pattern, undifferentiated spindle-shaped mesenchymal cells, and islands of mature hyaline cartilage with areas of calcification. Immunohistochemistry confirmed strong CD99 positivity. Following the diagnosis of ESMC, the patient underwent lid-sparing exenteration and was referred for adjuvant radiotherapy.
Conclusion
Extra Skeletal Mesenchymal chondrosarcoma (ESMC) is an exceptionally rare, high-grade malignant tumor. Only 44 cases of ESMC has been reported so far in the literature. This case underscores the importance of considering ESMC in cases of rapidly progressive proptosis and the necessity of histopathological confirmation for this aggressive malignancy