Primary Extraskeletal Osteosarcoma Arising from the Optic Nerve: A Rare Case Report.

Area of Science:

• Ophthalmology
• Oncology
• Pathology

Background:

• Extraskeletal osteosarcoma (ESOS) is a rare malignant bone tumor occurring outside the skeleton.
• Orbital ESOS is exceedingly rare, with limited documented cases in medical literature.
• Early diagnosis and management are crucial for improving patient outcomes.

Purpose of the Study:

• To report a rare case of extraskeletal osteosarcoma in the orbit.
• To describe the clinical presentation, diagnostic imaging, and surgical management of this rare orbital tumor.
• To emphasize the importance of histopathological confirmation for accurate diagnosis and treatment planning.

Main Methods:

• A 32-year-old female presented with proptosis and vision loss.
• Contrast-enhanced computed tomography (CT) revealed a calcified, enhancing lesion in the left optic nerve.
• Surgical intervention involved fronto-occipito-zygomatic osteotomy, maxillectomy, ethmoidectomy, and orbital exenteration.
• Histopathological and immunohistochemical analyses confirmed extraskeletal osteosarcoma.

Main Results:

• The patient presented with unilateral proptosis and complete vision loss.
• Imaging demonstrated a densely calcified, enhancing lesion involving the left optic nerve.
• Histopathology confirmed the diagnosis of extraskeletal osteosarcoma.
• The tumor required extensive surgical resection including orbital exenteration.

Conclusions:

• Extraskeletal osteosarcoma of the orbit is a rare entity requiring a high index of suspicion.
• Multimodality imaging and histopathological examination are essential for diagnosis.
• Aggressive surgical management followed by adjuvant chemotherapy and radiotherapy is often necessary.