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Isolated skull base primary Ewing's sarcoma: an extremely rare location
Goutham Cugati1, Manish Singh, Anil Pande
1Department of Neurosurgery, JSS Medical College and Hospital, Mysore, Karnataka, India.
Abstract:
A 16-year-old boy presented with history of headache and blurring of distant vision. He had UMN facial paresis and papilledema. Imaging revealed a heterogenous space occupying lesion in the middle cranial fossa base with mass effect and midline shift and non homogenous contrast enhancement with erosion of the petrous bone. He underwent gross total excision of the lesion. Histopathology and immunohistochemistry were suggestive of skull base Ewing's sarcoma. Bone scan was done to rule out primary involvement of other extracranial location. We have discussed the epidemiology, presentation, management and prognosis of such cases.
Insights
A rare skull base Ewing's sarcoma was diagnosed in a 16-year-old boy presenting with vision loss and facial weakness. Surgical removal and further evaluation were performed to assess the extent of this bone cancer.
Area of Science:
- Neuro-oncology
- Pediatric Oncology
- Skull Base Surgery
Background:
- Skull base Ewing's sarcoma is a rare malignancy, often presenting with non-specific neurological symptoms.
- Early diagnosis and surgical intervention are crucial for managing pediatric skull base tumors.
Observation:
- A 16-year-old male presented with headache, blurred vision, upper motor neuron facial paresis, and papilledema.
- Imaging demonstrated a heterogeneous mass in the middle cranial fossa base with mass effect, midline shift, and petrous bone erosion.
Findings:
- Gross total excision of the lesion was achieved.
- Histopathology and immunohistochemistry confirmed skull base Ewing's sarcoma.
- A bone scan was conducted to exclude extracranial metastasis.
Implications:
- This case highlights the importance of considering rare malignancies in the differential diagnosis of pediatric skull base lesions.
- Multidisciplinary management, including surgical resection and oncological workup, is essential for optimal outcomes.
- Further research into the epidemiology, presentation, and long-term prognosis of skull base Ewing's sarcoma is warranted.
