Intraorbital Ancient Schwannoma with Intracranial Extension: A Case Report

Abstract

Introduction: Intra-orbital ancient schwannomas are exceedingly rare nerve sheath tumors characterized by degenerative changes such as cystic necrosis, hyalinization, and calcification. These benign neoplasms often present diagnostic challenges due to their nonspecific clinical manifestations and potential for extensive local growth.  A 57-year-old male farmer presented with a 20-year history of progressive swelling in the right upper eyelid, which had enlarged to the size of a golf ball.

The lesion was associated with proptosis, inferior globe dystopia, exposure keratopathy, and complete vision loss in the affected eye. Systemic symptoms included intermittent headaches, low-grade fever, and weight loss. Magnetic resonance imaging revealed a large, heterogeneously enhancing mass arising from the superior aspect of the right orbit, with intracranial extension into the anterior cranial fossa and involvement of adjacent paranasal sinuses. Histopathological examination of the excised tumor demonstrated features consistent with an ancient schwannoma, and immunohistochemistry confirmed the diagnosis.

 This case highlights the indolent yet locally aggressive nature of intra-orbital ancient schwannomas, emphasizing the importance of early diagnosis and multidisciplinary management. Surgical excision remains the mainstay of treatment, with a favorable prognosis following complete resection. However, intracranial extension and prolonged symptom duration, as seen in this case, underscore the need for heightened clinical suspicion and timely intervention.

Conclusion: Intra-orbital ancient schwannomas are rare but should be considered in the differential diagnosis of orbital masses. Early imaging and histopathological confirmation are critical for optimal management and preservation of visual function.