Extraconal orbital craniopharyngioma

Area of Science:

• Ophthalmology
• Neurosurgery
• Pathology

Background:

• Orbital tumors can present with varied symptoms, including pain and swelling.
• Ectopic craniopharyngioma (CP) in the orbit is exceptionally rare, with limited documented cases.
• Understanding the pathogenesis of ectopic CP is crucial for diagnosis and treatment.

Purpose of the Study:

• To report a rare case of ectopic orbital craniopharyngioma (CP) in a female patient.
• To discuss the surgical management and histopathological diagnosis of this rare orbital tumor.
• To review and elucidate the proposed theories on the pathogenesis of ectopic CP.

Main Methods:

• Case presentation of a female patient with a 3-year history of right eye pain and swelling.
• Radiological evaluation revealing an extraconal solid-cystic orbital tumor.
• Surgical excision via supraorbital craniotomy with intraoperative squash smear and frozen section analysis, followed by definitive paraffin section and immunohistochemistry.

Main Results:

• Radiology suggested an epidermoid cyst, but intraoperative findings and subsequent histopathology confirmed craniopharyngioma (CP).
• Gross total excision of the orbital tumor was achieved.
• The patient's case represents one of the few documented instances of ectopic orbital CP.

Conclusions:

• Ectopic orbital craniopharyngioma is a rare entity requiring high clinical suspicion.
• Multimodality diagnostic approaches, including advanced imaging and histopathology, are essential.
• This case contributes to the understanding of rare orbital pathologies and the pathogenesis of ectopic CP.