Surgical Management of Pilocytic Astrocytoma of the Optic Nerve: A Case Report and Review of the Literature

Abstract

Optic nerve astrocytomas (ONAs) are frequent types of optic nerve gliomas (ONGs), which can affect the visual pathway. An 18-year-old male patient was admitted to our department with right-sided intraorbital/retrobulbar swelling, which progressively grew over several months. Clinical examination showed right-sided diplopia, mydriasis, low visual acuity (0.4), exophthalmus (3 cm), epiphora, and severe retrobulbar pain. There was a family history of high-grade (IV) astrocytomas in which two of the family members died due to the disease. Preoperative MRI scan revealed a soft tissue mass around the retrobulbar area of the right eye with intact orbital bony walls. Surgery was performed whereby it was dissected freely from the muscles and was separated from the optic nerve and the globe. Histopathologic analysis confirmed a benign astrocytoma. The follow-up examination revealed no recurrent or residual tumor. A systemic review of the literature indicates that early diagnosis and experienced multidisciplinary management are required in case of unilateral, resectable forms of ONAs with no distant metastasis, in order to provide a long-time survival of patients. Surgical intervention of unilateral ONAs is a relatively safe procedure, allowing complete or partial tumor removal with minimal morbidity and low recurrence rate.

An Atypical Case of Optic Neuropathy

“…WHO grade I pilocytic astrocytomas are slow growing, well circumscribed without invasion to surrounding tissue, and rarely require surgical intervention; in contrast, WHO grade IV pilocytic astrocytomas have more aggressive features with poorer outcomes . Previous tumor sequencing of anaplastic pilocytic astrocytomas has shown associations with BRAF mutation, p16 loss, and absence of p53 mutation . Radiotherapy is an option in this older patient; in children, adverse effects to the central nervous system may be prohibitive …”

“…Previous tumor sequencing of anaplastic pilocytic astrocytomas has shown associations with BRAF mutation, p16 loss, and absence of p53 mutation . Radiotherapy is an option in this older patient; in children, adverse effects to the central nervous system may be prohibitive …”

Imaging of supratentorial intraventricular masses in children: a pictorial review—part 2

“…There are no clinical features unique to PAs. The signs and symptoms, typically persisting for several months, directly correlate with the tumor size, location, and the presence of associated hydrocephalus [ 48 ].…”

Demographic and prognostic factors of optic nerve astrocytoma: a retrospective study of surveillance, epidemiology, and end results (SEER)

“…Of all these tumors, astrocytoma is the most common type [ 1 – 3 ]. ONAs are rare astrocytic tumors that occur in the optic nerve and reach out to the chiasm and the frontal lobe frequently [ 4 , 5 ]. The classification of ONAs is based upon the World Health Organization (WHO) criteria, and it include Grade I (pilocytic), Grade II (diffuse), Grade III (anaplastic), and Grade IV (glioblastoma) astrocytoma [ 6 ].…”