Case Report: Esthesioneuroblastoma Involving the Optic Pathways

Abstract

Olfactory neuroblastoma, or esthesioneuroblastoma, is an uncommon malignant tumor originating from the neural crest that commonly occurs in the upper nasal cavity. Its ectopic origin is extremely rare, especially when located in the optical pathways. This paper reports the case of a giant ectopic esthesioneuroblastoma of the optic pathways that were surgically treated through a cranio-orbital-zygomatic (COZ) craniotomy with extensive resection, in addition to a literature review. The patient is a 46-year-old female presenting with a 4-month history of visual loss in the left eye. Since she was previously blind in the right eye from a traumatic injury, it was evolving to loss of bilateral vision. Imaging depicted an expansive infiltrating lesion involving the entire path of the right optic nerve, extending to the optic chiasm, cisternal portion of the left optic nerve, bilateral optic tract, and hypothalamus. Investigation of pituitary function was unremarkable. Esthesioneuroblastoma is a rare tumor with poorly defined standard clinical management. Its ectopic presentation makes the diagnosis even more challenging, making it difficult to manage these cases properly. Surgeons should be aware of this rare possibility, as early aggressive treatment is likely to be associated with better results.

Keywords: cranio-orbito-zygomatic approach; ectopic tumor; esthesioneuroblastoma; olfactory neuroblastoma/esthesioneuroblastoma; skull base.

Figure 1

Preoperative post-contrast T1-weighted MRI (A,C) and postoperative images (B,D).

Figure 2

Olfactory neuroblastoma (Hyams III). Proliferation of cells forming lobe sketchs, separated by vascular and hylinized fibrous stroma. HE 10x (A). The cells are hyperchromatic, pleomorphic, and sometimes arranged in gland-like rings or tight annular formations with a true lumen (Flexner–Wintersteiner rosettes). Some mitotic figures can be seen. HE 20x (B). Immunohistochemical stains: Synaptophysin (C); Chromogranin (D); S100: positive in sustentacular periphery cells (E); Ki-67 (F).