Ganglioneuroma of the sphenoid wing: a case report and literature review

Abstract

Ganglioneuromas (GNs) are well-differentiated, slow-growing, benign tumors that are quite rare and usually found in the posterior mediastinum and retroperitoneum. They are composed of ganglion and Schwann cells and their origin remains in dispute. GNs have been reported as intraosseous lesions, such as in temporal and orbital bones. There are rare reports of intracranial lesions, mostly in the pituitary fossa. Most GN patients are children and are clinically asymptomatic. Diagnosis of GN requires histopathologic evaluation since no specific clinical or radiologic diagnostic features have been identified. We report the case of a 35-year-old man with recurrent sinusitis whose radiologic workup revealed a lytic right sphenoid wing lesion with microcalcifications. He underwent gross-total resection of the lesion and the pathologic findings were diagnostic of ganglioneuroma. To the best of our knowledge, this is the first reported case of sphenoid wing GN. The nature and origin of this tumor are discussed, and the GN literature is reviewed.

Figure 1.. A: Sagittal reformats of the axial non-contrast CT scan revealing the well-demarcated, oval lesion (asterisk) at the sphenoid bone. B: Post-contrast fat saturated axial T1-weight cranial MRI revealing enhancing well-demarcated lesion (asterisk) within the right sphenoid wing around the foramen rotundum.

Figure 2.. a: H&E stain at low (4 ×) magnification reveals an intraosseous tumor of moderate cellularity and boney specules, marked by asterisk. b: At higher magnification (20 x), randomly distributed large and dysplastic ganglion cells are seen in a background of neoplastic spindle cells with scant cytoplasm. c, d: H & E stains reveal irregular clusters of dysplastic ganglion cells with abnormal distribution of the chromophylic substance, binucleated cells (arrows) and infrequent satellite cells (arrowheads). e, f: At 10 × magnification, the neurofilament (directed against 70 kDa and 200 kDa polypeptides of non-phosphorylated neurofilament) (e) and at 20 × magnification, sections stained with silver (f) reveal bizarre and haphazardly oriented axons (arrowheads) as well as few large ganglion cells (arrows).