Granular Cell Tumor of the Sphenoid: A Unique Finding in a Pediatric Patient with Sickle Cell Disease and Jeavons' Syndrome

Objective: Granular cell tumors (GCT) of the sinonasal region are exceedingly rare, with only five occurrences having been described in the literature. We recount the case of a 12-year-old female with sickle cell disease and Jeavons' syndrome diagnosed with a GCT of the sphenoid sinus, a location not previously reported.

Methods: One patient with pathologically diagnosed GCT was identified at our institution and followed by the pediatric otolaryngology team.

Results: This 12-year-old female was noted to have an expansile bony lesion involving the right sphenoid bone on imaging performed after an episode of status epilepticus secondary to Jeavons' syndrome. Her only symptoms were occasional headaches, mouth-breathing and snoring. CT demonstrated an osseous expansion in the right sphenoid bone with extension to the sella turcica. Looking back at head imaging from three years prior, the lesion was retrospectively identified and noted to have slowly increased in size since that time. Initial biopsy revealed chronic and eosinophilic sinusitis but was nondiagnostic for tumor. She was followed closely for several months and repeat imaging studies showed qualitative change of the internal composition of the mass without dramatic change in size. Repeat sphenoid biopsy with subtotal resection of the lesion was diagnostic for granular cell tumor of the sphenoid bone.

Conclusion: While the majority of granular cell tumors are benign, they can be locally destructive and are optimally treated with surgical excision. Due to the challenging location of this sphenoidal GCT at the anterior and middle cranial base with unclear borders, the patient has not undergone total excision. There is some evidence that these tumors respond to radiation therapy, but the proximity to the optic nerve poses a challenge. Serial imaging has acknowledged the slow-growing, indolent nature of this tumor, and the patient remains asymptomatic.

Fig. 1 Noncontrast CT head, coronal view: osseous expansion involving body of right sphenoid bone with extension to pterygoid process and sella turcica, 2.1 cm × 3.5 cm × 3 cm. CT, computed tomography.

Fig. 2 Noncontrast CT head, sagittal view: osseous expansion involving right sphenoid bone with extension to sella turcica and optic canal, 2.1 cm × 3.5 cm × 3 cm. CT, computed tomography.

Fig. 3 Chronically inflamed respiratory mucosa with infiltrate of cells with polygonal granular cytoplasm with small oval to renal form nuclei.

Publication History

Article published online:
12 February 2021