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Primary Intraosseous Granular Cell Tumor of the Sphenoid and Central Skull Base in a Pediatric Patient
Delaney Sheehan1, Belinda Mantle2, Ashley Kraft3
1Department of Otolaryngology, University of AL at Birmingham, Birmingham, AL, USA.
Insights
A rare bone tumor, granular cell tumor (GCT), was found in a child's skull base. This case highlights GCTs in pediatric intra-osseous sphenoid and skull base locations.
Area of Science:
- Pediatric Oncology
- Neurosurgery
- Head and Neck Pathology
Background:
- Granular cell tumors (GCTs) are uncommon neoplasms that can occur in various body sites, typically affecting soft tissues.
- Primary bone involvement of GCTs, especially in the craniofacial region, is exceedingly rare.
Observation:
- A 12-year-old female with a history of sickle cell disease and Jeavons syndrome presented with seizures.
- Imaging and subsequent partial resection identified an expansile, benign granular cell tumor (GCT) within the sphenoid bone and central skull base.
- The tumor involved the sphenoid body and pterygoid process.
Findings:
- This represents the first reported case of a primary intra-osseous granular cell tumor of the sphenoid and central skull base in a pediatric patient.
- The GCT was benign and showed stable disease on imaging after a 36-month follow-up period.
- While generally benign, GCTs have a potential for aggressive behavior and malignant transformation.
Implications:
- This case expands the known anatomical distribution of GCTs, particularly in pediatric intra-osseous locations.
- Management of skull base tumors requires careful consideration of surgical limitations due to critical adjacent structures.
- Histological findings and clinical behavior are crucial for guiding treatment decisions in GCT cases.
Background:
Granular cell tumors occur in all ages and many anatomic sites. In the craniofacial region, they typically arise in soft tissue, not bone. We present a primary intra-osseous granular cell tumor of the sphenoid and central skull base arising in a 12- year- old girl.
Case Report:
A 12-year-old female with sickle cell disease and Jeavons syndrome presented with seizures. Imaging and partial resection revealed an expansile benign granular cell tumor (GCT) involving the sphenoid body, pterygoid process, and central skull base. The disease has remained stable after 36-month follow up.
Discussion:
GCT primarily involving the osseous sphenoid/skull base has not been previously reported in a child. Although mostly benign, some are aggressive, with malignant transformation in 1-2%. Surgery is the mainstay of treatment, but in the skull base this may be limited by adjacent critical structures. Decision-making is guided by anatomic extent, histology, and clinical behavior.
