Abstract
Objective:
To review various small round blue cell tumors (SRBCTs) with anterior skull base invasion and examine surgical technique and survival.
Background
: SRBCTs are a rare and heterogenous group of poorly differentiated malignant neoplasms that arise from widely varied cells but are united by their common histological appearance of large nuclei with scant cytoplasm. Commonly arising from the nasal cavity and paranasal sinuses, these tumors have a propensity to invade the anterior skull base due to the close anatomic relationship between these structures.
Setting
: Academic, tertiary referral, comprehensive cancer center.
Design
: Retrospective, case series.
Methods
: Between the years of 2005 and 2012, all patients with SRBCT of the head and neck with radiographic evidence of anterior skull base invasion were reviewed.
Results
: The patient data series was stratified by histologic subgroup, and sociodemographic analysis was performed. Tumor subgroups were divided by treatment type (surgical, adjuvant, both). In patients who underwent surgery, the approach was divided into: open anterior craniofacial, endoscopic, and combined. Survival analysis was then performed.
Conclusion
: SRBCTs present a unique clinical entity with both diagnostic and treatment challenges. These tumors tend to have locally aggressive behavior with historically poor clinical outcomes. Multidisciplinary team effort with multimodality surgical approaches and treatment appear to have an increasing role in prolonging survival and the comprehensive management of this unique group of tumors.