Anterior Skull Base Sarcomas: Report on Characteristics and Outcomes

Introduction: Sarcomas of the head and neck region are uncommon malignancies, accounting for ∼1% of all head and neck cancers. The aim of this study is to describe our experience on characteristics and treatment outcomes of skull base sarcomas.

Methods: Thirty-one patients with sarcomas of the anterior skull base treated with primary surgery presenting from 1979 to 2015 at a single institution were retrospectively reviewed. Patient, tumor, and treatment characteristics were recorded. Survival and recurrence outcomes were determined using the Kaplan–Meier method.

Results: The median age was 44 years (13–69). Eleven patients were female (35%). The majority of patients presented with clinical T4 tumors (28, 91%). Seven patients had a previous history of retinoblastoma, one patient had neurofibromatosis type 1, and one patient had a history of fibrous dysplasia. All patients underwent open anterior craniofacial resection apart from two who had endoscopic resection. The most frequent pathologies were leiomyosarcoma (19%), osteosarcoma (16%), chondrosarcoma (10%), and myxofibrosarcoma (10%).

Positive or close margins were present in 14 patients (45%), and 18 (58%) had high-grade tumors. Twenty-three patients (74%) received adjuvant radiation and four patients (13%) received adjuvant chemotherapy. There were nine postoperative complications (29%) including one mortality and three cerebrospinal fluid leaks. With a median follow-up of 74 months (range: 1–310), the 5-year overall survival (OS) and disease-specific survival (DSS) were 72 and 70%, respectively. There were 10 local, two regional, and five distant recurrences. The 5-year local (LRFS), regional (RRFS), and distant (DRFS) recurrence free survivals were 70, 91, and 72%, respectively. Patients with positive and negative margins had a 5-year DSS of 60 and 64%, respectively. Patients with high- and low-grade tumors had a 5-year DSS of 74 and 86%, respectively.

Discussion: Skull base sarcomas are rare. Disease-specific survival is ∼70% at 5 years. Due to the rarity of these tumors, management should be determined by a multidisciplinary team (Fig. 1).

Conclusion: This study contributes to an updated analysis of sarcomas of the head and neck. The accumulation of only 31 cases in such a large time period highlights the rarity of these tumors.

Fig. 1 Disease-specific survival rates, all cases.