Abstract
Object: Skull base meningiomas with extracranial extensions are rarely described. This study describes the clinical features, surgical management and clinical outcomes of these rare tumors and investigates risk factors associated with progression-free survival (PFS).Methods: The clinical data of 34 consecutive patients who underwent surgery for skull base meningiomas with extracranial extensions from 2007 to 2018 were retrospectively collected and analyzed.Results: The mean patient age was 47.9 ± 13.9 years; 50.0% were male. The most common symptoms on admission were ophthalmic. All patients underwent a multidisciplinary consultation before surgery, and received individualized surgical management. The gross total resection (GTR) rate was 55.9% (19/34). Twelve patients received post-operative adjuvant radiotherapy (RT). Twelve patients experienced tumor recurrence during the follow-up period. The median PFS duration was 54 months. The mean overall survival (OS) duration was 111 months. By univariate analysis, a higher histological grade (WHO grade II and III), Ki-67 LI ≥ 5 and the extent of resection (EOR) were significantly associated with tumor recurrence. Multivariate analysis revealed Ki-67 LI ≥ 5, the EOR and adjuvant RT as prognostic factor of PFS.Conclusions: These relatively rare meningiomas are difficult to resect and have a poor prognosis; they are more common in males and have a higher histological grade than intracranial meningiomas. Multidisciplinary collaboration and individualized surgical strategies are crucial for surgically managing these complex tumors. Total removal of the tumor remains challenging. Subtotal resection (STR) or partial resection (PR) followed by RT is a reasonable strategy when radical resection is infeasible. Adjuvant RT should be recommended especially for tumors with histopathological risk factors (Ki-67 LI ≥ 5 or high histological grade).
Highlights
Intracranial meningiomas are extracerebral, slow-growing, welldefined tumors that account for 13–26% of all primary intracranial neoplasms, and ∼25% of meningiomas arise in the cranial base [1]
Thirty-four cases of pathologically confirmed skull base meningioma with extracranial extensions were identified among 271 cases of surgically treated skull base meningiomas in our center in the study period
We have summarized eight approaches applicable for the treatment of these special entities, as outlined below. (a) The Derome approach (Figure 1) is usually used for tumors of anterior cranial fossa extending into the nasal cavity and paranasal sinus
Summary
Intracranial meningiomas are extracerebral, slow-growing, welldefined tumors that account for 13–26% of all primary intracranial neoplasms, and ∼25% of meningiomas arise in the cranial base [1]. Skull base meningiomas with extracranial extensions are a relatively rare clinical entity which extend into craniofacial structures [3]. These complex tumors involve both intracranial and extracranial structures, such as the anterior or middle cranial fossa and the infratemporal fossa, nasal cavity, paranasal sinuses, orbits or neck, etc., so multidisciplinary cooperation and individualized surgical strategies are required [4]. Presenting symptoms Headache Ophthalmic symptoms Mass on face or neck Nasal obstruction or discharge Cranial nerve disorders Initial or recurrent lesion Initial Recurrent Extracranial extensions Orbit Nasal cavity or paranasal sinus Infratemporal or pterygopalatine fossa Neck or parapharyngeal space Enhancement.
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