Spheno-Orbital Meningioma with Cavernous Sinus Extension

We present a case of a 37-year-old female, with progressive left eye proptosis and an extensive ipsilateral en plaque spheno-orbital meningioma, with diffuse involvement of the lateral wall of the cavernous sinus and the orbit.A cranio-orbital zygomatic approach was performed to reach all extension of the lesion. We made an extradural clinoidectomy and an extensive bone removal of the orbit, exposition of the superior and lateral walls of the cavernous sinus, unroofing of the optic canal, superior orbital fissure, foramen rotundum, and foramen ovale. We performed the peeling of the lateral wall of cavernous sinus with total removal and preservation of the cranial nerves anatomy and function.In this video, we illustrate the importance of the first time aggressive removal of such tumors. We also demonstrate the concept that secondary invasion of the cavernous sinus are suitable for removal with cranial nerve preservation.The link to the video can be found at https://youtu.be/GJmkqVa6jSs.

Hypofractionated Robotic Stereotactic Radiosurgery for Vagal Paragangliomas: A Novel Treatment Strategy for Cranial Nerve Preservation

Objective To provide the first description of hypofractionated stereotactic radiosurgery (SRS) and evaluate tumor control and safety for vagal paragangliomas (VPs), which begin at the skull base but often have significant extracranial extension. Study Design Retrospective chart review. Setting Tertiary-referral neurotology and neurosurgery practice. Subjects and Methods Five VPs in 4 patients (all male, ages 15-56 years) underwent SRS between 2010 and 2018. Outcome measures included tumor dimensions on serial imaging, cranial nerve function, and radiation side effects. Results CyberKnife hypofractionated SRS was performed. The prescription dose was 24 or 27 Gy (maximum dose 33.4 Gy; range, 29.3-35.5 Gy) delivered in 3 equal fractions. The mean isodose line was 79% (range, 76%-82%). Four VPs were treated primarily, and 1 tumor underwent SRS to treat regrowth 2 years after microsurgical subtotal resection via the modified infratemporal fossa approach. The treatment volume ranged from 8.81 to 86.3 cm3 (mean, 35.7 cm3). All demonstrated stable size (n = 3) or regression (n = 2) at last follow-up, 63 to 85 months after SRS (mean, 76 months). One patient had stable premorbid vocal fold paralysis from a prior ipsilateral glomus jugulare tumor resection. All others demonstrated normal vagal function following SRS. Treatment-related side effects, including dysgeusia (n = 1), mucositis (n = 1), and neck soft-tissue edema (n = 2), were self-limited. Conclusions Hypofractionated SRS appears to be both safe and effective for treating VPs, including large-volume and predominantly extracranial tumors, while preserving vagal function. SRS should be considered as a cranial nerve preservation option, especially in settings of contralateral lower cranial nerve deficits or in those with multiple paragangliomas risking both vagal nerves.

Stereotactic irradiation for treatment of acoustic neuromas

Stereotactic cerebral irradiation is a radiotherapeutic technique for precise delivery of ionizing irradiation to an intracranial target with simultaneous sparing of adjacent parenchyma. Treatment can be administered by a multi-source cobalt-60 device (gamma knife) or a dedicated linear accelerator.The target dose can be delivered in a large, single fraction (stereotactic radiosurgery SRS) or in multiple, daily increments (stereotactic radiotherapy SRT). The latter approach combines the physical dose localization advantages of SRS with the radiobiologic benefits of dose fractionation. This literature review will present the rationale and clinical results of SRS and SRT for the treatment of acoustic neuromas. Emphasis will be placed on tumor control, imaging outcome and cranial nerve preservation. The findings demonstrate that SRS and SRT represent reasonable therapeutic options for patients with acoustic neuromas.

Microsurgical Management of Jugular Foramen Schwannomas

BACKGROUND: Jugular foramen schwannomas are uncommon and surgically challenging lesions. OBJECTIVE: To determine the importance of surgical technique on morbidity and recurrence of jugular foramen schwannomas. METHODS: A retrospective review and case-control analysis of a single-senior-surgeon series of 81 patients with surgically treated jugular foramen schwannomas was performed, focusing on operative technique. Patients undergoing an aggressive, total tumor resection (series 1) were compared with those undergoing more conservative resection focusing on preserving the pars nervosa (series 2). RESULTS: There was a statistically significant (P = .04) decrease in permanent deficits of the cranial nerve 9/10 complex with a conservative technique. Recurrence was seen in 3 patients (5.7%) in series 1 and in 3 patients (10.7%) in series 2 (P = .36). Recurrence was treated with reoperation in 1 patient, radiation in 1 patient, and observation in the others. CONCLUSION: Although radical gross total resection is desirable, it is not optimal for cranial nerve preservation in patients with jugular foramen schwannomas. A more conservative approach resulted in a statistically significant decrease in lower cranial nerve deficits. There was a nonstatistically significant trend toward increasing recurrence, which may be treated with multiple modality therapy in the modern era.

Neuroendocrine Carcinoma of the Jugular Foramen

We describe what might have been the first reported case of a neuroendocrine carcinoma of the jugular foramen. A 50-year-old woman presented with progressive left-sided sensorineural hearing loss, vertigo, pulsatile tinnitus, headaches, and ataxia. Magnetic resonance imaging revealed a 4-cm left-sided jugular foramen tumor. The patient underwent near-total resection of the tumor. Despite lower cranial nerve preservation, postoperative paralysis of cranial nerves IX and X occurred, and vocal fold medialization was performed 5 days later. The final pathologic diagnosis was neuroendocrine carcinoma. The patient was treated with concurrent chemotherapy and intensity-modulated radiation therapy. This article will discuss the pathologic features and the management of jugular foramen tumors, along with the differential diagnosis of these rare tumors.

Cranial Nerve Preservation and Outcomes after Stereotactic Radiosurgery for Jugular Foramen Schwannomas

OBJECTIVE Jugular foramen region schwannomas are rare intracranial tumors that usually present with multiple lower cranial nerve deficits. For some patients, complete surgical resection is possible but may be associated with significant morbidity. Stereotactic radiosurgery is a minimally invasive alternative or adjunct to microsurgery for such tumors. We reviewed our clinical and imaging outcomes after patients underwent gamma knife radiosurgery for management of jugular foramen schwannomas. METHODS Thirty-four patients with 35 tumors (one patient had bilateral tumors) underwent radiosurgery between May 1990 and December 2005. Twenty-two patients had previous microsurgical resection and all patients experienced various cranial neuropathies. A median of six isocenters were used. Median marginal and maximum doses were 14 and 28 Gy, respectively. RESULTS None of the patients were lost to evaluation and the mean duration of follow-up was 83 months. Tumors regressed in 17 patients, remained stable in 16, and progressed in two. Five- and 10-year actuarial control rates were 97 and 94%, respectively. Preexisting cranial neuropathies improved in 20% and remained stable in 77% after radiosurgery. One patient worsened. The function of all previous intact nerves was preserved after radiosurgery. CONCLUSION Stereotactic radiosurgery proved to be a safe and effective management for newly diagnosed or residual jugular foramen schwannomas. Long-term tumor control rates and stability or improvement in cranial nerve function was confirmed.

Quality of life following acoustic neuroma surgery

In the treatment of acoustic neuroma, operative results have improved greatly during recent years, with high rates of functional cranial nerve preservation. Because of this, it has become more important to consider issues of patient satisfaction and quality of life (QOL) following treatment for these lesions. The authors have developed a novel questionnaire designed to measure QOL in patients with acoustic neuromas, and they administered it to 50 consecutive patients at least 6 months after acoustic neuroma surgery. Overall QOL was judged to be good but with definite minor difficulties, including some problems with hearing, facial nerve function, headache, tinnitus, dizziness, activity level, enjoyment of life, and emotional well-being. No significant differences were found between age groups and different operative approaches, and only minor differences were found in relation to tumor size. Patients with intracanalicular tumors fared no better than those with cerebellopontine angle tumors. Analysis of the data suggests an overall good outcome from acoustic neuroma surgery; however, when discussing the possible effects on postoperative QOL, even the potential minor problems should not be minimized, especially in patients undergoing operation for small or intracanalicular tumors.