Surgical treatment of glossopharyngeal nerve schwannomas: Results of two-center study (14 patients included) and literature review

Objective: This study is aimed to analyze the outcomes of surgical treatment of glossopharyngeal schwannomas based on pre- and postoperative neurological status assessment. Materials and methods: This paper is a retrospective analysis of examination and surgical treatment of 14 patients who were operated on in two large clinics from 2018 to 2021 inclusive. When analyzing the collected data, gender, age, disease symptoms, tumor size and location, surgical approach, tumor to cranial nerves (CN) ratio, jugular foramen (JF) condition, and tumor removal volume were taken into account. All tumors were divided into groups depending on tumor location relative to the JF. Particular attention was paid to assessing cranial nerves functions. Facial nerve function was assessed as per House-Brackmann Scale (HBS), hearing function as per Gardner-Robertson Scale (GRS). Results: 3 (21.4%) patients had total tumor removal: 2 patients had type A tumors and one had type B tumor. Subtotal resection took place in 7 (50%) cases. In 4 cases, a tumor was partially removed: 3 patients had type D tumors and one had type B tumor. 3 (21.4%) patients had preoperative FN deficit (HBS Grade II) and mild dysfunction. 5 (35.7%) patients had postoperative facial nerve deficit: HBS ІІ, 2; ІІІ, 1; V, 2. Preoperative sensorineural type hearing impairment on the affected side was diagnosed in 13 (92.6%) patients. Before surgery, 6 patients had non-serviceable hearing, which remained at the same level after surgery. None of the patients with grade I or II hearing before surgery had any hearing impairment postoperatively. In 2 (14.3%) cases, hearing improved from grade V to grade III after surgery. 6 (42.9%) patients developed new neurological deficit in the caudal group CN. Postoperative deficit of the caudal group CN occurred in type D tumors in 3 patients, type A tumors 2 patients, and type B tumors one patient. Conclusions: Applying a retrosigmoid approach only makes it possible to achieve total tumor removal in case of type A tumors. To remove other tumor types, it is necessary to select approaches that enable access to the jugular foramen and infratemporal fossa. Intraoperative neurophysiological monitoring is an extremely important tool in glossopharyngeal schwannoma surgery. The most common postoperative complication is a developed or increased deficit of the caudal CN group, which can lead to persistent impairments in the patients’ quality of life. Preservation of the CN VII and VIII function in most cases is a feasible task and shall be ensured as a standard for this pathology.

Treatment strategy for multisegmental cervicomedullary ependymoma: illustrative case

BACKGROUND Сervicomedullary ependymoma (CME) is a rare tumor of the central nervous system. The CME treatment strategy is insufficiently represented in the literature and is a complex task for neurosurgeons. OBSERVATIONS The authors describe an infrequent case of a large multisegmental CME that extended from the medulla oblongata to the cervical spinal cord at the level of the sixth cervical vertebra in a 21-year-old female. Neurological disorders presented with headache, dysphagia, hypophonia, and weakness in the limbs. Subtotal removal of the tumor was performed according to intraoperative neurophysiological monitoring (IONM) results. A wait-and-see approach with patient follow-up was chosen. LESSONS Total tumor removal of the CME is the most important favorable prognostic factor. Subtotal resection can be considered if the borders of the tumor are unclear and the result of IONM is unfavorable. The role of postoperative radiation therapy in the case of subtotal removal of the tumor remains controversial.

Photothermal Therapy as Adjuvant to Surgery in an Orthotopic Mouse Model of Human Fibrosarcoma

Surgery is still the first-line treatment for multiple solid cancers. However, recurrence is a common issue, especially when dealing with aggressive tumors or tumors that are difficult to completely remove due to their location. Getting clear surgical margins can be challenging, but treatment strategies combining surgery with other anti-cancer therapies can potentially improve the outcome. Photothermal therapy (PTT) is a technique that relies on photoabsorbing agents, such as gold nanoparticles, to transform light into local hyperthermia. This technique can be used to ablate tumor tissue where the photoabsorbing agent accumulates, sparing healthy surrounding tissue. In this study, we examined the potential of gold nanoparticle-based PTT as an adjuvant treatment to surgery in a mouse model of human fibrosarcoma. For this we performed subtotal tumor resection to mimic a clinical situation where total tumor removal is not achieved, and subsequent PTT was applied on the surgical field. Our results showed that animals undergoing adjuvant PTT after surgery presented sustained delayed tumor growth and improved survival when compared to animals that only underwent surgery. We believe that these findings show the potential of PTT as an adjuvant method to traditional tumor surgery and could pave way to more personalized treatment options.

Efficacy of the Suboccipital Paracondylar-Lateral Cervical Approach: The Series of 64 Jugular Foramen Tumors Along With Follow-Up Data

ObjectiveComplete resection of jugular foramen tumors with minimal cranial nerve complications remains challenging even for skilled neurosurgeons. Here, we introduce a modified paracondylar approach, named the suboccipital paracondylar-lateral cervical (SPCLC) approach for this purpose. We also share the follow-up data of our series and discuss the advantages and limitations of this modified paracondylar approach.MethodsWe included 64 patients with jugular foramen tumors who underwent surgery by the same senior neurosurgeon between November 2011 and August 2020. All patients were treated with the SPCLC approach, which aimed for gross total tumor removal in a single-stage operation. The clinical characteristics, including preoperative and postoperative neurological status, the extent of surgical resection, and follow-up data were retrospectively acquired and evaluated.ResultsThere were 48 schwannomas, nine meningiomas, three paragangliomas, one hemangiopericytoma, one chordoma, one endolymphatic sac tumor, and one Langerhans’ cell histiocytosis. The median age of our patients was 43 years (range: 21–77 years). Dysphagia, hoarseness, and tongue deviation were observed in 36, 26, and 28 patients, respectively. Thirty-two patients had hearing function impairments, including hearing loss or tinnitus. Gross total resection was achieved in 59 patients (59/64, 92.2%). Gamma Knife treatment was used to manage residual tumors in five patients. Postoperatively, new-onset or aggravative dysphagia and hoarseness occurred in 26 and 18 cases, respectively. Nine patients developed new-onset facial palsy, and one patient developed new-onset hearing loss. There were no cases of intracranial hematoma, re-operation, tracheostomy, or death. At the latest follow-up, hearing loss and tinnitus had improved in 20 cases (20/32, 62.5%), dysphagia alleviated in 20 cases (20/36, 55.6%), and hoarseness improved in 14 cases (14/26, 53.8%). Over a mean follow-up period of 27.8 ± 19.5 months (range: 3–68 months), tumor recurrence was observed in one patient.ConclusionThe SPCLC approach, modified from the paracondylar approach, and was less invasive, safe, and efficient for certain jugular foramen tumors. Taking advantage of the anatomic understanding, clear operational vision, and appropriate surgical skills, it is possible to achieve gross total tumor removal and the preservation of neurological function.

Endoscopic-assisted microsurgical resection of giant vestibular schwannoma in semi-sitting position

The main objectives of microsurgery for vestibular schwannoma are total tumor removal and preservation of facial and cochlear nerve function. For giant tumors, total tumor removal and facial nerve function preservation are challenging. The semisitting position has some advantages. In this video the authors show the removal of a giant vestibular schwannoma with the patient in a semisitting position. They demonstrate the advantages of the semisitting technique, such as the two-handed microsurgical dissection technique and a clear operative field. Finally, a small residual tumor in the internal auditory canal was removed by endoscopy. The patient’s facial function was House-Brackmann grade I at discharge. The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID2176

Midterm prognosis and surgical implication for clival chordomas after extended transsphenoidal tumor removal and gamma knife radiosurgery

Background Treating chordoma through surgery alone is often ineffective. Thus, surgery often performed with irradiation, with a reported 5-year survival rate of 60–75%. The clinical course varies, and disease rarity prevents larger number of clinical investigations. Methods In total, 19 patients with clival chordomas were retrospectively extracted from our institutional database. They were initially treated with maximal tumor removal using the extended transsphenoidal approach between March 2006 and January 2021. When total tumor removal was achieved, prophylactic irradiation was not performed. If tumor remnants or recurrence were confirmed, Gamma Knife (GK) radiosurgery was performed. The mean follow-up period was 106.7 months (ranged 27–224 months). The clinical course and prognostic factors were investigated. Results Total removal was achieved in 10 patients, whereas 4 patients suffered recurrence and required GK. GK was applied to 11 patients with a 50% isodose of 13–18 Gy (mean: 15.4 Gy), and eight patients remained progression free, whereas three patients suffered repeated local recurrence and died of tumor-related complications. The mean overall progression-free interval was 57.2 months (range: 6–169 months). One male patient died of tumor un-related lung cancer 36 months after the initial treatment, and other patients survived throughout the observational periods. The mean overall survival was 106.7 months (range: 27–224 months). Thus, the 5-year survival rate was 94.7%. Statistical analysis indicated that sex (men), > 15 Gy of 50% isodose by GK, and screening brain examinations as prophylactic medicine were significant favorable prognostic factors. Conclusions The favorable outcomes in this investigation suggest the importance of early detection and treatment. Surgery may enable better conditions for sufficient GK doses.

Transcavernous Resection of an Upper Clival Chondrosarcoma: “Cavernous Sinus as a Route”: 2-Dimensional Operative Video

Complete resection of skull base chondrosarcomas offers the potential for a durable, or even lifelong, cure and is best achieved at the first surgery.1,2 When a skull base chondrosarcoma is located at the upper clivus and in the interpeduncular cistern and invading laterally toward the petrous apex and cavernous sinus, the traditional approaches, ie, endonasal endoscopic or middle fossa approaches, are not adequate for the exposure and resection. The transcavernous sinus approach has been utilized to remove tumor from the cavernous sinus and as a corridor to the interpeduncular cistern and upper clivus, originally described for the clipping of basilar apex aneurysms. We present a case of a chondrosarcoma centered in the upper clivus and eroding the right posterior clinoid, analogous to the location of a giant basilar apex aneurysm. Detailed study of the tumor extension, bony invasion, and relationship with neuroanatomy dictated the most effective surgical approach.3,4 Neuronavigation and intraoperative magnetic resonance imaging (MRI) facilitated the gross total resection of the tumor in the Advanced Multimodality Image-Guided Operating (AMIGO) suite. Achieving a gross total tumor removal of this World Health Organization (WHO) grade I chondrosarcoma, adjuvant irradiation can be withheld1 and the patient monitored with serial imaging. The patient did well after the surgery. The patient consented for surgery and the use of photography.

Posterior approach to ventrally and dorsally located spinal meningiomas

Objective. To analyze the outcomes of posterior approach in the surgery of intradural extramedullary meningiomas located ventrally and dorsally in relation to the spinal cord denticulate ligaments.Material and Methods. The study included 29 patients with spinal intradural meningiomas operated on using posterior approach. Patients were divided depending on the tumor location relative to the denticulate ligaments into ventral (n = 13) and dorsal (n = 16) groups. The surgery duration, the degree of tumor resection, clinical outcomes, the presence and nature of complications, and the frequency of recurrence were assessed.Results. The average follow-up period was 29 (6 to 61) months. Total tumor removal was performed in 93.1 % of cases: 11 cases (84.6 %) in ventral group and 16 cases (100.0 %) in dorsal group. The average duration of surgery was 136 minutes for dorsal meningiomas and 181 minutes for ventral meningiomas (p < 0.05). Complications in the form of CSF leakage were registered in two patients (6.9 %). In 11 (84.6 %) patients with ventral meningiomas and 15 (93.7 %) patients with dorsal meningiomas, an improvement or preservation of neurological functions at the pre-surgery level was observed. Recurrences were observed in two patients (6.9 %).Conclusion. Patients with spinal meningiomas have a favorable neurological outcome and a low recurrence rate. Surgery is more complicated in patients with ventral meningiomas. In most cases, unilateral posterior approach is applicable for both ventral and dorsal meningiomas.

RARE-38. CLINICAL PRESENTATION OF MGA-NUTM1 FUSION TRANSCRIPT SARCOMA

BACKGROUND MGA-NUTM1 fusion gene tumor are recently described as new subtype of NUTM1-rearranged tumors. Regarding its rarity, standard treatment has not been reported. Here we described clinical presentation, radiologic finding, immunohistological profile, and treatment of a boy with MGA-NUTM1 fusion gene tumor. CASE REPORT: A 13-year-old boy with 2-month history of progressive right hemiparesis and headache. Magnetic resonance imaging (MRI) revealed 7.8 x10.6 x 8.0 cm well defined heterogeneous enhancing mass at left fronto-parietal lobe. CT chest and abdomen, bone scan, MRI spine, and CSF studies were unremarkable. He underwent craniotomy with total tumor removal. Pathology demonstrated high grade spindle cell sarcoma. The immunohistological profile was positive for BCOR, NUT1, and TEL1, but negative for CD34, STAT6, desmin, SMA, actin sarcomeric, EMA, PR, S100, SOX10, BCL 6, and SABT2. The INI-1 showed nuclear expression and Ki-67 was positive in 50% of tumor nuclei. Molecular test for MGA-NUTM1 fusion transcript was positive, while SYT-SSX1, SYT-SSX2, and SYT-SSX4 fusion transcripts were negative. Four months after operation, MRI showed newly-seen two small enhancing foci at lateral and inferior aspects of the surgical cavity. He underwent re-surgery. Then focal radiation (54Gy and boost up to 60Gy at recurrent area) to the resection cavity was decided. Post-radiation chemotherapy including ifosfamide 3 g/m2 and etoposide 150 mg/m2 on Day 1–2, and carboplatin 500 mg/m2 on Day 3, every 21–28 days was started. He has completed the first course of chemotherapy without any complication. CONCLUSION MGA-NUTM1 fusion CNS sarcoma is rare. Treatment may require surgery, radiation and chemotherapy.