Facial Schwannoma Management Outcomes: A Systematic Review of the Literature.

OBJECTIVE: To evaluate facial nerve outcomes of various management strategies for facial schwannomas by assimilating individualized patient data from the literature to address controversies in management. DATA SOURCES: PubMed-National Center for Biotechnology Information and Scopus databases. REVIEW METHODS: A systematic review of the literature was performed for studies regarding facial schwannomas. Studies were included if they presented patient-level data, type of intervention, pre- and postintervention House-Brackmann (HB) grades, and tumor location by facial nerve segment. RESULTS: Individualized data from 487 patients were collected from 31 studies. Eighty (16.4%) facial schwannomas were managed with observation, 25 (5.1%) with surgical decompression, 20 (4.1%) with stereotactic radiosurgery, 225 (46.2%) with total resection, and 137 (28.1%) with subtotal resection/stripping surgery. Stripping surgery/subtotal resection with good preoperative facial nerve function maintained HB grade 1 or 2 in 96% of cases. With a total resection of intradural tumors, preoperative HB grade did not significantly affect facial nerve outcome (n = 45, P = .46). However, a lower preoperative HB grade was associated with a better facial nerve outcome with intratemporal tumors (n = 56, P = .009). When stereotactic radiosurgery was performed, 40% of patients had improved, 35% were stable, and 25% had worsened facial function. Facial nerve decompression rarely affected short-term facial nerve status. CONCLUSION: The data from this study help delineate which treatment strategies are best in which clinical scenarios. The findings can be used to develop a more definitive management algorithm for this complicated pathology.

Clinical and Radiological Follow-up of Intraneural Perineuriomas.

BACKGROUND: Management of intraneural perineuriomas remains controversial, largely due to the lack of knowledge regarding the natural history of these lesions. OBJECTIVE: To describe the typical radiological growth pattern of intraneural perineuriomas and to determine how the pattern of growth relates to clinical progression. METHODS: We performed a retrospective review of the magnetic resonance imaging (MRI) studies and serial clinical examinations of a cohort of patients with biopsy-proven intraneural perineuriomas who had 2 MRI studies at least 2 yr apart. The outcome of interest was radiological growth in length or width of the intraneural perineurioma. Radiological growth was tested for association with clinical progression. RESULTS: Twenty patients were included in the study. By width, the lesions were on average larger on repeat imaging (P = .009). By absolute length, the lesions were on average longer on repeat imaging (P = .02). By lesion:landmark ratio, there was no difference in length of the lesions between sequential images (P = .09), with 10 (50%) lesions being shorter and 7 (35%) showing no change. No lesions grew to involve a new nerve or division of a nerve on sequential imaging. None of the variables tested were associated with clinical progression. CONCLUSION: We found that intraneural perineuriomas only rarely grow in length, do not grow to involve new nerves or nerve divisions, and growth does not correlate with clinical progression. These findings have significant ramifications for management of these tumors.

Quality of life related to symptomatic outcomes in patients with vestibular schwannomas: A Canadian Centre perspective.

Patients with vestibular schwannomas (VS) typically present with hearing loss and tinnitus as well as variable cranial nerve dysfunctions. Surgical resection, stereotactic radiotherapy and/or conservative management employing serial magnetic resonance or computed tomography imaging serve as the main treatment options. Quality of life (QoL) may be impacted by the extent of tumour burden and exacerbated or relieved by treatment. Subjective assessment and quality of life inventories provide valuable information in client centered approaches with important implications for treatment. The intention of QoL measurements affecting VS patients within a clinical setting is to facilitate discussions regarding treatment options and objectively evaluate patient- centered clinical outcomes in a naturalistic setting.

[Occipital condyle syndrome as the first symptom of a metastatic hepatocellular carcinoma. Two case reports]. / Sindrome del condilo occipital como primera manifestacion de un carcinoma hepatocelular metastasico. Presentacion de dos casos.

INTRODUCTION: Occipital condyle syndrome consists of the presence of unilateral occipital headache exacerbated by moving the head and is accompanied by paralysis of the ipsilateral hypoglossal nerve. One of its causes is infiltration of the base of the skull by bone metastases, especially those affecting the hypoglossal nerve due to infiltration as it passes through the osseous canal. CASE REPORTS: We report two clinical cases of occipital condyle syndrome secondary to metastatic hepatocarcinoma. The first is that of a 52-year-old male with liver cirrhosis secondary to liver pathology caused by hepatitis C virus with occipital condyle syndrome as the presenting symptom in disseminated hepatocarcinoma. The second case is that of a 56-year-old male after recurrence of hepatocarcinoma following a liver transplant, despite not fulfilling the Milan criteria. CONCLUSION: Occipital condyle syndrome is an alarm symptom and requires a thorough study by means of imaging tests, since it may be the first symptom of an undetected hepatocarcinoma.

TITLE: Sindrome del condilo occipital como primera manifestacion de un carcinoma hepatocelular metastasico. Presentacion de dos casos.Introduccion. El sindrome del condilo occipital consiste en la presencia de cefalea occipital unilateral que empeora con los movimientos cefalicos y se acompaña de paralisis del XII par ipsilateral. La infiltracion de la base del craneo por metastasis oseas se encuentra entre sus etiologias, especialmente las que afectan por infiltracion al nervio hipogloso en su paso a traves del canal oseo. Casos clinicos. Se presentan dos casos clinicos de sindrome del condilo occipital secundario a un hepatocarcinoma metastasico. El primero, un varon de 52 años con cirrosis hepatica secundaria a hepatopatia por virus de la hepatitis C, con sindrome del condilo occipital como sintoma inicial en un hepatocarcinoma diseminado; y el segundo, un varon de 56 años, tras recidiva de un hepatocarcinoma despues de un trasplante hepatico, a pesar de no cumplir los criterios de Milan. Conclusion. El sindrome del condilo occipital es un sintoma de alarma y requiere realizar un estudio completo mediante pruebas de imagen, puesto que puede ser la primera manifestacion de un hepatocarcinoma oculto.

Schwannoma of the trochlear nerve-an illustrated case series and a systematic review of management.

Schwannomas of cranial nerves in the absence of systemic neurofibromatosis are relatively rare. Among these, schwannomas of the trochlear nerve are even less common. They can be found incidentally or when they cause diplopia or other significant neurological deficits. Treatment options include observation only, neuro-ophthalmological intervention, and/or neurosurgical management via resection or sterotactic radiosurgery (SRS). In recent years, the latter has become an attractive therapeutic tool for a number of benign skull base neoplasm including a small number of reports on its successful use for trochlear Schwannomas. However, no treatment algorithm for the management of these tumors has been proposed so far. The goal of this manuscript is to illustrate a case series of this rare entity and to suggest a rational treatment algorithm for trochlear schwannomas, based on our institutional experience of recent cases, and a pertinent review of the literature. Including our series of 5 cases, a total of 85 cases reporting on the management of trochlear schwannomas have been published. Of those reported, less than half (40 %) of patients underwent surgical resection, whereas the remainder were managed conservatively or with SRS. Seventy-six percent (65/85) of the entire cohort presented with diplopia, which was the solitary symptom in over half of the cases (n = 39). All patients who presented with symptoms other than diplopia or headaches as solitary symptoms underwent surgical resection. Patients in the non-surgical group were mostly male (M/F = 3.5:1), presented at an older age and had shorter mean diameter (4.6 vs. 30.4 mm, p < 0.0001) when compared to the surgical group. Twelve patients in the entire cohort were treated with SRS, none of whom had undergone surgical resection before or after radiation treatment. Trochlear schwannoma patients without systemic neurofibromatosis are rare and infrequently reported in the literature. Of those, patients harboring symptomatic trochlear Schwannomas do not form a single homogenous group, but fall into two rather distinct subgroups regarding demographics and clinical characteristics. Among those patients in need of intervention, open microsurgical resection as well as less invasive treatment options exist, which all aim at safe relief of symptoms and prevention of progression. Both open microsurgical removal as well as SRS can achieve good long-term local control. Consequently, a tailored multidisciplinary treatment algorithm, based on the individual presentation and tumor configuration, is proposed.

Malignant triton tumor of trigeminal nerve-case report.

INTRODUCTION: Here we are presenting a unique case of malignant triton tumor of the trigeminal nerve in a 4-year-old boy who presented with diplopia and ptosis. INTERVENTION: Near total excision of the tumor was performed, and adjuvant chemotherapy and radiotherapy were administered. RESULTS: The patient is in good health and has no evidence of clinical and radiological tumor recurrence for 22  months.

Clinical perineural invasion of the trigeminal and facial nerves in cutaneous head and neck squamous cell carcinoma: Outcomes and prognostic implications of multimodality and salvage treatment.

BACKGROUND: Clinical perineural invasion (PNI) of the trigeminal and facial nerves from cutaneous head and neck squamous cell carcinoma (cutaneous HNSCC) is a rare clinical entity that poses unique therapeutic challenges. METHODS: A retrospective chart review of a skull base oncology database was performed of patients with the diagnosis of clinical PNI from a cutaneous HNSCC. Patients who were previously untreated underwent multimodal treatment entailing surgical resection and postoperative radiotherapy (PORT) and patients who were previously treated with radiotherapy underwent salvage surgical resection between the years 2006 and 2012. Survival outcomes, such as disease-free survival (DFS) and overall survival (OS), were analyzed and correlated with surgical factors, such as margin status, previous treatment, zone involvement, and trigeminal involvement (branch-specific), as well as the pretreatment and posttreatment pain scores. RESULTS: Of 21 patients with clinical PNI from cutaneous HNSCC, 7 patients (33%) were previously treated for their disease with primary radiotherapy. Negative tumor margins were achieved in 18 patients (86%). Three of the 7 patients (43%) undergoing salvage surgery had positive margins. One-year and 3-year DFS for previously untreated patients was 91% and 67%, respectively, whereas 1-year and 3-year DFS was 72% and 28%, respectively, for the previously treated patients. Previous radiotherapy, ophthalmic nerve involvement, and positive margins portended poorer survival outcomes in this study. CONCLUSION: The retrospective study of this rare clinical entity demonstrates that multimodal treatment can achieve favorable survival outcomes. © 2017 Wiley Periodicals, Inc. Head Neck 39: 1280-1286, 2017.

Causes and management of facial nerve palsy.

Facial nerve palsy causes disfigurement with cosmetic, functional and psychological repercussions. The facial nerve can be affected anywhere along its course. A comprehensive assessment considering all differential diagnoses is critical to optimal management, as prompt, appropriate therapy leads to better outcomes.

Facial Nerve Schwannomas: Review of 80 Cases Over 25 Years at Mayo Clinic.

OBJECTIVE: To elucidate the long-term clinical behavior, treatment, and outcomes of sporadic facial nerve schwannoma (FNS) in a large cohort of patients managed in the post-magnetic resonance imaging era. PATIENTS AND METHODS: Retrospective review at a single tertiary health care system (January 1, 1990, through December 31, 2015), evaluating 80 consecutive patients with sporadic FNS. RESULTS: Ninety-eight patients with FNS were identified; 10 with incomplete data and 8 with neurofibromatosis type 2 were excluded. The remaining 80 patients (median age, 47 years; 58% women) were analyzed. Forty-three (54%) patients presented with asymmetrical hearing loss, 33 (41%) reported facial paresis, and 21 (26%) reported facial spasm. Seventeen (21%) exhibited radiologic features mimicking vestibular schwannoma, 14 (18%) presented as a parotid mass, and 5 (6%) were discovered incidentally. Factors predictive of facial nerve paresis or spasm before treatment were female sex and tumor involvement of the labyrinthine/geniculate and tympanic facial nerve segments. The median growth rate among growing FNS was 2.0 mm/y. Details regarding clinical outcome according to treatment modality are described. CONCLUSION: In patients with FNS, female sex and involvement of the labyrinthine/geniculate and tympanic segments of the facial nerve predict a higher probability of facial paresis or spasm. When isolated to the posterior fossa or parotid gland, establishing a preoperative diagnosis of FNS is challenging. Treatment should be tailored according to tumor location and size, existing facial nerve function, patient priorities, and age. A management algorithm is presented, prioritizing long-term facial nerve function.

Endocrine tumors associated with the vagus nerve.

The vagus nerve (cranial nerve X) is the main nerve of the parasympathetic division of the autonomic nervous system. Vagal paragangliomas (VPGLs) are a prime example of an endocrine tumor associated with the vagus nerve. This rare, neural crest tumor constitutes the second most common site of hereditary head and neck paragangliomas (HNPGLs), most often in relation to mutations in the succinate dehydrogenase complex subunit D (SDHD) gene. The treatment paradigm for VPGL has progressively shifted from surgery to abstention or therapeutic radiation with curative-like outcomes. Parathyroid tissue and parathyroid adenoma can also be found in close association with the vagus nerve in intra or paravagal situations. Vagal parathyroid adenoma can be identified with preoperative imaging or suspected intraoperatively by experienced surgeons. Vagal parathyroid adenomas located in the neck or superior mediastinum can be removed via initial cervicotomy, while those located in the aortopulmonary window require a thoracic approach. This review particularly emphasizes the embryology, molecular genetics, and modern imaging of these tumors.

Atypical teratoid/rhabdoid tumor (ATRT) arising from the 3rd cranial nerve in infants: a clinical-radiological entity?

Atypical teratoid/rhabdoid tumor (ATRT) is a rare embryonal tumor of the central nervous system with preponderance in very young children, the majority of whom are younger than 3 years of age at diagnosis. Historically, outcomes of this aggressive disease, even with extensive multimodal therapy, have been dismal. Recent improvements have come from therapies directed exclusively towards ATRT, but misdiagnosis or delays in the correct diagnosis lead to significantly worse survival rates. ATRTs most commonly occur supratentorially but have been described in virtually all central nervous system locations, including the cerebellopontine angle cistern, meninges, and spinal canal, and extradural locations. ATRTs originating from cranial nerves are rare. Here, we describe three cases of solitary ATRT arising from the 3rd cranial nerve (CN III) or close to its origin in the midbrain, all of which presented in patients within 6 months of birth, with isolated unilateral oculomotor nerve palsy and strikingly similar magnetic resonance imaging (MRI) features. These MRI features include IV contrast enhancement, relative T2 hyposignal, and restricted water diffusion on apparent diffusion coefficient images, findings which are consistent with angiogenesis and high cellularity, and hence, suggestive of malignancy. We conclude that ATRT should be placed high on the differential diagnosis list when encountering a young infant presenting with isolated, unilateral 3rd nerve palsy and a small, solitary tumor arising from CN III that demonstrates malignant conventional and diffusion-weighted imaging features on MRI.

[Oculomotor nerve schwannoma in a child: Case report and literature review]. / Schwannome du nerf oculomoteur chez l'enfant: cas clinique et revue de la littérature.

An isolated schwannoma of the oculomotor nerve is rare in children without an associated neurofibromatosis. A 13-year-old girl, with a previous medical history of migraine, was admitted for disabling ophthalmic migraine with oblique diplopia. The clinical examination showed a right incomplete ophthalmoplegia with reduced ipsilateral visual acuity (8/10). There was no particular skin reaction. The MRI revealed a right (isosignal-T1 and isosignal-T2) nodular schwannoma located within the cisternal segment of the oculomotor nerve. The angio-CT performed later confirmed the absence of any vascular malformation. The treatment consisted of analgesics and corticotherapy, with complete regression of symptoms three weeks later and a normal MRI follow-up. Therefore, radiosurgery was not performed.

Atypical teratoid/rhabdoid tumor (AT/RT) arising from the acoustic nerve in a young adult: a case report and a review of literature.

Atypical teratoid/rhabdoid tumors (AT/RTs) are rare, highly malignant central nervous system tumors that predominantly occur in young children. A 22-year-old woman presented with a 4-year history of relapsing tinnitus and gradual hearing loss. Neuroimaging revealed an enhanced intrinsic left internal auditory canal mass. The patient underwent radiotherapy treatment. Three years later, the tumor size continued to increase, as observed by imaging, and ultimately evolved into the left cerebellopontine angle. As a consequence, a total tumor resection was performed, and a pathological diagnosis of AT/RT was made. Aggressive radiotherapy and chemotherapy treatment continued; however, the tumor recurred within 11 months after the total tumor resection. The patient died within 4 months of the second operation. Histopathologically, the tumor contained characteristic rhabdoid cells with areas that resembled a classical primitive neuroectodermal tumor. Immunostaining showed loss of INI1 protein expression in tumor cells, and fluorescence in situ hybridization showed a hemizygous deletion of the hSNF5/INI1 gene region on 22q11.2. This is the first report of an AT/RT that arised from the acoustic nerve in a young adult. Despite manifold diagnostic and therapeutic advances, the prognosis of patients with AT/RT remains poor.

Clinical and radiological guidance in managing facial nerve schwannomas.

OBJECTIVE: To present a review of all patients diagnosed with a facial nerve schwannoma (FNS) managed in our center over almost two decades, and suggest guidelines for their classification and management. STUDY DESIGN: Retrospective case review SETTING: Tertiary referral center PATIENTS: Twenty-eight patients with a facial nerve schwannoma INTERVENTION: Conservative or surgical management depending on clinical and radiological features MAIN OUTCOME MEASURE: Patient demographics, site of tumor, and clinical symptoms, including facial nerve function (House-Brackmann score) at baseline and follow-up. In those managed surgically, operative approach and surgical outcomes were also recorded. RESULTS: Of 28 patients, 16 were male. Mean age at presentation was 46 years. The majority presented with either facial weakness or hearing loss. The internal auditory canal segment of the facial nerve was the most commonly affected (19/28, 68%). Multi-segmental lesions were found in almost half (46%) of patients. Facial weakness was most commonly associated with involvement of the labyrinthine segment (89%). Overall, 16 (57%) patients were managed surgically. CONCLUSION: FNS may be difficult to distinguish on both clinical and imaging grounds from other cerebellopontine pathologies on the basis of audiovestibular symptoms alone. The presence of facial weakness in combination with imaging findings suggestive of FNS is highly suggestive for FNS. In patients with brainstem compression, rapid tumor growth, or House-Brackmann greater than 4, we suggest a surgical approach based on preoperative audiovestibular status, helping optimize long-term facial function and minimize morbidity. Facial nerve reanimation at the time of primary surgery is preferred.

Cranial and spinal oligodendrogliomatosis: a case report and review of the literature.

INTRODUCTION: Oligodendrogliomatosis is a rarely reported entity in literature associated with poor prognosis in terms of length and quality of life. In this paper, we describe oligodendrogliomatosis in a 15-year-old male who initially presented with altered mental status due to diabetic ketoacidosis. CLINICAL PRESENTATION: He was refractory to temozolomide initially but demonstrated disease regression with radiotherapy (XRT). More recently, he has had disease recurrence, which was stabilized with temozolomide therapy for a period of time. CONCLUSION: Contrary to most reports in literature, our patient has had excellent quality of life since his initial diagnosis and continues to carry good prognosis. In addition to oligodendrogliomatosis, our patient also developed multiple intracranial cavernomas secondary to radiation therapy, which have remained stable and asymptomatic.

Facial nerve paralysis after pre-operative embolization of a paraganglioma.

Vascular tumors pose a challenging problem in treatment, as surgical planning can be extensive. Often times, pre-operative embolization is required to minimize blood loss during surgery. With the advent of new biochemical compounds, embolization modalities have evolved over the past decade. Although rare, side effects and complications of embolic materials have been cited sporadically in the literature. We present an interesting case of a patient afflicted with facial paralysis and other cranial neuropathies following embolization of a paraganglioma, along with the appropriate imaging that confirms the etiology of her paralysis.

Primary glioblastoma of the trigeminal nerve root entry zone: case report.

Gliomas of the cranial nerve root entry zone are rare clinical entities. There have been 11 reported cases in the literature, including only 2 glioblastomas. The authors report the case of a 67-year-old man who presented with isolated facial numbness and was found to have a glioblastoma involving the trigeminal nerve root entry zone. After biopsy the patient completed treatment with conformal radiation and concomitant temozolomide, and at 23 weeks after surgery he demonstrated symptom progression despite the treatment described. This is the first reported case of a glioblastoma of the trigeminal nerve root entry zone.

Management of vagal paragangliomas: review of 17 patients.

Vagal paragangliomas are very rare benign vascular tumors of neuroendocrine nature, and are much less frequent than carotid and jugulo-tympanic tumors. The goal of this retrospective study is to review the clinical and genetic findings, surgical treatment, and complications of vagal paragangliomas, as well as to discuss the management options. During the period 1990-2013, 17 patients with vagal paragangliomas were referred to our institution. There were ten patients with isolated tumors, and seven with multicentric paragangliomas. There were nine women and eight men. Mean age of patients was 51.4 years. Five cases had a positive family history of paraganglioma (29.4 %). Germline mutations of SDH genes were found in six of our patients (35.3 %). Many options were considered in the management of vagal paragangliomas. Surgical treatment was performed in 11 young patients (64.7 %) using different approaches: in 4 patients the tumor was resected through a transcervical approach; in 3 through a transcervical-transmandibular approach; in 1 it was resected using a transcervical-transmastoid approach, and in 3 a type A infratemporal fossa approach was performed. In all operated cases, the removal of the tumor led to sacrificing of the vagus nerve. Postoperative hypoglossal nerve deficit was reported in 4 cases (36.3 %). In six elderly patients (35.3 %), we decided to "wait-and-scan" in order to avoid creating greater morbidity than that of the tumor itself. Many factors should be considered in the treatment of vagal paragangliomas: the age and general condition of the patient, the biological behavior of the tumor, tumor size, genetic results, bilaterality, multicentricity, lower cranial nerve function, and of course the potential morbidity of the surgical treatment itself. Rehabilitation and, possibly surgery, are necessary to treat postoperative lower cranial nerve deficits.

Long-term outcomes of facial nerve schwannomas with favorable facial nerve function: tumor growth rate is correlated with initial tumor size.

OBJECTIVE: The study aimed to report long-term outcomes of facial nerve schwannomas (FNS) with favorable facial nerve function by observation, and to discuss about the relationship between initial tumor size and tumor growth. METHODS: 21 facial nerve schwannoma cases with favorable facial nerve function were managed by observation. They were divided into larger size group (size ≥10mm) and smaller size group (size <10mm) according to initial tumor size. RESULTS: They were followed up for 6.4±1.7years. 18 of 21 cases (85.7%) maintained House-Brackmann Grade III or better. Growth rate of the tumors in larger size group was 72.7%, much higher than 10% in smaller size group (p<0.05). CONCLUSIONS: Observation was feasible for most FNS with favorable facial nerve function, and growth rate of the tumors was associated with tumor size.