Search

Objective: We identified the potential role of electroacupuncture (EA) as an alternative therapy to traditional Chinese medicine (TCM) in a rare case of postoperative gastroparesis after gastric schwannoma (GS)., Clinical Presentation: A 31-year-old woman presented with impaired gastric emptying after gastrectomy for GS and was diagnosed with postoperative gastroparesis syndrome (PGS). The symptoms were slightly relieved after routine placement of the jejunal tube; however, symptoms such as dietary intolerance and impaired gastric emptying persisted. After the consultation, the patient agreed to undergo EA therapy., Intervention and Results: The patient was able to tolerate oral intake after seven days of EA treatment, and the frequency and amount of food intake increased. The jejunal tube was removed at the outpatient follow-up two weeks after discharge, and the patient resumed a semi-liquid diet and was able to eat small amounts of rice. Reexamination of the upper digestive tract angiography showed that part of the contrast agent passed through the pyloric sinus, which showed improvement., Conclusion: EA stimulation increased tolerance to transoral feeding in patients with postoperative gastroparesis and facilitated the passage of contrast agents through the pyloric sinus. No adverse effects were observed during treatment, and the treatment was well accepted and tolerated by patients. A review article noted the benefits of acupuncture for gastrointestinal disorders but lacked high-quality evidence to support this. 1 Therefore, the therapeutic role of EA needs to be further elucidated to provide high-quality evidence-based medical evidence for its clinical use., (Copyright © 2023. Published by Elsevier Inc.)

Background: Schwannomatosis (SWN) is a rare tumor suppressor syndrome that predisposes affected individuals to develop multiple schwannomas and, less often, meningiomas. The most common symptom is chronic, severe pain. No medications are broadly effective in treating SWN-associated pain. The clinical trial described in this manuscript is a phase 2, randomized, double-blind, placebo-controlled study investigating the safety and efficacy of tanezumab - a humanized monoclonal antibody that inhibits nerve growth factor - for treatment of SWN-related pain. As the first therapeutic trial for SWN-related pain, it also aims to evaluate trial endpoints, understand recruitment patterns, and improve clinical trial design in this rare disease., Aims: The primary objective of this trial is to assess the analgesic efficacy of subcutaneous tanezumab 10 mg in subjects with SWN who continue pre-existing pain therapy (excluding non-steroidal anti-inflammatory drugs). The secondary objective is to assess safety in this population. Exploratory objectives include assessment of pain features, quality of life, and predictive biomarkers., Methods: The study is comprised of four periods (pre-treatment, double-blind treatment, single-arm treatment, safety follow-up) across 10 months with a delayed-start trial design to allow all participants to receive tanezumab. Forty-six participants will be enrolled and randomized 1:1 to receive either tanezumab or placebo subcutaneously in the double-blind treatment period; all participants receive tanezumab during the single-arm treatment period., Conclusions: This study is the first therapeutic trial for SWN patients and targets a biological driver of SWN-related pain. It aims to establish a model for future pain studies in SWN and other rare diseases., Clinical Trial Registration: NCT04163419 on ClinicalTrials.gov., Competing Interests: Declaration of Competing Interest JLWD reports no conflicts of interest. VLM reports consulting for The Neurofibromatosis Network and grant funding from the Children's Tumor Foundation. JTJ reports paid consulting for Navio Theragnostics, CereXis, Recursion Pharmaceuticals, Health2047, Creative Educational Concepts. <1% equity in Navio Theragnostics. Royalties from Elsevier. KIL reports consulting for SpringWorks Therapeutics, grant funding from the Neurofibromatosis Therapeutic Acceleration Program and Department of Defense. MP reports no conflicts of interest. PW reports grant funding from the Neurofibromatosis Therapeutic Acceleration Program. LX reports no conflicts of interest. MTB was a full-time employee of Pfizer and owns Pfizer stock. SS is a full-time employee of Pfizer and owns Pfizer stock. AM reports no conflicts of interest. SRP is co-founder of NFlection Therapeutics and NF2 Therapeutics; serves on the scientific advisory board and owns stock in SonALAsense; and consults for Akouos., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: This study aimed to clarify the risk of communicating hydrocephalus in cerebellopontine angle tumors, focusing on distinct tumor types and treatment modalities, i.e., tumor resection and stereotactic radiosurgery (SRS)., Methods: This study was a retrospective single-center cohort study. The cumulative incidences of symptomatic communicating hydrocephalus in schwannoma and meningioma patients were evaluated. A multivariate Cox model was used to assess the hazard ratios for the risk factors and odds ratios of distinct treatment subgroups., Results: A total of 405 cases, including 286 schwannomas and 119 meningiomas, were retrospectively reviewed. The risk of hydrocephalus was significantly higher in schwannomas than that in meningiomas (hazard ratio, 4.70 [95% confidence interval, 1.78-12.4, P = 0.002]). Patients with schwannomas who received SRS without tumor resection showed a significantly higher incidence than meningioma cases: 10.6% versus 1.4% (P = 0.037). We identified specific subgroups that were prone to increase the risk of hydrocephalus when treated with SRS alone. The result showed that patients with vestibular schwannoma of Koos grade III had a greater benefit from tumor resection than from SRS in preventing hydrocephalus (odds ratio, 0.089 [95% confidence interval, 0.011-0.743, P = 0.025])., Conclusions: Symptomatic communicating hydrocephalus is more frequent in schwannoma than that in meningiomas. Primary treatment with tumor resection lowers the risk of hydrocephalus in specific subgroups of vestibular schwannoma., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background and Objective: Cranial nerve schwannomas almost always arise from sensory or mixed nerves. Motor cranial nerves, such as the trochlear nerve, are rarely associated with schwannomas. No consensus has yet been made for surgical intervention because of the low number of reported cases of trochlear nerve schwannomas. This study comprises a systematic review of the literature and our experience for surgically treated trochlear nerve schwannomas., Methods: Three databases (Web of Science, PubMed, and Cochrane Library) were searched without date restrictions. Studies were included if they were published in the English literature and presented patients of any age who underwent surgical treatment for trochlear schwannoma. Data extracted from the included studies were combined with our experience., Results: Forty-one studies, presenting 43 patients, met the inclusion criteria. The total number of patients was 45 after our experience was added. The most common symptoms were diplopia (62.2%), headache (46.7%), and motor weakness (37.8%). Mean age during the diagnosis was 45.1 years. Although the subtemporal transtentorial approach (n = 14) is the most preferred method, its application has decreased in recent years. In the last decade, the lateral suboccipital approach (n = 11) has gained popularity. Residual postoperative trochlear nerve deficit was detected in 81% of patients. The probability of neurologic deficit was not statistically associated with tumor volume (P = 0.914), location (P = 0.669), or resection rate (P = 0.554)., Conclusions: Although trochlear schwannomas are rare and their treatment involves challenges, total resection with the proper approach provides the most desirable results., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: This study evaluated the characteristics of vestibular schwannomas (VS) in young patients, including clinical features, treatment, prognosis, and histopathologic characteristics., Methods: We retrospectively reviewed medical records and follow-up data for 36 pediatric patients <21 years of age who were surgically treated for VS in the Chinese PLA General Hospital between 2008 and 2019., Results: Mean patient age was 17.4 years. Mean tumor size was 2.8 cm. Hearing loss (n = 32, 88.9%) and tinnitus (n = 20, 55.6%) were the most common symptoms. Ten patients (27.8%) had impaired facial nerve function after surgery. Gross total resection (GTR) was achieved in 26 cases (72.2%). The median tumor Ki-67 level was 5%. Tumor size was related to incomplete tumor resection (odds ratio, 0.2; 95% confidence interval, 0.1-0.9) and postoperative facial nerve dysfunction (odds ratio, 24.9; 95% confidence interval, 1.2-539.1). Tumor size was nonlinearly associated with prognosis and 2.2 cm corresponded to the inflection point at which the probability of tumor remnant and postoperative facial nerve dysfunction significantly increased. The GTR and low Ki-67 groups achieved better 3-year tumor control rate. Histopathologic findings confirmed the presence of cellular schwannoma subtype in young patients., Conclusions: Tumor size is an important factor affecting the prognosis of VS in young patients. For large VS, surgical treatment should be the first choice, rather than wait-and-scan. VS in young patients shows high tumor proliferation and a tendency to relapse. The cellular schwannoma subtype requires special attention; an accurate histopathologic diagnosis is necessary for young patients with VS, and a closer follow-up strategy should be adopted for cellular VS., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Trigeminal schwannomas are complex lesions that may be related to many critical neurovascular structures. We present the case of a 59-year-old male presenting a history of left-sided trigeminal neuralgia. Preoperative imaging demonstrated a mass highly suggestive of a trigeminal schwannoma, and microsurgical resection was indicated considering the progressive symptomatology and important mass effect (Video 1). A middle fossa route including an anterior petrosectomy was chosen. The patient was placed supine with the head rotated to the contralateral side, and an arcuate incision was performed. A V-shaped zygomatic osteotomy was done to mobilize the temporalis muscle more inferiorly and better expose the middle fossa floor. Following craniotomy, peeling of the dura propria from the lateral wall of cavernous sinus was carried out starting by coagulation of middle meningeal artery. Some tumor was already identified and removed, and then the anterior petrosectomy was performed until we exposed the posterior fossa dura. The middle fossa dural incision was connected with the other one at the posterior fossa dura, by coagulation of the superior petrosal sinus. The tentorium was completely cut toward the incisura. After lesion debulking, the tumor was progressively removed by peeling the arachnoid from the lesion to maintain arachnoid planes and preserve the nerves and their blood supply. Postoperative imaging demonstrated complete tumor resection. The patient's symptoms improved, and there were no neurologic deficits on follow-up. Extensive laboratory training is fundamental to be familiarized with the normal anatomic nuances and prepared to face the anatomy distorted by lesion. Informed consent was obtained from the patient for the procedure and publication of this operative video., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Schwannomas at the craniocervical junction commonly originate from the lower cranial nerves or C1 and C2 nerves. To date, very few cases of C1 schwannomas have been described in the literature, and the majority involve either the intra- or the extradural compartment, but not both. To our knowledge, this report documents the first case of a dumbbell-shaped C1 schwannoma that encompassed both intra- and extradural compartments and was accompanied by hydrocephalus., Case Description: The patient was admitted to our hospital, where magnetic resonance imaging revealed a tumor at the craniocervical junction, extending from the C1 level of the right first cervical vertebra into the cerebellopontine angle with 2 giant cysts. We removed the tumor by performing a midline posterior craniectomy and cervical laminectomy. Intraoperatively, the tumor was found to originate from the right C1 posterior root. The pathological diagnosis was of a schwannoma. The patient was subsequently discharged without any neurologic deficits., Conclusions: To our knowledge, we present the first case of a dumbbell-shaped C1 schwannoma with intracranial extensions and accompanying hydrocephalus. The tumor had spread inside and outside the dura, but was safely removed. Our findings in this case emphasize that to achieve safe resection, detailed case-specific preoperative consideration is essential., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Posterior mediastinal tumors are not infrequent, and among them neurogenic masses and schwannomas are the most common histologic varieties. These benign, initially asymptomatic tumors later become symptomatic as a result of mass effect. Surgical excision is the preferred therapy, and the approach can be determined according to the dimensions of the lesion. This report describes the case of a giant schwannoma originating from the left vagus nerve in a middle-aged woman whose symptoms were exertion-induced dyspnea and atrial fibrillation., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Giant sacral schwannomas are very rare, and less than 1%-5% of spinal schwannomas are found in the sacral region. These frequently grow to considerable size because of permissive anatomic location and benign, slow growth of tumor. They can be unnoticed before reaching a huge size., Case Description: We report a rare case of a giant sacral schwannoma in a 46-year-old man. The patient presented with difficulty in passing urine, episodic constipation, and swelling of the right lower extremity for 6 months. Magnetic resonance imaging revealed 160 x 110 x 110 mm encapsulated heterogenous solid mass originated from left S1 spinal nerve extending into the pelvis and abdomen. Sigmoid colon and rectum were displaced to the right side, and bladder was displaced anteriorly. Left side of the S1 and S2 vertebral bodies, left S1 and S2 neural foramen were also eroded. It also compressed ureters causing bilateral hydronephrosis. The patient underwent a 2-stage procedure in which complete resection was achieved., Conclusions: We report the second case of a completely resected giant sacral schwannoma with bilateral hydronephrosis in the literature. Performing a 2-stage procedure is important in giant sacral schwannomas. Morbidity can be minimized, and extent of resection can be maximized with the help of combined anterior/posterior approach., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Many different benign and malignant tumors develop in the chest wall and pose a diagnostic and therapeutic challenge to clinicians. Chest wall schwannomas of the intercostal nerve are rare. This report describes the clinical and imaging findings of a patient who presented with persistent and progressive chest pain. The intercostal tumor was treated using wide excision with chest wall reconstruction and titanium plate fixation. Schwannoma was diagnosed on the basis of histopathologic examination., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Ectopic intracranial schwannomas (those that do not arise from a named cranial nerve) are rare. They account for <2% of surgically resected central nervous system schwannomas., Case Description: We report the case of a 14-year-old boy presenting with a left conductive hearing loss and temporal bone deformity. No facial or cranial nerve deficits were present. Cross-sectional imaging demonstrated a large expansile extra-axial temporal bone mass, extending into and distorting the middle cranial fossa. At surgical resection the tumor was functionally and anatomically distinct from the facial nerve or any other identifiable neural structure within the middle ear or temporal bone. Histology confirmed a World Health Organization grade 1 schwannoma., Conclusions: This is the first reported case of a giant juvenile ectopic schwannoma within the temporal bone., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Tumors arising from oculomotor nerve are rare, with few cases reported in the literature. Generally, whereas schwannomas are well encapsulated tumors, neurofibromas tend to invade the entire nerve fibers. These differences influence surgical resection and neurological clinical outcome, with neurofibroma often requiring the sacrifice of the nerve. Accordingly, an incorrect preoperative diagnosis can lead to incomplete patient counseling before surgery., Case Description: We report 2 cases: a patient with oculomotor schwannoma and a patient with oculomotor neurofibroma. After tumor resection, the patient with a diagnosis of schwannoma recovered with 3rd nerve palsy, while patient with the neurofibroma developed a complete oculomotor nerve deficit. For each patient, surgical strategy and neurological outcome are elucidated in relation with differences in preoperative magnetic resonance imaging and histology., Conclusions: To the best of our knowledge, this is the first report of an oculomotor neurofibroma. When an oculomotor nerve tumor is suspected, a careful preoperative evaluation of magnetic resonance imaging guides in distinguishing the different histology, in selecting the treatment strategy, and in correctly informing the patient on expected postoperative neurologic outcome., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: This report presents the third case of trochlear schwannoma arising from the pineal region and the first case to be resected using a paramedian infratentorial supracerebellar approach. Schwannomas of cranial nerves have traditionally been thought to arise from the transitional point where the axonal envelopment switches from glial cells to Schwann cells; however, recent temporal bone histopathologic evidence from vestibular schwannomas challenges this view. Of the 38 cases of pathology-confirmed trochlear schwannoma in the literature, there are only 2 cases arising from the pineal region, where the nerve sheath transition zone is located. Here, we discuss an unusual case of trochlear schwannoma arising from this transition zone., Case Description: A 65-year-old man was admitted to our institute after a traffic accident. He complained of headache and dizziness, and a computed tomography scan revealed an isodense mass in the pineal region with obstructive hydrocephalus. Magnetic resonance imaging with contrast showed an enhancing mass in the pineal region. The tumor was subtotally resected using a paramedian infratentorial supracerebellar approach, and pathology confirmed the diagnosis of trochlear schwannoma., Conclusions: Trochlear schwannoma should be considered when a mass is identified in the pineal region. This diagnosis should still be entertained for mass lesions along the free tentorial edge because the tumor may arise distant from the glial-Schwann transition zone located by the dorsal midbrain. We propose a treatment algorithm for this rare tumor that seeks to maximize functional outcome., (Copyright © 2020. Published by Elsevier Inc.)

Background: Sacral schwannomas are rare tumors arising from nerve sheath Schwann cells. They are classified into 3 types: first type schwannoma arising from the sacrum, second type schwannoma eroding the sacrum, and third type schwannoma extending extensively, involving anterior, posterior, and retroperitoneal space. Among these type 2 sacral tumors, according to Klimo's classification, an eroding sacrum extending all over posteriorly, anteriorly, and laterally is still rarer. Only 38 cases have been reported in literature reviews. Only 6 cases of giant tumors of >10 cm in any 1 dimension have been reported., Case Description: We present such a giant sacral schwannoma, with invasiveness eroding the sacrum; compressing the ureters, aortic bifurcation, bladder, and bowel; and presenting as lower abdominal pain, hydronephrosis, dysuria, and constipation. This is the first case reported in the literature that has described a large retroperitoneal tumor compressing retroperitoneal structures-aortic bifurcation vascular compression and ureter compression causing hydronephrosis., Conclusions: Though complete resection, which caused various severe postoperative complications in the reported cases, is the best option, a less morbid procedure would be appropriate; hence we underwent subtotal excision of the tumor, with complete recovery of our patient's symptoms without neurologic deficit., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Benign peripheral nerve sheath tumors are well known to neurosurgeons and a relatively commonly seen pathology. Intraneural ganglion cysts, once thought to be rare and poorly understood, are increasingly recognized in clinical practice and better understood based on the advent of high-resolution imaging. There are few reports of different nerve lesions in the same anatomic location appearing concurrently. Herein we present a patient with 2 distinct pathologies explaining 2 distinct symptom complexes-sensory changes in the superficial peroneal distribution (from a schwannoma of the superficial peroneal nerve) and mild motor weakness in the tibialis anterior (from an intraneural ganglion cyst arising from the superior tibiofibular joint affecting this motor branch). Recognition of the 2 pathologies allowed targeted surgical approaches, which led to resolution of the symptoms., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Hypoglossal schwannomas are rare, benign intracranial neoplasms; they represent 5% of all nonvestibular schwannomas., Case Description: A 22-year-old male patient had presented 4 months before admission with left-sided hearing loss, an increase in the base of support with lateralization to the left, and dysphagia to solids. Physical examination on admission showed hypotrophy of the tongue and deviation to the left. Magnetic resonance imaging showed evidence of an extraaxial lesion compressing the medulla oblongata and pons, which protruded through the hypoglossal canal. The patient was prepared for surgical treatment in 2 stages: in the first surgery, a left retrosigmoidal approach with total resection of the intracranial lesion was performed. The biopsy reported a schwannoma, and correlating the signs, symptoms, and imaging, the diagnosis of a hypoglossal nerve schwannoma was established. In the second surgery, a lateral cervical approach was performed, with subtotal resection of the lesion, leaving a remnant adhered to the nerve in the hypoglossal canal., Conclusions: Hypoglossal schwannomas are a rare entity, in which surgery is the most viable option with high cure rates. However, its complete resection, without leaving permanent neurologic sequels, is a challenge., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Schwannomas are the most common benign tumors in the intraspinal location, with slow-growing and nonaggressive features. Calcification is not a common histopathological and radiological feature in schwannoma., Case Description: We report the case of a 43-year-old man with soreness in the lower back, left buttock, and lower limb of 1 month's duration. On magnetic resonance imaging and computed tomography, an intradural mass with extensive calcification was found at the L1-L2 level. The tumor was completely resected, with sacrifice of 1 nerve root. Postoperatively, his symptoms were relieved, and no dysfunction of the lower limbs and sphincter was observed. Pathologically, the tumor was diagnosed as a schwannoma with extensive calcification. At the 7-month follow-up examination, no complaints were found., Conclusion: Although intraspinal schwannoma with extensive calcification is rare, which increases the difficulty of an accurate diagnosis preoperatively, the tumor can be completely removed with or without excision of the nonfunctional nerve roots., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: Lesions affecting the sciatic nerve (SN) can mimic lumbar radiculopathy. In patients presenting with sciatica, approximately 10% have a nondiscogenic etiology. Through neurological examination and imaging, it may be possible to confirm nondiscogenic sciatica (NDS). This study aims to present a series of 6 patients with infragluteal NDS, highlighting clinical and imaging aspects that may suggest this diagnosis., Methods: This is a retrospective study of 6 patients treated for NDS from 2010 to 2018. The mean and median ages were 41.2 and 38.5 years, respectively, with all patients female., Results: All patients presented with sciatic pain, tenderness to deep infragluteal palpation, and a positive Tinel's sign related to the SN. Four patients were referred for surgical treatment, whereas 1 underwent pharmacological therapy and 1 underwent incisional biopsy and radiotherapy. In our series, 6 different causes for NDS were diagnosed: 1 nontumorous cause: extrauterine endometriosis and 5 tumors: metastasis from rectal adenocarcinoma, low-grade sarcoma, schwannoma, high-grade sarcoma, and myxoma., Conclusions: Differentiating between discogenic and NDS can be challenging for clinicians. When patients present with sciatic pain, a Tinel's sign related to the SN elicited at the deep infragluteal region, tenderness to deep infragluteal palpation, occasionally with an SN motor deficit and imaging findings of the lumbar spine that do not justify a discogenic source, the cause should be considered nondiscogenic and they should be scheduled for magnetic resonance imaging of the gluteal and pelvic region., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Schwannoma originating from the cervical vagus nerve is a rare neoplasm. Vagal nerve schwannoma (VNS) usually occurs between the third and fifth decades of life., Methods: We operated one patient for cervical schwannoma causing internal carotid artery (ICA) compression., Results: The patient underwent en bloc excision via transcervical approach under general anesthesia. Pathological examination demonstrated the diagnosis of schwannoma., Conclusions: Our case shows that VNS can cause ICA compression and therefore brain ischemia., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Intracranial schwannomas are rarely confined to the skull. We here report a large schwannoma localized in the petrous apex that presented with intratumoral hemorrhage., Case Description: A 35-year-old woman with mild hearing disturbance and ear fullness underwent computed tomography scan and magnetic resonance imaging, which demonstrated a tumor accompanied with intratumoral hematoma in the right petrous apex. Bone marrow was totally destroyed, but the bone cortex was relatively preserved. Pathologic specimen showed that the tumor was composed of proliferation of elongated neoplastic cells and positive for S-100 protein. It showed a nuclear palisading pattern, compatible with schwannoma. The lack of any cranial nerve signs and relative preservation of canals through which cranial nerves pass suggested the neurilemma cells surrounded vessels or mismigrated fetal neurilemma cells in the petrous apex as origin of the tumor. Benign nature of the tumor and total disappearance of the symptoms, supposedly due to the spontaneous absorption of the hematoma, made the patient choose a wait-and-watch approach. Magnetic resonance imaging studied 7 years after the diagnosis showed significant decrease of the tumor volume and disappearance of the hematoma., Conclusions: Although it is a large intraosseous schwannoma in the petrous apex, it has a benign nature, its size is reduced due to the hematoma absorption, and the patient is asymptomatic. We observed the patients for 7 years after the diagnosis., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Sacral schwannomas are very rare nerve sheath tumors. Patients usually present with a variety of nonspecific symptoms, which often lead to a delay in diagnosis. Although most schwannomas are benign, they present surgical challenges owing to their proximity to neurologic and other anatomic structures., Case Description: This 58-year-old female presented with a 2-month old history of left-sided perineal and radicular pain secondary to a right S2 sacral nerve root schwannoma. The sacral mass demonstrated homogenous enhancement with cystic changes in a T2-weighted magnetic resonance imaging sequence. The patient underwent S1-S3 laminectomy and tumor excision through a posterior surgical approach. Intraoperative monitoring was used to distinguish nonfunctional tissue during tumor resection. The patient had an unremarkable postoperative course., Conclusions: Sacral schwannomas can present with a variety of nonspecific symptoms. They pose unique challenges given their location, size, and involvement of surrounding structures. Complete surgical resection is the main goal of sacral schwannoma treatment. A combined anterior-posterior surgical approach and a multidisciplinary surgical team are associated with improved outcomes., (Published by Elsevier Inc.)

Schwannomatosis is a rare affection predisposing to multiple peripheral neurologic tumors development. Approximatively, one third of patients with schwannomatosis are carriers of a germline mutation in LZTR1 (Leucin Zipper Transcription Regulator 1). Tumorigenesis in schwannomatosis responds to a somatic 5-hit/3-step mechanism resulting in a loss of function (LOF) of LZTR1 and the contiguous genes of locus 22q11.2q12.2. Effectively, LZTR1 is mapped on 22q11.2 and centromeric to SMARCB1 also implicated in the determinism of schwannomatosis and NF2, responsible for neurofibromatosis type 2. On a somatic point of view, LZTR1 mutations are known to drive with a significant frequency glioblastoma (GB) development. We report here two families in which segregate both multiple schwannomas and GB. In the first family, the proband received a diagnosis with of schwannomatosis after a surgery for a lumbar schwannoma at age 43, molecularly confirmed by identification of a germline heterozygous mutation in LZTR1. Her father, having unremarkable medical history deceased from an apparently isolated GB at age 59. In the second family, LZTR1-related schwannomatosis was diagnosed in the index case at age 70 after multiple schwannomas surgeries. Her elder sister had no neurological medical history before occurrence of a lethal GB at age 78. Molecular analysis of GB sample from both affected relatives showed the presence of the familial mutation. These observations hypothesize a potential link between schwannomatosis and the GB development., (Published by Elsevier Masson SAS.)

Background: Migratory tumors of the spinal cord are rare and can present as a missing tumor intraoperatively. This can lead to unnecessary abandonment of surgery or unnecessary laminectomies., Case Description: We present a case of migratory intradural extramedullary tumor of the spine, which was missing intraoperatively. The surgery was abandoned, and on reimaging the tumor was found to have migrated. Here we summarize a review of the literature of such cases and the lessons we learned from our experience, emphasizing the measures to prevent tumor displacement and the importance of intraoperative imaging., Conclusions: Migratory schwannoma should be suspected in case of a missing lesion. Intraoperative ultrasonography and myelography use should be definitely considered where facility for intraoperative magnetic resonance imaging is not available. Preventive measures should be taken to avoid tumor migration in all routine cases of intradural extramedullary lesions., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Schwannoma originating from the greater superficial petrosal nerve is an extremely rare type of facial nerve schwannoma located in the middle cranial fossa around the midportion of the petrous bone. Here, we provide the first report of greater superficial petrosal nerve schwannoma presenting with contralateral facial palsy and hemiparesis due to compression of the pyramidal tract by associated intradural peritumoral cyst., Case Description: A 69-year-old woman presented with a 6-month history of gradually worsening gait unsteadiness. Magnetic resonance imaging demonstrated a well-defined tumor occupying the right middle cranial fossa and extending into the tympanic cavity. Notably, the tumor accompanied a large cyst in the intradural space, resulting in a leftward midline shift. Extradural exploration through thinned periosteal dura mater revealed the tumor within the interdural space. After debulking of most of the tumor in a pull-out, piecemeal fashion, intradural exploration revealed the peritumoral cyst located between the meningeal dura mater and brain parenchyma. Following the disappearance of the mass effect from the tumor, fenestration of the peritumoral cyst, which had been deeper-seated than the tumor, was easily achieved while avoiding excessive retraction of the temporal lobe. Postoperatively, mild left hemiparesis involving the face resolved completely and no new symptoms such as right facial palsy, hearing disturbance, or xerophthalmia developed as postoperative complications., Conclusion: The combination of extradural and intradural approaches in the appropriate order is essential for fenestration of an intradural peritumoral cyst along with removal of an interdural tumor., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Hydrocephalus and intracranial hypertension are rare signs of spinal tumors when presenting in isolation, particularly with benign tumors., Case Description: Herein reported is a case of a 53-year-old woman who presented with headache, blurry vision, communicating hydrocephalus, and intracranial hypertension. No primary intracranial pathology was identified, and there were no clinical signs or symptoms of intraspinal pathology. Lumbar puncture revealed elevated opening pressure, cerebrospinal fluid protein, and suspected tumor cells in the cerebrospinal fluid, thus prompting spinal imaging. A primary lumbar schwannoma was subsequently determined to underlie her symptoms, which resolved with tumor resection., Conclusions: Clinical suspicion of spinal pathology should be maintained in patients with unexplained intracranial hypertension, even in the absence of localizing signs of spinal pathology., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Neoplasms located in the Meckel cave account for 0.2%-0.5% of all intracranial tumors. This area is the site of many types of pathologic lesions, most often trigeminal nerve schwannomas and meningiomas. Melanin-containing tumors are rare in this area. These tumor types can be suspected if the magnetic resonance characteristics of a tumor has some differences in comparison with other types of central nervous system neoplasms. In fact, differential diagnosis of melanotic tumors is based mainly on the histopathologic criteria and immunohistochemical profile. This article presents a case report of melanotic schwannoma of the Meckel cave and a literature review of the problem., Case Description: A 23-year-old man underwent a 2-stage surgery for a dumbbell pigmented mass lesion located in the Meckel cave. No signs of recurrence were seen on follow-up magnetic resonance imaging (MRI) 3.5 years after the operation., Conclusions: Melanin-containing tumor can be suspected in the presence of radiologic characteristics, such as a hyperintense MRI signal on T1-weighted images and a hypointense signal on T2-weighted images. If a black extracerebral tumor is detected, the main course of surgical treatment is maximal excision despite it possibly being a malignant melanoma and the temptation to perform partial resection because of an unfavorable prognosis. Chemotherapy can be justified in the presence of an aggressive melanotic schwannoma., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Frontoethmoidal schwannomas are rare. No case manifesting exertional cerebrospinal fluid (CSF) rhinorrhea has ever been reported to the best of our knowledge., Case Description: In this report, we describe an extremely rare case of frontoethmoidal schwannoma extending through the olfactory groove with exertional CSF rhinorrhea as the initial symptom. A 50-year-old woman was presented to our clinic for frequent nasal discharge on exertion. A postcontrast computed tomographic scan demonstrated heterogeneously enhanced tumor from the anterior cranial fossa to the anterior ethmoid sinus. A gadolinium-enhanced T1-weighted magnetic resonance image revealed a well-defined heterogeneously enhanced tumor situated in the midline anterior cranial fossa and anterior ethmoid sinus. After the resection, the defect of the right anterior skull base was reconstructed with a fascia graft and adipose tissue taken from the abdomen, as well as a pedicle periosteum flap. A histologic examination revealed the tumor as schwannoma. Her rhinorrhea completely resolved. She regained her sense of smell and taste 1 month after the operation., Conclusion: According to previous reports, olfactory groove, and paraolfactory groove/periolfactory groove schwannomas can be divided into 4 types: subfrontal, nasoethmoidal, frontoethmoidal, and ethmofrontal. Among them, a frontoethmoidal schwannoma can manifest exertional CSF rhinorrhea as an initial symptom., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Giant intradural extramedullary schwannoma of the cervical spine usually causes severe spinal cord compression. This type of tumor has a low incidence. Patients present progressive loss of strength and other functions of the spinal cord. This article shows the clinical images of a 75-year-old male with the diagnosis of giant intradural extramedullary schwannoma and the cases reported in the literature., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Study Objective: To show a surgical video in which an incidentally found Meckel diverticulum was resected with a natural orifice-assisted laparoscopic approach during para-aortic resection of a retroperitoneal schwannoma., Design: Case report (Canadian Task Force classification III)., Setting: Tertiary referral center in New Haven, Connecticut., Interventions: This is a step-by-step illustration for resection of a retroperitoneal para-aortic schwannoma and of an incidentally found Meckel diverticulum. The patient was a 39-year-old white woman diagnosed with stage IV choriocarcinoma with metastasis to the lungs and left para-aortic area. She received chemotherapy in the form of etoposide, methotrexate, actinomycin-D, cyclophosphamide, oncovine (EMA-CO) and had an excellent clinical response with resolution of all metastatic disease except for the para-aortic mass. Therefore, she was taken to the operating room for laparoscopic resection of the persistent left para-aortic mass. After placement of four 5-mm abdominal ports, the pelvis and abdomen were explored and revealed an incidental Meckel diverticulum as well as the 5 cm left para-aortic mass. The peritoneum overlying the para-aortic mass was incised and the retroperitoneum explored. Given the proximity to the mass, left ureterolysis was performed. The retroperitoneal attachments were resected, and the left para-aortic mass was removed without any complications. At this point attention was turned to the Meckel diverticulum. In order not to extend the abdominal incisions, a posterior colpotomy was performed in the cul-de-sac equidistant from the uterosacral ligaments. Endo-GIA (Covidien, New Haven CT) was introduced through the 10-mm port site at the posterior colpotomy. Meckel diverticulum was resected without narrowing the lumen of the distal ileum. The specimen was removed in a contained manner through posterior colpotomy., Measurements and Main Results: The procedure was performed without any complications. The patient had an uneventful postoperative course and was discharged home on postoperative day 0. Pathology revealed a retroperitoneal schwannoma with negative margins and benign Meckel diverticulum without ectopic gastric or pancreatic tissue. The patient has been disease-free since the completion of surgery., Conclusion: Laparoscopic resection of the retroperitoneal schwannoma and Meckel diverticulum were successfully performed in this patient with history of stage IV choriocarcinoma. To our knowledge, this is the first report describing a natural orifice-assisted laparoscopic approach for resection of Meckel diverticulum. Natural orifice-assisted laparoscopy should be considered when the surgeon needs to remove a large specimen and/or to introduce >5-mm diameter instruments into the peritoneal cavity without having to extend the abdominal incisions., (Copyright © 2017 AAGL. Published by Elsevier Inc. All rights reserved.)

Background: Schwannomas arising from the cranial nerves controlling extraocular eye movements are very rare and usually present with some degree of diplopia., Case Presentation: We report a 50-year-old woman who presented with isolated left-sided trigeminal neuralgia of 6 months' duration. Imaging demonstrated a homogeneously enhancing mass in the left ambient cistern, and the patient was brought to the operating room for resection. A retrosigmoid approach was used, and the mass was directly visualized arising from the trochlear nerve and compressing the dorsal root entry zone of the trigeminal nerve. A gross total resection of the mass was achieved, and microvascular decompression of the trigeminal nerve was performed. The tumor was pathologically confirmed as a schwannoma. At 3-month follow-up, the patient's facial pain was resolved, and her extraocular eye movements were intact., Conclusions: A total of 32 pathology-confirmed cases of trochlear schwannoma have been previously reported in the English-language literature. Most of these tumors arose from the cisternal segment of the nerve, and most patients presented with frank trochlear nerve palsy on exam. We report the first case of trochlear schwannoma presenting with isolated trigeminal neuralgia., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Intramedullary schwannomas of the spinal cord are rare tumors. They are most commonly observed in the cervical region; however, few have been described in the conus medullaris. The association of intramedullary schwannomas with syringomyelia is also rare. In this report, we present a case of intramedullary schwannoma of the conus medullaris with syringomyelia, which was treated surgically., (Copyright © 2017. Published by Elsevier Taiwan.)

Introduction: This study assessed the treatment outcomes of upper extremity benign tumors using the patient-rated outcome measures of Hand20 questionnaire., Methods: In total, 304 patients who underwent surgery for benign bone and soft tissue tumors of the upper limb were included. Tumors were classified into three size groups: <1 cm, 1-3 cm, and >3 cm. Tumors were divided with respect to location: digit, hand, wrist, forearm, elbow, upper arm, or axilla. We prospectively assessed responses to the Hand20 questionnaire that was administered both before and after surgery., Results: The mean Hand20 and pain scores significantly improved after surgery in patients with ganglion cysts, giant cell tumors of the tendon sheath, enchondromas, or pyogenic granulomas. For patients with hemangiomas, schwannomas, or glomus tumors, although the mean pain scores improved significantly following surgery, there were no significant changes in the mean Hand20 scores. However, the statistical power for this analysis was low. The mean Hand20 and pain scores improved significantly, regardless of the size grouping. The mean Hand20 scores significantly improved after surgery in patients with finger, thumb, hand, or wrist tumors. Except for elbow to axillary tumors, the mean pain scores significantly improved in all patients., Conclusion: The results of Hand20 and pain scores suggest that most patients with benign hand tumors are indicated for surgery, but the degree of improvement differs according to tumor pathology and location but not size., (Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Schwannomas are usually benign tumours arising from Schwann cells of peripheral nerve sheath. Retroperitoneal location is extremely rare compromising 0.5-5% of all schwannomas, except in patients having von Recklinghausen's disease, in whom retroperitoneal location is more frequent and malignant cases are known to occur. Complete surgical excision with negative margins is the definitive treatment on which the diagnosis is usually made due to nonspecific radiologic appearance. In this article we would like to present an unusual cause of recurrent, abdominal pain in an eighty-six-year-old male., (Copyright © 2017 Pan-Arab Association of Gastroenterology. All rights reserved.)

Objective: Patients with neurofibromatosis type 2 (NF2) are prone to develop multiple intracranial neoplasms, such as schwannomas of the cranial nerves and meningiomas. The aim of our study was to investigate 1) the median growth rate per year, 2) the growth-free interval, and 3) the growth patterns of NF2-associated intracranial tumors., Methods: All available magnetic resonance (MR) images of patients from the regional neurofibromatosis center were collected. The depicted tumors' volumes were then calculated if the tumors met the following inclusion criteria: contrast enhanced T1-weighted MRI datasets had to be available from at least two investigations and tumors had to be measurable on at least two slices., Results: One-hundred and eighty-eight tumors from 52 patients (20 male, 32 female) met the inclusion criteria for volumetric analysis. Overall, the median follow-up time was 76.5 months per patient (range 13-199 months). After 5 years, the median tumor size was 196% ± 338% for vestibular schwannomas (VS), 204% ± 702% meningiomas (M), 128% ± 64.9% for non-vestibular schwannomas (NVS) and 139% ± 270% for pre-operated tumors of the cerebellopontine angle (TX), respectively. The median time to 20% tumor progression was 21 months for VS, NVS and TX, and 17 months for M. Overall, saltatory growth was the most common growth pattern (46.9%)., Conclusions: Most NF2-associated tumors display a saltatory growth pattern. Meningiomas and untreated vestibular schwannomas grow rapidly and overall at similar rates. Tumors of the CPA that have been operated on and non-vestibular schwannomas show less relative growth per year., (Copyright © 2016 Elsevier Inc. All rights reserved.)

A 44-year-old man was admitted to our hospital with severe back pain continuing for 12 years. Computed tomography of the chest revealed a 14 mm diameter tumor with calcification located in the right seventh rib. We performed right seventh rib and sixth to seventh intercostal muscle partial resection. Microscopically, the tumor showed typical features of a schwannoma composed of Antoni A and Antoni B tissues. The tumor had positive immunohistochemical staining for S-100 protein. The pathological diagnosis was intraosseous schwannoma. The patient's severe back pain disappeared and there was no recurrence or metastasis of the tumor during a 1-year follow up., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The phenomenon of intracerebral schwannoma is exceedingly rare, and its etiology still a matter of debate. No documented cases of intracerebral schwannoma containing vascular elements consistent with those of an arteriovenous malformation (AVM) have been reported. We describe such a case here., Case Description: A left temporal intraparenchymal lesion was discovered incidentally in a 34-year-old man after he suffered a mild trauma. The lesion was resected and found on histologic examination to be an intracerebral schwannoma with AVM-like vasculature. The patient made a full recovery after resection., Conclusions: To our knowledge, this is the first case of an intracerebral schwannoma with AVM-like characteristics to be reported in the literature. We hypothesize that the co-occurrence of this rare pathologic entity is caused by an interrelated etiologic process, with the tumor microenvironment of the schwannoma inciting the development of the vascular malformation., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Olfactory schwannomas of the anterior cranial base (ACB) are rare tumors, and their association with hereditary hemorrhagic telangiectasia (HHT) has not previously been described. The majority of ACB schwannomas arise from the sinonasal tracts and may demonstrate intracranial extension. We report a case of an olfactory schwannoma-dense adherence to the basal frontal lobe. Complete tumor resection was performed through a unilateral extended endonasal endoscopic approach with preservation of the contralateral olfactory bulb. Anterior cranial base repair was achieved with the use of a mucoperichondrial vascularized pedicled nasoseptal flap., Clinical Presentation: A 25-year-old woman with a history of migraines presented with unilateral epistaxis and progressive worsening of her headache symptoms. The patient had a history of HHT. Nasal endoscopy showed mild telangiectasias, but no clear evidence of a mass. A computerized tomographic scan showed a large left-sided expansile lesion in the left ethmoid region with expansion and remodeling of the anterior cranial base and medial left orbit. Magnetic resonance imaging with contrast showed the mass to be avidly enhancing. Angiography was performed and demonstrated a mild vascular blush., Conclusions: We report a rare case of HTT and olfactory schwannoma completely resected with a unilateral extended endoscopic endonasal approach. Reconstruction was performed with the use of nasoseptal flap. This is the first reported single-stage fully endoscopic endonasal unilateral approach for resection of an olfactory schwannoma with preservation of the contralateral olfactory cleft. The patient's sense of smell and taste was maintained after surgery., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: Gamma knife radiosurgery (GKRS) has been established as an effective and safe treatment for intracranial schwannoma. However, serious complications can occur after GKRS, including hydrocephalus. The pathophysiology and risk factors of this disorder are not yet fully understood. The objective of the study was to assess potential risk factors for hydrocephalus after GKRS., Methods: We retrospectively reviewed the medical radiosurgical records of 244 patients who underwent GKRS to treat intracranial schwannoma. The following parameters were analyzed as potential risk factors for hydrocephalus after GKRS: age, sex, target volume, irradiation dose, prior tumor resection, treatment technique, and tumor enhancement pattern. The tumor enhancement pattern was divided into 2 groups: group A (homogeneous enhancement) and group B (heterogeneous or rim enhancement)., Results: Of the 244 patients, 14 of them (5.7%) developed communicating hydrocephalus. Communicating hydrocephalus occurred within 2 years after GKRS in most patients (92.8%). No significant association was observed between any of the parameters investigated and the development of hydrocephalus, with the exception of tumor enhancement pattern. Group B exhibited a statistically significant difference by univariate analysis (P = 0.002); this difference was also significant by multivariate analysis (P = 0.006)., Conclusion: Because hydrocephalus is curable, patients should be closely monitored for the development of this disorder after GKRS. In particular, patients with intracranial schwannomas with irregular enhancement patterns or cysts should be meticulously observed., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: To investigate the outcomes of jugular foramen schwannomas (JFSs) and to evaluate the risk factors for tumor recurrence and poor final outcomes., Methods: Between 1993 and 2013, 133 patients (68 female patients) with JFSs were surgically treated. Clinical charts were reviewed, and scheduled follow-up examinations were performed., Results: The average preoperative Karnofsky Performance Scale (KPS) score was 79.6. The JFSs were classified as follows: 65 cases, type A; 15 cases, type B; 5 cases, type C; and 48 cases, type D. Gross total resection was achieved in 107 (80.5%) patients. Transient and permanent morbidities affecting cranial nerves IX and X were 19.8% and 11.5%, respectively. After a mean follow-up duration of 108.0 months, 13 (9.9%) patients experienced recurrence. The most recent KPS scores averaged 83.7. Compared with the preoperative KPS score, the most recent KPS score was improved in 87 (65.4%) patients and stabilized in 29 (21.8%) patients. The presence of a solid tumor (hazard ratio [HR] = 5.815, P = 0.010), nontotal resection (HR = 4.613, P = 0.007), and pathologic mitoses (HR = 11.018, P < 0.001) were independent risk factors for tumor recurrence. Decreased preoperative KPS score (per 10 points) (odds ratio [OR] = 2.483, P = 0.027), a less soft tumor consistency (OR = 2.257, P = 0.039), and a solid tumor (OR = 3.755, P = 0.041) were risk factors for poor long-term outcomes., Conclusions: Quality of life and preservation of neurologic function are the goals of surgical treatment of JFSs. Favorable long-term surgical outcomes for JFSs can be achieved. Morbidity of cranial nerves IX and X is significant, and patients with nontotal resection or pathologic mitosis should be followed closely., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Facial nerve schwannomas are rare lesions that constitute only 0.8% of all intrapetrous mass lesions. The least frequent lesions are tumors originating in the greater petrosal nerve (GPN). We present a case of a GPN schwannoma with temporal lobe edema in which the patient was operated on using an extradural and intradural approach to prevent complications., Case Description: A 66-year-old woman with vertigo and abnormal magnetic resonance imaging findings was referred to our department. Computed tomography scan revealed an isodense subtemporal mass with partial rim calcification and petrosal bone apex erosion. Magnetic resonance imaging confirmed a 22-mm left middle fossa lesion with heterogeneous enhancement and edema of the temporal lobe. A left temporal craniotomy to the middle fossa was performed. The initial extradural exploration revealed the tumor to be in the Glasscock triangle, mainly involving the location of the GPN. The tumor was removed through an intradural approach in piecemeal fashion. Finally, using an extradural and intradural middle fossa approach, the tumor was totally removed, leaving the capsule on the middle fossa floor with continuous facial nerve monitoring. The postoperative course was uneventful without complications of xerophthalmia and facial palsy., Conclusions: GPN schwannomas are very rare lesions. The extradural and intradural middle fossa approach was used to preserve the tumor capsule around the GPN. Using this technique, one can safely protect the geniculate ganglion and the GPN., (Copyright © 2016 Elsevier Inc. All rights reserved.)

A 4-year-old castrated labrador retriever presented for cardiac evaluation to determine the etiology of cardiogenic pulmonary edema diagnosed 1 month prior. A large pedunculated mass involving the ventral aspect of the mural mitral valve leaflet and the endocardial surface of the left ventricular free wall, resulting in severe mitral regurgitation, was identified on echocardiogram. Histopathology and immunohistochemistry of this mass and other endocardial masses identified at necropsy for S-100 protein were consistent with a diagnosis of schwannoma. To the authors' knowledge, this is the first case of a benign intracardiac schwannoma described in the left heart of a dog., (Copyright © 2015 Elsevier B.V. All rights reserved.)