Primary intracranial pediatric ganglioneuroblastoma-report of two cases and review of an unusual masquerader.

Ganglioneuroblastoma (GNB) is a rare neurogenic tumor with a predilection for extracranial sites. Its primary intracranial occurrence is anecdotal, with less than ten cases reported in pediatric literature. We report two cases of this unusual entity. The first was a 1-year-old boy who presented with a progressive scalp swelling that radiologically appeared to be a mesenchymal tumor. The tumor was resected completely, and its histopathological examination was suggestive of a pigmented GNB. The clinico-radiological presentation and melanin pigmentation of the tumor were unique features of the case. The second case was a 7-year-old girl who presented with left hemiparesis and raised intracranial pressure due to a large right parietal intraparenchymal tumor that histologically proved to be a GNB. The child succumbed to disease progression a month after undergoing near-total resection of the tumor. Analysis of our cases in the light of a literature review reveals that pediatric intracranial GNBs have diverse clinico-radiological features and can easily be confused for commoner pathologies in both intra- and extra-axial locations.

A Novel Way for Efficient and Safe Posterior Fossa Relaxation: The Extradural Opening of the Cisterna Magna in Retrosigmoid Craniotomy.

Cerebrospinal fluid drainage is a common practice to provide brain relaxation during intradural surgery. In retrosigmoid approaches, cerebrospinal fluid can be drained from the cisterna magna to provide brain relaxation in the posterior fossa. To our knowledge, most techniques to achieve cerebrospinal fluid release concern intradural opening of the cisterns. We describe a novel way for the extradural opening of the cisterna magna in retrosigmoid surgery that avoids direct cerebellar contact. Patients elected for surgical treatment of cerebellopontine angle tumors are positioned supine with a roll under the ipsilateral shoulder and the head turned to the contralateral side. After performing a retrosigmoid craniotomy, the surgical microscope is tilted, and the foramen magnum is approached extradurally. A horizontal dural slit is made at the level of the cisterna magna, and cerebrospinal fluid is drained without having direct cerebellar contact. After brain relaxation, the intradural surgery can proceed as usual. This slight adaptation for a very common practice avoids the need for direct cerebellar retraction when approaching the cisterna magna to drain cerebrospinal fluid. It is a clean and easy step to perform, that we believe improves surgical efficacy and could potentially diminish cerebellar harm because it obviates the need for intradural opening of the cisterna magna.

Surgical management of a thoraco-lumbar extradural cyst in a pediatric patient with Klippel-Trenaunay syndrome: a case report.

Introduction and importance: Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by the abnormal development of blood vessels, soft tissues, bones, and the lymphatic system. The syndrome is rare, with few cases reported worldwide, especially those describing an association between KTS and spinal extradural meningeal cysts (SEMC). This report highlights a rare case of a pediatric patient with KTS who underwent successful surgical decompression of a thoraco-lumbar extradural cyst, highlighting the importance of reevaluating surgical interventions in KTS patients. Case presentation: A 15-year-old girl diagnosed with KTS 4 days postnatally, was referred to our clinic due to chronic back pain and spastic paraparesis. These symptoms were attributed to a compressive extradural thoraco-lumbar cyst. Diagnostic evaluations confirmed the presence of the cyst, leading to the decision to proceed with surgical intervention. Clinical discussion: The surgical approach involved a laminoplasty at T11-T12-L1, resulting in the total removal of the cystic lesion. The patient exhibited a complete resolution of symptoms postoperatively, with no significant complications reported during the surgery. Conclusion: With this case, the authors question the fear of surgical intervention in KTS patients, which is often avoided due to concerns of high-risk complications like excessive bleeding or poor wound healing, and hint at a possible association between KTS and extradural meningeal cysts.

Computed tomographic and magnetic resonance imaging features of canine cervical epidural dirofilariasis.

An 8-year-old neutered male Maltese dog presented with a month-long history of progressive nonambulatory tetraparesis. MRI revealed a well-defined, centrally nonenhanced, T1-weighted hypointense, extradural structure located in the vertebral canal at the level of the C5 vertebral body. CT demonstrated a hypoattenuating, space-occupying structure in the same area. Surgery revealed a long, narrow parasite in the epidural space. An adult Dirofilaria immitis was confirmed by multiplex polymerase chain reaction (PCR) assay. This is a novel report describing the MRI and CT features of cervical epidural dirofilariasis in a dog.

A separate-dural-incision method of extradural dumbbell spinal schwannoma resection: cumulative experience at a single center.

OBJECTIVE: To present our experience in the surgical management of completely extradural dumbbell spinal schwannomas with a new surgical strategy. METHOD: This study is a case series of patients treated at the Neurosurgery Department of the First Affiliated Hospital of USTC, between January 2018 and June 2021. RESULTS: 24 patients met the inclusion criteria, with cervical and lumbar spines being the most frequent locations. All patients underwent surgical treatment. Total gross resection was accomplished in all patients. Two cases had numbness and no case exhibited motor deficit. There was no postoperative CSF leakage or wound infection. CONCLUSION: Based on a limited number of observations, we conclude that our technique was feasible and effective for the treatment of extradural dumbbell spinal schwannomas. CLINICAL TRIAL: http://www.chictr.org.cn/ , No. ChiCTR2400086171.

Spinal extradural meningioma: Report of two cases.

Purely extradural spinal meningiomas are rare and often confused with metastases and schwannomas. Only a few cases have been described in the literature, and they are located mainly in the cervical and dorsal regions. We present two cases of extradural meningiomas, one well-defined nodular growth present at a cervicodorsal junction that was confused with schwannoma preoperatively and the other typical meningioma extending both intra and extradurally in the upper dorsal spine.

Cerebrospinal Fluid Leaks: Challenges in Localizing Spontaneous Spinal Leak Sites and Minimally Invasive Treatment.

The confirmation of cerebrospinal fluid (CSF) leaks in the setting of spontaneous intracranial hypotension (SIH) by imaging involves a growing toolset of multimodal advanced spinal and skull base imaging techniques, for which exists a unique set of challenges for each CSF leak type. Furthermore, the repertoire of minimally invasive CSF leak treatment beyond nontargeted epidural blood patch administration has grown widely, with varied practices across institutions. This review describes current diagnostic imaging and treatment modalities as they apply to the challenges of CSF leak localization and management.

Evaluation and Treatment of Lumbar Spine Extradural Cysts: A Narrative Review.

The main objective was to describe the different types and characteristics of lumbar spine extradural cysts and their optimal treatment options with a focus on endoscopic technique. We searched Pubmed, EMBASE, Medline, and Google Scholar for articles published between 1967 and 2020 using the keywords "Spinal Cyst," "Extradural Cyst," and "Lumbar Cyst." The various anatomical and histological types of the extradural cysts with their presentations, etiologies, imaging, and optimal treatment with a focus on endoscopic techniques were reviewed from the articles. Lumbar spinal cysts are relatively rare pathologies that might cause radicular symptoms similar to lumbar disc herniation. Spinal extradural cysts are classified either histologically based on the cyst lining tissues (synovial cysts or non-synovial, ganglion cysts) or anatomically based on the structure of origin (epidural cysts, ligamentum flavum cysts, discal cysts, post-discectomy pseudocysts, posterior longitudinal ligament cysts, facet cysts). Surgical excision is the recommended treatment of symptomatic cysts with endoscopic techniques being a viable option. Extradural lumbar cysts can be identified based on their histological structure or depending on their structure of origin. Regardless of their classification, they could all give similar clinical findings, and the optimal treatment would be surgical excision with endoscopic technique being a viable option with a satisfactory outcome.

A Rare Case of a Spontaneous Thoracic Epidural Hematoma in a Young Male Weightlifter.

Spontaneous spinal epidural hematoma (SSEH) is the accumulation of blood in the epidural space of the spinal cord. Acute SSEH is a rare phenomenon that presents with a wide variety of neurologic symptoms and most often is a surgical emergency. We present a previously healthy 34-year-old male with sudden onset progressive weakness and tingling in the right lower extremity that progressed to the left lower extremity while bench pressing weights, resulting in complete lower extremity paralysis. Magnetic resonance imaging (MRI) revealed a 3.0 cm extradural mass centered in the dorsal and left lateral canal. After a T1-T4 hemilaminectomy was performed which was followed by inpatient rehabilitation, the patient had a favorable outcome improving from The American Spinal Injury Association Impairment Scale (AIS) grade A, complete impairment, to AIS grade C, incomplete impairment on discharge. Initially, the patient had complete motor and sensory paralysis below the level of T4, and upon discharge, the patient was able to attain modified independence in activities of daily living, mobility, and transfer. Due to the lack of risk factors for SSEH in this patient, the etiology is most likely related to the Valsalva maneuver while weightlifting. Lesions in the thoracic region with rapid progression of neurologic symptoms are indicators of poor prognosis, so this case highlights the importance of prompt recognition and intervention for improved outcomes to prevent devastating neurologic defects.

Extradural giant thoracic schwannoma in a pediatric patient; a case report.

INTRODUCTION: Giant extradural thoracic schwannomas are very rare tumors in the pediatric age group and often occur together with neurofibromatosis. Giant schwannomas span across more than two vertebral segments and have an extraspinal extension of over 2.5 cm. In this case, we report on a 5-year-old boy with a purely extradural giant schwannoma without accompanying neurofibromatosis. CLINICAL PRESENTATION: A 5-year-old male patient was admitted to the orthopedics and traumatology outpatient clinic with complaints of difficulty in walking following waist and left leg pain after falling from a chair. Contrast-enhanced spinal MRI and cranial MRI showed an extradural spinal lesion measuring 22 × 18 × 35 mm that pushed the spinal cord to the right at the T10-12 level and extended into the left foramen at the T11-12 level. The patient was operated. The tumor was removed completely by performing bilateral laminoplasty at the T10-11-12 levels. Histopathology result reported schwannoma. CONCLUSION: Giant schwannomas are slow-growing tumors that rarely occur in childhood. In these patients, spinal traumas can lead to serious neurological deficits. Early diagnosis and successful surgery can prevent permanent neurological damage.

Surgical Management of Extradural Tumors at the Craniovertebral Junction - Insights from a Tertiary Care Center.

BACKGROUND: Craniovertebral junction (CVJ) tumors are challenging due to their unique anatomical location. This study aimed to evaluate the complexities in dealing with such precarious CVJ extradural lesions over the decade. METHODS: Twenty-seven patients of extradural CVJ tumors operated between 2009 and 2018 were included. The demographic details, neurological status, surgical approach, extent of resection, type of fixation, complications, and outcome at final follow-up were recorded for each patient. RESULTS: The mean age of the patients was 39.5 ± 20 years. Most (17/27) of the patients had involvement of a single level. Clivus was the most common (9/17) involved region followed by atlas (7/17) vertebrae. Majority of the patients (13/27) were operated through the posterior-only approach. About 15 patients (55.5%) had instability or extensive lesions that necessitated posterior fixation. None of the patients underwent anterior fixation. Gross and near total excision were achieved in 10 patients (37%) and 3 patients (11%) respectively while 14 patients underwent subtotal excision of tumor. On histopathological analysis, clival chordoma (8/27) was found to be the most common pathology followed by giant cell tumor (6/27), plasmacytoma (4/27), and multiple myeloma (2/27). Most patients (13 out of 27) had the same neurological status after the surgery. Six patients (22%) improved post-operatively with decreased weakness and spasticity. Thirteen (48%) patients underwent adjuvant radiotherapy. CONCLUSIONS: This retrospective study provides valuable insights into managing extradural CVJ tumors and highlights the importance of individualized approaches for optimal outcome.

Obstacles to receiving postoperative radiation therapy following separation surgery for metastatic spine disease.

OBJECTIVE: Obtaining timely postoperative radiotherapy (RT) following separation surgery is critical to avoid local recurrence of disease yet can be a challenge due to scheduling conflicts, insurance denials, and travel arrangements. In patients undergoing metastatic spine surgery for spinal cord compression, the authors sought to: 1) report the rate of postoperative RT, 2) describe reasons for patients not receiving postoperative RT, and 3) investigate factors that may predict whether a patient receives postoperative RT. METHODS: A single-center retrospective case series was undertaken of all patients who underwent metastatic spine surgery for extradural disease between January 2010 and January 2021. Inclusion criteria were patients with intermediate or radioresistant tumors with evidence of spinal cord compression who underwent surgery. The primary outcome was the occurrence of RT within 3 months following surgery. Multivariable logistic regression analysis was performed controlling for age, BMI, race, total number of decompressed levels, tumor size, other organ metastasis, and preoperative RT or chemotherapy to predict patients receiving postoperative RT. RESULTS: Of 239 patients undergoing spine surgery for metastatic disease, 113 (47.3%) received postoperative RT while 126 (52.7%) did not. In the postoperative RT group, 24 (21.2%) received stereotactic body radiation therapy while 89 (78.8%) received conventional external-beam radiation therapy. The most common reasons for patients not receiving postoperative RT included death or transfer to hospice (31.0%), RT not being recommended by radiation oncology (30.2%), and loss to follow-up (23.8%). On critical review with the radiation oncology department, the authors estimated that 101 of 126 (80.2%) patients who did not receive postoperative RT were potential candidates for postoperative RT. Patients who received postoperative RT had more documented inpatient (48.7% vs 32.5%, p < 0.001) and outpatient (100.0% vs 65.1%, p < 0.001) radiation oncology consultations than those who did not. Additionally, patients who received postoperative RT had a higher rate of postoperative chemotherapy (53.1% vs 25.4%, p < 0.001), while patients who did not receive postoperative RT had a higher rate of preoperative RT (7.1% vs 31.0%, p < 0.001). Multivariable analysis confirmed that patients who received preoperative RT had lower odds of undergoing postoperative RT (OR 0.14, 95% CI 0.06-0.34; p < 0.001), and patients who underwent postoperative chemotherapy had higher odds of undergoing postoperative RT (OR 3.83, 95% CI 2.05-7.17; p < 0.001). CONCLUSIONS: In the current study reflecting real-world care of patients with metastatic spine disease after undergoing separation surgery, 47% of patients did not receive postoperative RT, and 80% of those patients were potential candidates for postoperative RT. Radiation oncology consultation and postoperative chemotherapy were significantly associated with receiving postoperative RT, whereas preoperative RT was significantly associated with not receiving postoperative RT. The lack of timely postoperative RT highlights a potential gap in metastatic spine tumor care and underscores the necessity for prompt radiation oncology consultation and effective planning.

Successful detection of multiple communicating holes in multiple spinal extradural arachnoid cysts by using time-spatial labeling inversion pulse magnetic resonance imaging: illustrative case.

BACKGROUND: Spinal extradural arachnoid cysts (SEACs) communicate with the subarachnoid space through small communicating dural holes. The precise preoperative detection of all communicating holes, followed by minimally invasive dural closure, is the ideal treatment to prevent postoperative spinal deformities, especially in cases of multiple SEACs. However, standard imaging methods often fail to detect communicating hole locations. Although a few cases of successful single-hole detection via cinematic magnetic resonance imaging (MRI) have been reported, this modality's ability to detect multiple holes has not been demonstrated. OBSERVATIONS: The authors describe the case of a 14-year-old male with myelopathy due to multiple SEACs at T5-8 and T8-12. Myelography revealed a complete block at the T8 level; no cephalic cyst or communicating holes were identified. Time-spatial labeling inversion pulse (T-SLIP) MRI revealed cerebrospinal fluid flow into the cyst at T10 and T7. A limited laminectomy or hemilaminectomy was performed at T7 and T10, and two dural holes were closed without a total cystectomy. The patient's gait disturbance and rectal disorder disappeared. The cysts were confirmed to have completely disappeared on conventional MRI at 1 year postoperatively. LESSONS: T-SLIP MRI, a cinematic MRI, is useful for detecting multiple communicating holes in SEACs.

Determination of the Efficiency of Magnetic Resonance Imaging in the Evaluation of Compressive Myelopathy.

Background The phrase "compressive myelopathy" refers to compression of the spinal cord, either internally or externally. This compression might arise from various sources such as a herniated disc, post-traumatic compression, and epidural abscess as well as epidural or intradural neoplasms. Magnetic resonance imaging (MRI) plays a crucial role in differentiating between compressive and non-compressive myelopathy. After eliminating compressive lesions, attention is directed toward intrinsic cord-related causes of acute myelopathy including vascular, infectious, and inflammatory pathologies. Aims The study aimed to assess different etiologies of compressive myelopathy, analyze the MRI features of spinal cord compressive lesions, classify the lesions depending on site, and correlate MRI findings with intraoperative findings and histopathology in operated cases. Material & methods A total of 50 patients, who exhibited clinical symptoms indicative of compressive myelopathy sent to the Radiology department, Rangaraya Medical College (RMC), Kakinada for MRI spine were included in the study. It's an observational cross-sectional study. Statistical Package for Social Sciences (SPSS) version 22.0 (IBM Corp., Armonk, USA) was used for statistical calculations. Result Among the 50 cases of compressive myelopathy, the etiologies are distributed as follows: trauma (22 cases), infection (12 cases), primary neoplasm (eight cases), and secondary neoplasm (eight cases); extradural compressive lesions (84%) and Intradural-extramedullary lesions (16%). Conclusion Utilizing MRI successfully assessed the spinal cord integrity and characterized spinal tumors. Consequently, the study concludes that MRI is a highly definitive, sensitive, and accurate tool for evaluating compressive myelopathy.

Traumatic injury to the posterior fossa: a secondary analysis and description of case series from the NEXUS head injury dataset.

Background: Traumatic brain injuries involving the posterior fossa are rare and case reports indicate they often result in severe outcomes. We seek to describe characteristics and outcomes of traumatic posterior fossa injuries. Methods: We performed a planned secondary analysis of all patients with posterior fossa injuries enrolled in the NEXUS head computed tomography (CT) validation study dataset. The dataset includes prospectively collected data on all patients undergoing non-contrast cranial CT following blunt traumatic head injury from April 2006 to December 2015, at four emergency departments comprising community and university sites, as well as urban, suburban and rural settings in California (Antelope Valley Hospital, San Francisco General Hospital, UCLA Ronald Reagan Medical Center, UCSF Fresno Community Regional Medical Center). We classified each patient into one of three injury patterns: Type I-notable traumatic injuries primarily above the tentorium, with minimal posterior fossa involvement; Type II-notable traumatic injuries both above and within the posterior fossa; and Type III-notable traumatic injuries primarily within the posterior fossa. We extracted demographic data for each patient as well as physician assessments of the NEXUS head CT and Canadian Head CT rule clinical criteria, mechanisms of injury, patient outcomes, and the location and types of intracranial injuries sustained. Findings: Of 11,770 patients in the database, 184 (1.6%) had posterior fossa injuries on CT imaging. Mean age was 55.4 years (standard deviation 22.5 years, range 2-96 years); 131 (71.2%) were males. We identified 63 patients with Type I injuries, 87 with Type II injuries, and 34 Type III injuries. The most common mechanisms of injury were falls (41%), pedestrian vs automobile (15%), and motor vehicle collisions (13%). On presentation most patients had altered mental status (72%), abnormal behavior (53%), or a neurologic deficit (55%). The majority of individuals, 151 (82%), had clinically important injuries and 111 (60%) required neurosurgical intervention. The dispositions for the subjects included 52 deaths (28%), 49 (27%) patients discharged home, and 48 (26%) discharged to rehabilitation facilities. When compared to individuals with Type I and Type II injuries, patients with Type III injuries had lower mortality (6% vs 30% and 35%) and higher percentage of patients discharged home (60% vs 19% and 21%). Interpretation: Patients with Type I and II injury patterns (those that involve both the posterior fossa and supratentorium) experienced high mortality and disability. Patients with Type III injuries (isolated posterior fossa) had a better prognosis. Funding: None.

Earlier Radiation Is Associated with Improved 1-Year Survival After Metastatic Spine Tumor Surgery.

OBJECTIVE: In patients undergoing metastatic spine surgery, we sought to 1) report time to postoperative radiation therapy (RT), 2) describe the predictive factors of time to postoperative RT, and 3) determine if earlier postoperative RT is associated with improved local recurrence (LR) and overall survival (OS). METHODS: A single-center retrospective cohort study was undertaken of all patients undergoing spine surgery for extradural metastatic disease and receiving RT within 3 months postoperatively between January 2010 and January 2021. Time to postoperative RT was dichotomized at <1 month versus 1-3 months. The primary outcomes were LR, OS, and 1-year survival. Secondary outcomes were wound complication, Karnofsky Performance Status, and modified McCormick Scale (MMS) score. Regression analyses controlled for age, body mass index, tumor size, preoperative RT, preoperative/postoperative chemotherapy, and type of RT. RESULTS: Of 76 patients undergoing spinal metastasis surgery and receiving postoperative RT within 3 months, 34 (44.7%) received RT within 1 month and 42 (55.2%) within 1-3 months. Patients with larger tumor size (ß = -3.58; 95% confidence interval [CI], -6.59 to -0.57; P = 0.021) or new neurologic deficits (ß = -16.21; 95% CI, -32.21 to -0.210; P = 0.047) had a shorter time to RT. No significant association was found between time to RT and LR or OS on multivariable logistic/Cox regression. However, patients who received RT between 1 and 3 months had a lower odds of 1-year survival compared with those receiving RT within 1 month (odds ratio, 0.18; 95% CI, 0.04-0.74; P = 0.022). Receiving RT within 1 month versus 1-3 months was not associated with wound complications (7.1% vs. 2.9%; P = 0.556) (odds ratio, 4.40; 95% CI, 0.40-118.0; P = 0.266) or Karnofsky Performance Status/modified McCormick Scale score. CONCLUSIONS: Spine surgeons, oncologists, and radiation oncologists should make every effort to start RT within 1 month to improve 1-year survival after metastatic spine tumor surgery.

Selective Vestibular Neurectomy through the Presigmoid Retrolabyrinthine Approach in the Treatment of Meniere's Disease.

BACKGROUND: Meniere's disease (MD) is a disabling disease, especially in patients who are refractory to medical therapy. Moreover, selective vestibular neurectomy (VN), in these selected cases, can be considered a surgical alternative which preserves hearing function and facial nerve. METHODS: We retrospectively studied 23 patients with MD diagnosis and history of failed extradural endolymphatic sac surgery (ELSS) who underwent combined micro-endoscopic selective VN, between January 2019 and August 2023, via a presigmoid retrolabyrinthine approach. All patients were stratified according to clinical features, assessing preoperative and postoperative hearing levels and quality of life. RESULTS: At the maximum present follow-up of 2 years, this procedure is characterized by a low rate of complications and about 90% vertigo control after surgery. No definitive facial palsy or hearing loss was described in this series. One patient required reintervention for a CSF fistula. Statistically significant (p = 0.001) difference was found between the preoperative and the postoperative performance in terms of physical, functional, and emotive scales assessed via the DHI questionnaire. CONCLUSIONS: Selective VN via a presigmoid retrolabyrinthine approach is a safe procedure for intractable vertigo associated with MD, when residual hearing function still exists. The use of the endoscope and intraoperative neuromonitoring guaranteed a precise result, saving the cochlear fibers and facial nerve. The approach for VN is a familiar procedure to the otolaryngologist, as is lateral skull base anatomy to the neurosurgeon; therefore, the best results are obtained with multidisciplinary teamwork.

The trans-sylvian trans-petrosal "half & half" approach-a how I do it.

BACKGROUND: Petroclival meningiomas are one of the most challenging tumors to be operated in the realm of neurosurgery. Many approaches have been developed over the years. METHOD: The authors describe the Half & Half (H&H) approach whose main indication is petroclival meningiomas with suprasellar extension. The part of the tumor located above CN III and in the retrochiasmatic space is addressed through a trans-sylvian, while the petroclival portion is through an extradural anterior petrosectomy approach. The wide surgical corridor given by this approach allows extensive tumor resection while avoiding the risk associated with the manipulation of intracavernous neurovascular structures. CONCLUSION: The H&H approach is an effective strategy to maximize the safe resection of petroclival meningiomas.

Primary intramedullary extradural Ewing sarcoma.

Ewing sarcoma is the second most frequent primary bone tumour of childhood and adolescence. The aim of this report is to describe the imaging, pathology, clinical findings, and treatment of a primary intradural extramedullary Ewing sarcoma with a unique intracranial metastatic component in a pediatric patient. A 14-year-old girl with a history of mood disorders presented to the emergency department with a 3-week history of neck torticollis, cervical pain, paresis, and paresthesia of the upper and lower extremities on the left side. Initially, non-organic causes such as somatization or conversion disorder were suspected. She returned 3 months later when her symptoms worsened. MRI of the head and spine was performed, and demonstrated the presence of a suprasellar, retro-chiasmatic mass lesion. There was also diffuse leptomeningeal enhancement, another well-defined intradural extramedullary lesion the sacral region and several multifocal cauda equina soft tissue nodules. The patient first underwent surgery. The patient was also treated with a combination of chemotherapy (vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide (VDC/IE)) and radiation as per the Children's Oncology Group AEWS1221 protocol. Most recent imaging conducted 22 months after the initial mass discovery revealed improvement of the suprasellar mass lesion with residual stable appearance of the prominence and enhancement of the pituitary stalk and tuber cinereum. There was interval improvement of the spinal lesions with no convincing residual. Clinically, at almost three years since initial imaging findings, and 25 months since completing treatment, she is stable from an oncology perspective.

Case report of a primary ectopic extradural and extraspinal meningioma of the brachial plexus.

Primary ectopic extradural and extraspinal meningiomas are rare. We present a unique case of this type of meningioma in the brachial plexus. A 25-year-old man consulted us because of neuropathic supraclavicular pain and the appearance of a supraclavicular mass whose volume had increased. Clinical examination found paresis of the deltoid, biceps brachii and brachialis muscles rated as M4 (MRC) and a strong Tinel sign at the supraclavicular fossa, over the palpable mass. There was no sign pointing towards central nervous system involvement or altered general condition. MRI revealed a mass measuring 53 × 24 mm invading the C5-C6 plexus roots and the primary upper trunk, but not the bone or spinal area. This lesion was hyperintense on DWI/ADC, hyperintense on T2 with hypointense spots, and hypointense on T1 with intense heterogeneous gadolinium enhancement. Excisional biopsy was done 6 months after symptoms started. The tumor had developed at the C5 root, which was fibrous and at the C6 root, which was grossly normal. Anatomical pathology confirmed the WHO grade 1 meningioma, meningothelial and psammomatous histological subtypes. At 6 months, a follow-up MRI found no postoperative tumor remnants or recurrence. During the postoperative course, persistent paralysis of the deltoid muscle at 5 months justified a nerve transfer. This is a rare case of ectopic extraspinal and extradural meningioma of the brachial plexus. The diagnosis of an ectopic meningioma must be considered when a patient presents with a brachial plexus tumor causing neurological deficits. The extradural nature is not sufficient to rule out this diagnosis.