Intradural Invasion Research Articles

A Rare Case of Sacrococcygeal Teratoma With Medullar Invasion

Background and Importance: Sacrococcygeal Teratoma (SCT) with medular invasion is rare. Case Presentation: We report a case of an 11-month male infant, with no prenatal history of any abnormality, presenting since birth, a mass in the buttock extended to retrorectum associated with a right side hypotonic limb and monoplegia. Medullar and abdominal pelvic Magnetic Resonance Imaging (MRI) showed a sacrococcygeal tissue mass and intradural lumbosacral invasion with a high level of alpha-fetoprotein. Surgical excision was done successfully with an uneventful follow-up. Conclusion: Despite of the intradural invasion, those SCT are in major cases mature and present low risk of malignancy or recurrence.

Radiation Disrupts the Protective Function of the Spinal Meninges in a Mouse Model of Tumor-induced Spinal Cord Compression.

Radiation Disrupts the Protective Function of the Spinal Meninges in a Mouse Model of Tumor-induced Spinal Cord Compression.

Prognostic impact of the \u201cSekhar grading system for cranial Chordomas\u201d in patients treated with pencil beam scanning proton therapy: an institutional analysis

BackgroundSkull base chordomas are rare and heterogeneously behaving tumors. Though still classified as benign they can grow rapidly, are locally aggressive, and have the potential to metastasize. To adapt the treatment to the specific needs of patients at higher risk of recurrence, a pre-proton therapy prognostic grading system would be useful. The aim of this retrospective analysis is to assess prognostic factors and the “Sekhar Grading System for Cranial Chordomas” (SGSCC) by evaluating the larger cohort of patients treated at our institution as to determine its reproducibility and ultimately to ensure more risk adapted local treatments for these challenging tumors.MethodsWe analyzed 142 patients treated for skull base chordomas between 2004 and 2016. We focused the analysis on the 5 criteria proposed for the SGSCC (tumor size, number of anatomic regions and vessels involved, intradural invasion, as well as recurrence after prior treatment) and classified our patients according to their score (based on the above mentioned criteria) into three prognostic groups, low-risk, intermediate-risk and high-risk. The three groups were then analyzed in regards of local control, local recurrence-free survival and overall survival.ResultsThe median follow up was 52 months (range, 3–152). We observed 34 (24%) patients with a local recurrence, resulting in a local control of 75% at 5 years. Overall survival was 83% at 5 years, 12 (9%) patients had died due to local progression. When split into the three prognostic groups according to the SGSCC the observed local control was 90, 72 and 64% (p = 0.07) in the low-, intermediate- and high-risk group, respectively. A similar correlation was observed for local recurrence-free survival with 93, 89 and 66% (p = 0.05) and for overall survival with 89, 83 and 76% (p = 0.65) for the same prognostic groups.ConclusionsAfter splitting our patient cohort into the three SGSCC risk groups, we found a trend towards better outcome for those patients with lower as opposed to higher scores. These results suggest that this prognostic grading system published by Sekhar et al. could be integrated in the management decision-tree for patients with skull base chordoma.

Skull Base Invasion Patterns of Malignant Head and Neck Tumors: A Neurosurgical Perspective

Objective Craniofacial resection (CFR) and temporal bone resection (TBR) on malignant head and neck tumors (MHNTs) invading skull base require accurate and precise determination of the tumor invasion. We investigated tumor skull base invasion patterns and surgical results in CFR and TBR cases. Methods We performed either CFR or TBR for 75 selected patients with the possibility of en bloc resection over the period between 2011 and 2018. The medical charts of the selected patients were reviewed. Results Primary tumor onset site (TOS) groups were: (1) nasal cavity/ethmoid sinus, 20 cases; (2) orbit, 10 cases; (3) maxillary sinus, 28 cases; and (4) external ear/temporomandibular joint, 17 cases. Grades for tumor invasion depth (TID) included: (I) extracranial invasion and skull base bone invasion; (II) extradural invasion; or (III) intradural invasion. Patients in groups 1 and 2 had a significantly higher frequency of grade II and III invasions than patients in groups 3 and 4. The main invasion site was nasal cavity superior wall and ethmoid sinus superior wall for group 1 tumors, orbit superior wall, and lateral skull base sphenoid bone for group 2 and 3 tumors, and lateral skull base temporal bone for group 4 tumors. Positive resection margins represented a significant negative prognostic factor. TID and TOS did not affect skull base margin status. Conclusion Skull base invasion of MHNTs exhibits certain fixed patterns in sites susceptible to invasion based on the TOS. The frequencies of extradural and intradural invasions differed, indicating the importance for accurate preoperative tumor evaluation.

C2 neurofibromas in neurofibromatosis type 1: genetic and imaging characteristics.

OBJECTIVEC2 nerve root neurofibromas have been reported frequently in patients with neurofibromatosis type 1 (NF1), although their genetic and imaging characteristics are unexplored. The aim of this study was to characterize genetic and spinal imaging findings in a large cohort of NF1 patients with C2 neurofibromas.METHODSThe authors performed a review of national NF1 referrals between 2009 and 2016. Inclusion criteria were at least 1 C2 root neurofibroma and cervical-spine or whole-spine MRI scans available for analysis. Blinded imaging review was performed by a neuroradiologist with an interest in NF1.RESULTSFifty-four patients with 106 C2 neurofibromas were included. The median age was 32.5 years (range 15-61 years), and there were slightly more male patients (33 vs 21 female patients). Splice-site (30%) and missense (20%) variants were frequent. Spinal neurofibromas were distributed in all spine regions (65%) or in the cervical spine alone (22%). Most (93%) C2 neurofibromas were visible on MRI scans of the head. Intradural invasion and cord compression in the cervical spine included the C2 level in 95% and 80% of patients, respectively. Compared with all other cervical spine neurofibromas in these patients, C2 neurofibromas had higher rates of intraspinal extension (75% vs 32%; OR 6.20, 95% CI 3.85-9.97; p < 0.001), intradural invasion (53% vs 26%; OR 3.20, 95% CI 2.08-4.92; p < 0.001), and cord compression (25% vs 13%; OR 2.26, 95% CI 1.35-3.79; p = 0.002). However, C2 neurofibromas had lower rates of extraforaminal growth beyond the transverse process (12% vs 62%; OR 0.09, 95% CI 0.05-0.16; p < 0.001).CONCLUSIONSC2 neurofibromas are associated with an aggressive intraspinal phenotype, limited growth outside the spinal canal, and an uncommon genetic profile. These observations require future study.

Extended endoscopic endonasal surgery for clival chordoma and chondrosarcoma: Our experience in 14 cases

ObjectiveTo report our experience in the management of chordoma and chondrosarcoma with extended endoscopic endonasal surgery. MethodWe performed a retrospective analysis of a series of 14 patients with clival chordoma or chondrosarcoma who had extended endoscopic endonasal surgery from 2008 to 2016 performed by the same multidisciplinary team. ResultsWe had fourteen patients (male/female 2:1), with a mean age of 49 years for chordoma and 32 for chondrosarcoma. The most common clinical presentation was diplopia in 78.5% of cases, followed by dysphagia in 28.6%. Histologically, 71.4% were chordomas and 28.6% were chondrosarcomas. In addition, invasion of at least two-thirds or more of the clivus was found in 81% of the cases; in 57.1% there was intradural invasion, and in 35.7% invasion of the sella turcica. In 42.8% of cases, the degree of resection was total and in 21.5% subtotal. The most common complication was CSF fistula, occurring in 28.6% of the cases, with only one case requiring surgery to repair it. Adjuvant treatment with Proton Beam was performed in 35.7% of cases and with conventional radiotherapy in 21.5%. Mean follow-up was 53.5 months and tumour recurrence or progression was found in 21.5% of the cases, two of which had not received adjuvant treatment. There were no deaths. ConclusionThe extended endoscopic endonasal approach (EEEA) performed by an experienced team is a good alternative for the management of these lesions. Intradural invasion may be related to an increased risk of complications and worse clinical presentation, in addition to a lower rate of total resection.

Open Approaches to the Anterior Skull Base in Children: Review of the Literature.

Introduction Skull base lesions in children and adolescents are rare, and comprise only 5.6% of all skull base surgery. Anterior skull base lesions dominate, averaging slightly more than 50% of the cases. Until recently, surgery of the anterior skull base was dominated by open procedures and endoscopic skull base surgery was reserved for benign pathologies. Endoscopic skull base surgery is gradually gaining popularity. In spite of that, open skull base surgery is still considered the "gold standard" for the treatment of anterior skull base lesions, and it is the preferred approach in selected cases. Objective This article reviews current concepts and open approaches to the anterior skull base in children in the era of endoscopic surgery. Materials and Methods Comprehensive literature review. Results Extensive intracranial-intradural invasion, extensive orbital invasion, encasement of the optic nerve or the internal carotid artery, lateral supraorbital dural involvement and involvement of the anterior table of the frontal sinus or lateral portion of the frontal sinus precludes endoscopic surgery, and mandates open skull base surgery. The open approaches which are used most frequently for surgical resection of anterior skull base tumors are the transfacial/transmaxillary, subcranial, and subfrontal approaches. Reconstruction of anterior skull base defects is discussed in a separate article in this supplement. Discussion Although endoscopic skull base surgery in children is gaining popularity in developed countries, in many cases open surgery is still required. In addition, in developing countries, which accounts for more than 80% of the world's population, limited access to expensive equipment precludes the use of endoscopic surgery. Several open surgical approaches are still employed to resect anterior skull base lesions in the pediatric population. With this large armamentarium of surgical approaches, tailoring the most suitable approach to a specific lesion in regard to its nature, location, and extent is of utmost importance.

Imaging Appearances and Pathologic Characteristics of Spinal Epidural Meningioma.

Spinal epidural meningioma is an uncommon tumor. This study aimed to analyze the imaging and pathologic characteristics of this rare tumor. Fourteen confirmed cases of epidural meningioma were retrospectively reviewed, and imaging characteristics and pathologic findings were analyzed to identify the typical features. The mean age of the patients (4 men, 10 women) was 44.9 years. Twelve tumors were in the cervical spinal canal, and 2, in the thoracic spinal canal. There were 9 en plaque meningiomas, 4 dumbbell-shaped meningiomas, and 1 fusiform/ovoid meningioma. The epidural meningiomas extended over 2-5 spinal segments (mean, 3.2 spinal segments). A soft epidural mass was seen in 12/14 (86%) patients. Dural calcification was seen in 8/14 (57%) tumors. Tumor caused intervertebral foramen enlargement in 10/14 (71%) patients and adhered to the nerve roots in 11/14 (79%) patients. Intradural invasion was seen in 8/14 (57%) patients. The dural tail sign was present in 13/14 (93%) tumors on contrast-enhanced T1WI. Regarding pathologic type, 10 of 14 (71%) were psammomatous, 2 of 14 (14%) were meningothelial, 1 of 14 (7%) was angiomatous, and 1 of 14 (7%) was transitional. During follow-up (mean follow-up, 73.4 months; range, 4-192 months), 7 patients had recurrence. Recurrences were between 4 and 192 months after the operation. Epidural meningioma has 3 different growth patterns. Dural thickening, calcification, invasion, and epidural mass formation are characteristic features of epidural meningioma. Regular follow-up imaging is required to detect recurrence.

353 C2 Segmental Neurofibromas in Patients with Neurofibromatosis Type 1

Abstract INTRODUCTION Segmental neurofibromas affecting the C2 nerve roots in patients with neurofibromatosis type 1 (NF1) can be particularly aggressive, though their clinical course and imaging characteristics are unexplored. The aim of this study was to present clinical and radiological outcomes of C2 neurofibromas in patients referred to a supra-regional NF1 centre. METHODS Imaging review of regional NF1 referrals 2009–2016. Inclusion criteria: (1) diagnosis of NF1; (2) at least one C2 root neurofibroma; (3) magnetic resonance imaging of the C-spine or whole spine. Multivariate logistic regression analysis was used to identify factors associated with need for surgery. RESULTS &gt;54 patients with 106 neurofibromas were included. The median age was 32.5 years (range 15–61 years) and there was a slight male excess (M: F, 33:21). 32% had myelopathy. Neurofibromas were distributed in all spine regions (65%) or the cervical spine alone (22%). Intradural invasion and cord compression in the cervical spine included the C2 level in 95% and 80% of patients, respectively. Compared to all other cervical spine neurofibromas, C2 neurofibromas had higher rates of intraspinal extension (75% vs. 33%; OR = 6.03, 95% CI 3.75 - 9.71; P &lt; 0.001), intradural invasion (53% vs. 26%; OR = 3.13, 95% CI 2.04 - 4.82; P &lt; 0.001) and cord compression (25% vs. 13%; OR = 2.20, 95% CI 1.32 - 3.69; P = 0.003). However, C2 neurofibromas had lower rates of extraforaminal growth beyond the transverse process (12% vs. 63%; OR = 0.08, 95% CI 0.04 - 0.15; P &lt; 0.001). 13% of patients underwent surgery to decompress the C2 level. Factors associated with surgery included myelopathy (P = 0.03) but not radiological cord compression (p &gt; 0.99). CONCLUSION C2 neurofibromas are particularly aggressive tumours due to preferential intraspinal growth.

Advanced esthesioneuroblastoma with hyperostosis of the anterior skull base

Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare type of neuroectodermal tumor arising from the superior portion of the nasal cavity, sometimes locally invading the anterior skull base. We report the case of a 44-year-old man having a 3-year history of nasal obstruction and anosmia, acute worsening of vision in his right eye, and facial numbness on the right side. Brain computed tomography and magnetic resonance imaging revealed an extensively enhanced soft tissue tumor involving bilateral nasal and orbital cavities, ethmoid sinuses, the floor of the anterior cranial fossa, and intradural invasion. In particular, prominent hyperostosis, a rare associated radiological finding, was observed on both sides of the orbital roofs. Bifrontobasal craniotomy involving a radical removal of the soft tissue mass, including the intradural extension, was performed; the bilateral hyperostosis of the orbital roofs was drilled off for decompression of the narrowed orbital cavities. Pathological reports confirmed esthesioneuroblastoma. The postoperative course was smooth, and the vision improved. The tumor did not recur for 1 year after postoperative radiotherapy and adjuvant chemotherapy.

Clinicopathological significance of p16, cyclin D1, Rb and MIB-1 levels in skull base chordoma and chondrosarcoma.

ObjectiveTo investigate the expression of p16, cyclin D1, retinoblastoma tumor suppressor protein (Rb) and MIB-1 in skull base chordoma and chondrosarcoma tissues, and to determine the clinicopathological significance of the above indexes in these diseases.MethodsA total of 100 skull base chordoma, 30 chondrosarcoma, and 20 normal cartilage tissue samples were analyzed by immunohistochemistry. The expression levels of p16, cyclinD1, Rb and MIB-1 proteins were assessed for potential correlation with the clinicopathological features.ResultsAs compared to normal cartilage specimen (control), there was decreased expression of p16, and increased expression of cyclin D1, Rb and MIB-1 proteins, in both skull base chordoma and chondrosarcoma specimens. MIB-1 LI levels were significantly increased in skull base chordoma specimens with negative expression of p16, and positive expression of cyclin D1 and Rb (P < 0.05). Significantly elevated MIB-1 LI was also detected in skull base chondrosarcoma tissues, while there was negative expression of p16, cyclin D1 and Rb (P < 0.05). In skull base chordoma, p16 negatively correlated with cyclin D1 and Rb, while cyclin D1 positively correlated with Rb. Additionally, p16, cyclin D1, Rb, or MIB-1 expression showed no correlation with age, gender, or pathological classification of patients with skull base chordoma (P > 0.05). However, p16 and MIB-1 levels correlated with the intradural invasion, and expression of p16, Rb and MIB-1 correlated with the number of tumor foci (P < 0.05). Further, the expression of p16 and MIB-1 appeared to correlate with the prognosis of patients with skull base chordoma.ConclusionsThe abnormal expression of p16, cyclin D1 and Rb proteins might be associated with the tumorigenesis of skull base chordoma and chondrosarcoma.

Intradural invasion of papillary thyroid carcinoma from C8 nerve root: a case report

Intradural invasion of papillary thyroid carcinoma from C8 nerve root: a case report

Endoscopic Endonasal Removal of a Large Petrous-Apex Epidermoid Tumor With Clival Involvement and Intradural Invasion

: Introduction: Epidermoid tumors comprise 1% of intracranial tumors. Although reported, intraosseous epidermoid tumors are even more rare. Cystic lesions of the petrous apex are uncommon and surgically challenging; the most rare pathology is presumed to be epidermoid.\r\nCase Presentation: This is a case of a 61-year-old woman with a large skull-base tumor extending inferiorly from the C1-C2 articulation and superiorly to the tuberculum sella. The lesion replaced the left-sided petrous apex, and the lateral extension of the tumor reached the styloid process. The tumor displayed intradural invasion medial to the internal auditory meatus, producing an intra-axial mass at the level of the upper pons. The patient presented with a 4-month history of headache and total unilateral deafness. Under image guidance, an endoscopic endonasal approach was used to totally resect the tumor. By following the tumor’s dural defect, the intra-axial part of the tumor was safely resected, and the dural defect was successfully repaired. \r\nConclusions: Image-guided endoscopic endonasal surgery is a versatile approach that can safely and easily address a large epidermoid tumor in this challenging region, obviating the need for demanding and sophisticated transcranial surgery.

Benign sacrococcygeal teratoma with spinal canal invasion and paraplegia

Sacrococcygeal teratomas can have a wide variety of clinical presentations depending on their size, vascularity, and degree of mass effect upon adjacent structures. Intradural invasion of a sacrococcygeal teratoma is a rare variant that has generally been associated with a favorable neurologic outcome. In this report, we present the case of a neonate with paraplegia secondary to a large Altman type III sacrococcygeal teratoma with extension into the spinal canal. The neoplasm was completely removed using a combined anterior and posterior approach after preoperative embolization of the lateral sacral arteries. Pathology showed a mature teratoma. The patient remains paraplegic without evidence of tumor recurrence at 1 year of age.

A rare case of oncocytic Schneiderian papilloma with intradural and intraorbital extension with notes of operative techniques

Epithelial cells of cylindrical cell papilloma are oncocytes, which arise from the sinonasal respiratory epithelium, hence the term Oncocytic Schneiderian papilloma.This is a rare and benign neoplasm of the nose and paranasal sinuses and it should be considered in the work-up of all unilateral nasal polypoid lesions. Clinically behaviour is comparable to inverted papillomas for local recurrence and malignancy coexistence. We report a case arisen from the nasoethmoidal space that extended to the anterior skull base through a bone dehiscence with intradural invasion and orbital space involvement. Surgical therapy is the treatment of choice, the endonasal endoscopic approach can be used in most of the cases and this surgical technique is safe and suitable also in presence of an extra nasal extension. We describe our experience for management of this kind of lesions and some notes on our operative technique.

Frontoethmoidal Osteomas Complicated by Intracranial Mucocele and Pneumocephalus: Report of Two Cases

Introduction: Paranasal sinuses osteomas are benign slow-growing tumors that rarely give neurological complications. Intradural invasion with symptoms secondary to brain compression is extremely rare. Infectious complications have been described.

Staging of surgical approach of sinonasal inverted papilloma

Objective: In this study, we tried to make the staging of surgical approach of inverted papilloma (IP) and investigated the recurrence rate of IP. Patients and methods: Operating staging was as follows: When the IP was limited to the middle meatus, anterior and posterior ethmoid, or sphenoethmoid recess, standard endoscopic sinus surgery (SESS) was performed. When the lesion extended from the middle meatus into the maxillary sinus or originated from the medial wall of the maxillary sinus, radical endoscopic sinus surgery (RESS) was performed. And, when the IP originated from or involved the posterolateral, anterior, inferior wall of the maxillary sinus, intraorbital involvement, extensive growth of the lesion into the frontal or sphenoid sinus, or intradural invasion, external approach with endoscope assistance (Ex + E) was performed. Results: 14 (36%) patients underwent SESS, 9 (23%) patients underwent RESS, and 16 (41%) patients underwent Ex + E. Malignancy occurred in no patient, and recurrences developed in four patients (10%). One of these recurrences happened after SESS, one after RESS and two after Ex + E. Conclusion: In this study, there was no significance of recurrence rate in each group. Better visualization can be obtained by combining the endonasal operation with an external procedure.

Surgical removal of a cavernous sinus chondroma

Chondromas at the base of the skull are most commonly found in the parasellar and sellar regions, and present varying degrees of involvement of the cavernous sinus. However, those confined mainly to the cavernous sinus are rare, and only a few reports show detailed radiological and surgical findings. This 40-year-old woman experienced sudden right orbital pain followed by right third and sixth cranial nerve palsies. Computerized tomography scan and magnetic resonance image depicted a well-circumscribed mass in the right cavernous sinus. A frontotemporal craniotomy with orbito-zygomatic osteotomy was used to approach this lesion intradurally. We drilled away the anterior clinoid process using a high-speed air drill extradurally, exposed the intrapetrous carotid artery, and dissected the right sylvian fissure from the distal, fully exposing the surface of the right cavernous sinus. The soft and grayish tumor was easily removed using sucker and tumor forceps; the venous bleeding from the sinus itself was easily controlled. The tumor was successfully removed via a superior triangle. Intradural invasion of the tumor and heavy venous bleeding from the sinus were not observed. Histologically, the mass was diagnosed as a mature chondroma. The tumor was presumed to originate from the right posterior clinoid process. The lesion was totally removed, and postoperative right ophthalmoplegia had fully disappeared within 6 months after surgery. Successful surgical attack of lesions in the cavernous sinus requires comprehensive and precise knowledge of the microsurgical anatomy and neuroradiology.

Parasellar Aspergillus granuloma extending from the sphenoid sinus: Report of two cases

Sphenoid sinus aspergillosis is a rare disease known to show an aggressive course with high mortality. Early diagnosis, though difficult, is required to prevent lethal fungal meningoencephalitis. We describe two cases of parasellar Aspergillus granuloma extending from the sphenoid sinus clinically indistinguishable from intracranial neoplasms. In the first patient, the fungus colony was visualized by computed tomography (CT) and magnetic resonance imaging (MRI) as a calcified concretion and total removal was curative. In the second patient, partial removal and subsequent antifungal therapy had minimal effect. The prognosis of the patients with this disease depends on prompt surgical treatment before intradural invasion occurs, and CT and MRI are useful diagnostic maneuvers for detecting calcified Aspergillus colonies.

Primary intracranial extradural Burkitt-type lymphoma

An unusual case of primary extranodal Burkitt's-type lymphoma of the frontal base dura and adjacent bone with intradural invasion is reported in a 5-year-old child who presented with unilateral loss of vision in the left eye. Tissue diagnosis was obtained through a craniotomy and the patient received radiotherapy plus intravenous and intrathecal chemotherapy. The tumor regression and improvement in vision was well documented by means of CT and visual evoked potential studies (VEP). A review of the literature on this exceptionally rare tumor is presented.