Primary Intraosseous Granular Cell Tumor of the Sphenoid and Central Skull Base in a Pediatric Patient.

BACKGROUND: Granular cell tumors occur in all ages and many anatomic sites. In the craniofacial region, they typically arise in soft tissue, not bone. We present a primary intra-osseous granular cell tumor of the sphenoid and central skull base arising in a 12- year- old girl. CASE REPORT: A 12-year-old female with sickle cell disease and Jeavons syndrome presented with seizures. Imaging and partial resection revealed an expansile benign granular cell tumor (GCT) involving the sphenoid body, pterygoid process, and central skull base. The disease has remained stable after 36-month follow up. DISCUSSION: GCT primarily involving the osseous sphenoid/skull base has not been previously reported in a child. Although mostly benign, some are aggressive, with malignant transformation in 1-2%. Surgery is the mainstay of treatment, but in the skull base this may be limited by adjacent critical structures. Decision-making is guided by anatomic extent, histology, and clinical behavior.

Predictive value of suprasellar extension for intracranial infection after endoscopic transsphenoidal pituitary adenoma resection.

OBJECTIVE: To investigate the relationship between suprasellar extension (SSE) and intracranial infection after endoscopic endonasal transsphenoidal approach (EETA) for pituitary adenoma resection. METHODS: We retrospectively analyzed 94 patients with suprasellar extended pituitary adenoma admitted to the Department of Neurosurgery of the Affiliated Hospital of Guilin Medical College from January 2018 to December 2021. We measured the preoperative magnetic resonance sagittal SSE and collected clinical data and divided the patients into groups according to the presence of postoperative intracranial infection. The critical value for the SSE was calculated by using a working characteristic curve for the subjects. The risk factors for intracranial infection after EETA resection of pituitary adenomas were analyzed by multivariate regression analysis. RESULTS: Among the 94 patients, 12 cases (12.8%) were placed in the infection group and 82 cases (87.2%) in the non-infection group. The cut-off value for the SSE in the sagittal position was 15.6 mm, the sensitivity was 75%, the specificity was 87.8%, and the area under the curve (AUC) was 0.801. The coronary cut-off value for the SSE was 15.8 mm, the sensitivity was 66.7%, the specificity was 79.3%, and the AUC was 0.787. The SSE values in the sagittal and coronal positions were correlated with postoperative intracranial infection (P < 0.05). After univariate analysis, those with significant differences were included in the multivariate regression analysis. It was concluded that the extension distance of the tumor above the sella in the sagittal position was ≥ 15.6 mm, the tumor texture was hard, and the postoperative cerebrospinal fluid leakage were the independent risk factors for intracranial infection after EETA resection of suprasellar extended pituitary tumors (P < 0.05). CONCLUSIONS: The value of SSE on sagittal MRI can predict intracranial infection in patients with suprasellar extended pituitary adenoma after endoscopic endonasal transsphenoidal resection. This finding recommends neurosurgeons pay more attention to the imaging characteristics of pituitary adenomas and select appropriate treatment plans in combination with the intraoperative conditions to reduce the incidence of intracranial infection.

How I do it: micro-neurosurgical resection of spheno-orbital meningioma with customized orbit reconstruction technique.

BACKGROUND: Spheno-orbital meningioma (SOM) is a rare intracranial pathology with intraosseous hypertrophy and intraorbital extension. METHOD: We described a middle-aged female with SOM who was managed in a micro-neurosurgical manner. The titanium implant was customized and applied to rebuild the orbital wall to prevent postoperative enophthalmus. CONCLUSION: Despite technical demands, favorable cosmetic, and clinical outcomes without complications can be achieved by meticulous surgical technique following radical resection. CLINICAL TRIAL REGISTRATION: NA.

Sphenoid bone localization in the setting of highly aggressive and refractory Multiple Myeloma: A case report.

Multiple Myeloma (MM) is the most common primary osseous malignancy in adults, and it mainly affects men aged between 50 and 70 years. Although rare, central nervous system (CNS) involvement in MM is possible, and it has very poor prognosis. Therefore, fast and accurate diagnosis of CNS manifestation of MM is paramount. Here we describe a case of sphenoid bone localization in a patient with highly aggressive and refractory MM.

Unilateral proptosis as a neglected case of sphenoid wing meningioma: a case report and healthcare challenges in rural Indonesia.

Proptosis, an abnormal protrusion of the eyeball, is a manifestation of a wide variety of pathologies. The complication to be vision- or life-threatening makes early diagnosis is important, especially in rural primary health centers (PHCs) with far-distance referrals to capable hospitals. This case report examines a patient with obvious unilateral proptosis and blurry vision on the right eye for 4 years, with neglect because of prior inadequate diagnosis and explanation before the current complication. Examination shows no light perception, obvious proptosis (30 mm), exodeviation, and ophthalmoplegia. Referral for radiological examination showed a regular extra-axial lesion, well-defined, and broad-based on the right sphenoid wing with hyperostosis. The patient was diagnosed with sphenoid wing meningioma, which complicated into proptosis and blindness. This report aims to explain the current challenges of rural PHCs in Indonesia critically and to emphasize that rural PHCs should overcome the low level of public education, self-awareness of health, and tendency to reject the referral process. Clinicians also have a crucial role in early detection and prompt treatment to reduce further neglected cases.

Clinical Characteristics and Prognostic Analysis of Osteolytic and Hyperostosis Sphenoid Orbital Meningiomas: A Single-Center Experience.

Reactive hyperostosis of spheno-orbital meningiomas (SOMs) often occurred in the sphenoid wing, while osteolytic SOMs (O-SOMs) were rarely discussed. This study preliminarily evaluated the clinical characteristics of O-SOMs and analyzed prognostic factors affecting the recurrence of SOMs. We retrospectively analyzed the medical records of consecutive patients who underwent surgery for a SOM between 2015 and 2020. According to the bone changes of sphenoid wing, SOMs were divided into O-SOMs and hyperostosis SOMs (H-SOMs). A total of 31 procedures were performed in 28 patients. All cases were treated by pterional-orbital approach. It was confirmed that 8 cases were O-SOMs and the other 20 cases were H-SOMs. Total tumor resection was performed in 21 cases. There were 19 cases with Ki 67 ≥3%. The patients were followed up for 3 to 87 months. Proptosis improved in all patients. All O-SOMs had no visual deterioration, while 4 H-SOMs cases had visual deterioration. There was no significant difference in clinical outcomes between the two types of SOM. The recurrence of SOM was related to the degree of resection, but not to the type of bone lesions, invasion of cavernous sinus and Ki 67.

Endoscopic Transorbital Approach for the Management of Spheno-Orbital Meningiomas: Literature Review and Preliminary Experience.

OBJECTIVE: The endoscopic transorbital approach (ETOA) is a minimally invasive approach that could be particularly appropriate for management of spheno-orbital meningiomas. The aim of this study was to perform a systematic review of the literature on the management of spheno-orbital meningiomas via the minimally invasive ETOA, searching for clinical scenarios in which this approach could be best indicated. A secondary aim was to describe 4 illustrative cases. METHODS: A systematic review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Data including patient demographics, tumor features, and surgical and postoperative outcomes were collected. Cases from our initial experience with ETOA were included in the data. RESULTS: Data of 58 patients from 9 selected records and from our surgical series were collected. Subtotal, near-total, and gross total resection rates were 44.8%, 10.3%, and 32.7%, respectively. Symptom improvement after surgery was 100% for proptosis, 93% for visual impairment, and 87% for ophthalmoplegia. The most common postoperative complications were transient ophthalmoplegia and maxillary nerve hypoesthesia. Cerebrospinal fluid leak was reported in 2 patients. CONCLUSIONS: Our findings support the use of the ETOA for management of spheno-orbital meningiomas, particularly in at least 3 clinical scenarios: 1) when predominant hyperostotic bone is present; 2) when a globular tumor not showing excessive medial or inferior infiltration is being treated; 3) as part of a multistage treatment for diffuse lesions.

Diffuse Calvarial Hyperstosis.

Calvarial hyperstosis can be an idiopathic benign finding or secondary to a metabolic pathology. We herein describe a case of diffuse calvarial hyperstosis. A 26-year-old man known to have end-stage renal disease on regular hemodialysis, tertiary hyperparathyroidism, extensive brown tumors, and severe developmental impairment with skeletal deformities was referred to us for macrocephaly. On examination, the patient was chairbound, with speech and motor developmental delay, and frontal bossing. Brain computed tomography revealed diffuse hyperstosis of the calvarium and facial bones expansion with multiple sclerotic and lytic areas, causing subsequent narrowing of the basilar skull foramina. Brain magnetic resonance imaging demonstrated an extensive expansile bone marrow abnormality in the calvarium and skull base. There was mild generalized prominence of cortical sulci and ventricular system. The findings were in keeping with his known hypermetabolic state and tertiary hyperparathyroidism. The patient was managed conservatively with regular follow-up in the clinic.

Sphenoid dysplasia in patients with neurofibromatosis type 1: Clinical features and imaging findings including cerebrospinal fluid alterations.

INTRODUCTION: Neurofibromatosis Type 1 (NF-1) is a genetic disorder that occurs in 1:2500-3000 live births and may involve multiple organs. An uncommon but well-known phenomena is sphenoid dysplasia, which appears in 3-11% of the patients, and may result in significant lowering of the orbit. Understanding the cause and development of this process might predict its course and its treatment. METHODS: The records of 29 patients with PNF in the head and neck region were searched for location of the PNF and type of surgery. Photographs were studied for facial disturbances, including vertical dystopia, hypertelorism, ptosis, exophthalmos and enophthalmos. MR and CT scans were studied for the presence of PNF, aspect of the sphenoid bone, alteration in the CSF circulation, and other tumour involvement. RESULTS: Fourteen of 29 patients with PNF in the head and neck presented with sphenoid dysplasia. All 14 patients had their PNF located in the periorbital region. Increased locoregional CSF collections were seen in almost all of these patients. Besides glioma of the optical nerve or hamartomas in the cerebrum and cerebellum, there were no other tumours found. Surgical treatment varies from simple excision to excessive debulking of the PNF, reconstruction of the orbit and even enucleation. CONCLUSION: Although sphenoid dysplasia is uncommon in NF-1 patients, early recognition is important to prevent facial disfigurement and vision loss. Clinicians should be aware that abnormalities in the periorbital region and especially vertical dystopia might be the first sign of sphenoid dysplasia. Performing a CT or black-bone MR scan would be the next step to confirm or exclude sphenoid dysplasia and abnormal CSF dynamics.

Endoscopic endonasal approach for resection of a recurrent spheno-orbital meningioma resulting in complete resolution of visual symptoms: A case report and review of literature.

PURPOSE: Spheno-orbital meningiomas are rare tumors, accounting for up to 9% of all intracranial meningiomas. Patients commonly present with proptosis, and visual deficits. These slow growing tumors are hard to resect due to extension into several anatomical compartments, resulting in recurrence rates as high as 35-50%. Although open surgical approaches have been historically used for resection, a handful of endoscopic approaches have been reported in recent years. We aimed to review the literature and describe a case of spheno-orbital meningioma with severe vision loss which was resected with an endoscopic endonasal approach achieving complete resolution of visual symptoms. METHODS: A systematic review of literature was conducted in accordance with the PRISMA guidelines. PubMed, Cochrane, and Web of Science databases were queried for spheno-orbital meningiomas resected via an endoscopic endonasal approach. Furthermore, the presentation, surgical management, and post-operative outcomes of a 53-year-old female with a recurrent spheno-orbital meningioma are described. RESULTS: The search yielded 26 articles, of which 8 were included, yielding 19 cases. Average age at presentation was 60.5 years (range: 44-82), and 68.4% of patients were female. More than half of the cases achieved subtotal resection. Common complications associated with endoscopic endonasal surgery included CN V2 or CN V2/V3 hypoesthesia. Following surgical intervention, visual acuity and visual field remained stable or improved in the majority of the patients. CONCLUSION: Endoscopic approaches are slowly gaining momentum for treatment of spheno-orbital meningiomas. Further studies on the clinical benefits of this approach on patient outcomes and post-operative complications is warranted.

Extradural Clinoidectomy in Clinoidal Meningiomas: Analysis of the Surgical Technique and Evaluation of the Clinical Outcome.

The surgical treatment of clinoidal meningiomas is currently still discussed in the literature. Different surgical approaches have been proposed and evaluated, in multiple studies, in order to improve the surgical outcomes. The aim of this study is to evaluate the advantages of extradural clinoidectomy in the context of tumor removal radicality for visual function improvement. A retrospective analysis was performed on 74 patients-of which 26 patients with clinoidal meningiomas were in group III, according to Al Mefty classification-who underwent surgery at the Policlinico Umberto I Hospital between 2000 and 2019. Further, extradural clinoidectomy was performed on 15 patients (Group A), and 11 patients underwent the pterional approach only (Group B). Additionally, visual impairment was present in all 26 patients before surgery. Next, visual function assessment was performed on all patients, both in presurgery and postsurgery. Radiological follow up was performed at 3 and 6 months, and then every 12 months. Gross Total Resection (GTR) was achieved in 13/15 (86.7%) patients who underwent clinoidectomy, and in 4/11 (36.4%) patients who did not undergo clinoidectomy. Visual function improvement was achieved in 12/15 (80%) patients who underwent clinoidectomy and in 4 of 11 (36.4%) who did not undergo clinoidectomy. According to our study, extradural clinoidectomy is the most suitable method for facilitating the gross total resection of clinoidal meningiomas. Our experience and data suggest that a higher rate of total resection and, subsequently, the best visual outcomes are achieved. Extradural drilling via the anterior clinoid process reveals a wider surgical corridor for meticulous tumor resection.

A practical overview of CT and MRI features of developmental, inflammatory, and neoplastic lesions of the sphenoid body and clivus.

The sphenoid bone is an unpaired bone that contributes to the formation of the skull base. Despite the enormous progress in transnasal endoscopic visualisation, imaging techniques remain the cornerstones to characterise any pathological condition arising in this area. In the present review, we offer a bird's-eye view of the developmental, inflammatory, and neoplastic alterations affecting the sphenoid body and clivus, with the aim to propose a practical diagnostic aid for radiologists based on clinico-epidemiological, computed tomography, and magnetic resonance imaging features.

Extracranial Craniopharyngioma Confined to the Sphenoid Sinus.

ABSTRACT: Craniopharyngioma is rare epithelial tumor that develops along the craniopharyngeal duct, and most of these tumors occur in the sellar and suprasellar regions. Although it rarely occurs in the extracranial region, sphenoid solitary lesions were not reported in previous literature. In this study, we report a case of infrasellar craniopharyngioma within the sphenoid sinus without intracranial lesion. A patient with intermittent headache visited a private clinic and presented with sphenoid lesions based on the Magnetic resonance imaging scan results. The mass was completely removed using endoscopic endonasal transsphenoidal approach without any complications and showed characteristic pathologic findings, which lead to the diagnosis of craniopharyngioma.

The Effects of the Application of the "One Disease, One Product" Program to Nursing Practice for Patients After the Resection of Pituitary Adenomas Using the Endonasal Transsphenoi.

OBJECTIVE: To explore the application and effect of the "one disease, one product" project to the nursing care of patients who have undergone pituitary tumour surgery using the nasal sphenoid approach. METHODS: This is a prospective research study. In a standard treatment control study, 132 patients undergoing transnasal pituitary tumour surgery were divided into the control group (n = 71) and the observation group (n = 61). The control group was given routine pituitary tumor care, and the "one disease, one product" nursing model was used on the experimental group. The anxiety level of patients, the incidence of postoperative complications, postoperative hospitalization, and levels of satisfaction and capability of group members were measured between the control and experimental groups. RESULTS: There was no difference in the level of anxiety between the 2 groups before admission (P = 0.634). The anxiety level of the patients in the observation group decreased after the "one disease, one product" nursing intervention (P = 0.012), but in the control group, it did not decrease significantly (P = 0.149), and the anxiety level in the control group was significantly higher than in the observation group on day 1 preoperatively (P < 0.001). CONCLUSIONS: "One disease, one product" nursing can reduce the preoperative anxiety and postoperative satisfaction of pituitary adenoma surgery patients through the sphenoid sinus approach. It is worthy of popularization and application in pituitary adenoma resection through the sphenoid sinus approach.

[A Case of Recurrent Breast Cancer Detected with Diplopia Caused by Sphenoid Bone Metastasis].

We report a case of sphenoid bone metastasis from breast cancer detected with diplopia, as first site of recurrence. Forty- year-old woman with left breast cancer underwent breast-conserving surgery and sentinel lymph node biopsy. The diagnosis was papillotubular carcinoma, pT1pN0, ER(+), PgR(+), HER2(-). Tweleb years later, the examination of diplopia revealed left abducens nerve palsy for sphenoid bone metastasis from breast cancer. Radiation therapy(a total dose of 36 Gy with VMAT)was administrated as topical treatment, but diplopia did not improve. After that, systemic treatment was performed, and 2 years and 6 months have passed since the recurrence was found, she is still alive. We need to be careful of orbital metastasis as a symptom of metastasis from breast cancer.

Hypoplasia of medial pterygoid process in sphenoid bone relates to decreased mesenchymal cell proliferation in the Runx2-haploinsufficient cleidocranial dysplasia mouse model.

OBJECTIVE: Hypoplasia of the medial pterygoid process of the sphenoid bone is a distinct skeletal phenotype in runt-related transcription factor 2 (Runx2) heterozygous mice and patients with cleidocranial dysplasia. The aim of this study was to investigate the involvement of Runx2 in hypoplasia by regulating cell proliferation in the mesenchymal cell condensation region. DESIGN: A total of thirty mouse embryos were used. The medial pterygoid process region in the Runx2+/+, Runx2+/-, and Runx2-/- mouse embryos were histologically investigated. Immunohistochemistry for Runx2 and proliferating cell nuclear antigen (PCNA) was carried out. RESULTS: In embryonic day 14.5, mesenchymal cell condensation appeared at the future medial pterygoid process in Runx2+/+ mice, but was obscure in Runx2+/- mice. In these areas, cells showed a dual expression of Runx2 and PCNA in both Runx2+/+ and Runx2+/- mice. However, the number of Runx2- and PCNA-positive cells was decreased in Runx2+/- mice. In Runx2-/- mice, mesenchymal cell condensation appeared on embryonic day 18.5 at the medial pterygoid process region, associated with a few PCNA-positive cells. Moreover, the PCNA-positive cell rate in the medial pterygoid process was significantly lower in Runx2-/- mice than in Runx2+/+ and Runx2+/- mice. On embryonic day 18.5, Runx2+/- and Runx2-/- mice showed significantly shorter axial length of medial pterygoid process compared to that in Runx2+/+ mice. CONCLUSIONS: The present study demonstrates that Runx2 is involved in cell proliferation in the mesenchymal cell condensation region of the medial pterygoid process during mouse embryonic development.

Surgical Treatment of Recurrent Spheno- Orbital Meningioma.

OBJECTIVE: The extensive bone infiltration and carpet-like growth characteristics of spheno-orbital meningioma (SOM) make it hard to remove entirely, and recurrence and proptosis are the main reasons for reoperation. The authors report 20 cases of surgical treatment for recurrence of SOM, including surgical technique and symptom improvement. METHODS: The clinical data and follow-up results of 20 cases of recurrent SOM at our institution from 2000 to 2017 were retrospectively analyzed. RESULTS: All of the 20 patients with recurrence had received at least one operation before admission, with a mean age of 56 years and 70% female. The mean follow-up time was 36 months (172 months). All patients mainly showed symptoms such as proptosis and headache, and were found to be affected by supraorbital fissure during the operation. in 17 patients with recurrence, the affected sphenoid wing became tumor-like hyperplasia. Patients with extraocular muscle involvement have obvious protrusion and are often accompanied by diplopia. After surgical removal of the tumor, the symptoms of proptosis in 19 patients were significantly improved. During the follow-up, only 3 cases of proptosis recurred. After 15 patients underwent Simpson grade IV resection, 4 patients (27%) relapsed again. Five patients underwent Simpson III resection, and only 1 patient (20%) had tumor recurrence 18th months after surgery, and no proptosis recurred. CONCLUSIONS: The complete surgical removal of recurrent SOM is practically impossible. The main direction of surgical treatment should be to improve the symptoms of proptosis.

Visual Outcomes in Spheno-Orbital Meningioma: A 10-Year Experience.

BACKGROUND: Spheno-orbital meningiomas (SOMs) present distinct surgical challenges because they involve important neurovascular structures, such as the orbit, cavernous sinus, superior orbital fissure, and optic canal. Resection thus focuses on maximum safe resection while preserving these neurovascular structures. Our objective was to describe our method of surgical management of SOMs and summarize visual outcomes. METHODS: A retrospective chart review was performed to identify patients who underwent surgery for SOM in 2011-2021. Demographics, preoperative visual summary, operative details, visual outcomes, and recurrence data were collected. RESULTS: The 33 patients (10 male, 23 female) had a mean age of 56 years (range 27-74 years). The mean tumor volume was 39 mL (range 4.7-220 mL). The mean follow-up period was 18 months (range 1-120 months). Thirty-two patients had preoperative radiographic evidence of proptosis (based on exophthalmos index), 23 (70%) presented with diminished visual acuity, and 10 (30%) had a concomitant visual field deficit. At last postoperative follow-up, vision was stable for 25 patients (83.3%), improved for 1 (3.3%), and worsened for 3 (10%; 2 occurring after tumor recurrence beyond 2 years). Proptosis was stable or improved in all patients. One patient had an enucleated eye. A total of 7 patients (21%) had recurrence of the tumor at 19-72 months from the operation. CONCLUSIONS: In this study, maximum safe resection, including periorbital resection, provided adequate visual and cosmetic outcomes, as well as reasonable tumor control in the long term. Rigid orbital reconstruction was not required to prevent pulsatile enophthalmos.

A single-center experience with giant cell tumors of sphenoid bone and clivus.

OBJECTIVE: To present pathologic, clinical, and treatment findings for giant cell tumors (GCTs) of sphenoid bone and clivus. METHODS: We describe the optimal treatment algorithm in patients with a histopathologic diagnosis of bone GCT by presenting the effects of denosumab treatment in both pediatric and adult patients with GCT undergoing endoscopic transnasal surgery. Clinicopathologic correlation is crucial for the differential diagnosis of GCT and the choice of treatment modality. CONCLUSION: GCT of bone is a local aggressive tumor that accounts for about 3%-7% of all bone tumors. GCTs located in the cranium are extremely uncommon neoplasms. There are no defined guidelines for the treatment of GCTs in skull base. Following surgical resection of the tumor, the addition of denosumab treatments to radiotherapy has a significant role in preventing the recurrence of GCT and in promoting regression of residual tumor size.

Invasion of the pterygoid plates: an indicator for regional lymph node failure in maxillary sinus cancer.

BACKGROUND: The aim of this study was to evaluate the long-term treatment results of combined superselective intraarterial chemotherapy and radiation therapy for advanced maxillary sinus cancer (MSC) and the incidence of regional lymph node failure, and to reveal the clinical and anatomical predictive factors for metastasis. METHODS: We retrospectively evaluated 55 consecutive patients with locally advanced squamous cell carcinoma of the maxillary sinus who were treated with external radiotherapy and superselective intraarterial chemotherapy. Elective nodal irradiation (ENI) was performed only in the clinical node-positive (cN+) cases and not in the clinical node-negative (cN0) cases. RESULTS: Thirty-eight patients were cN0, and 17 were cN+ at diagnosis. Regional lymph node metastases occurred in 7 of 38 patients with cN0, and 2 of 17 with cN+ during the median follow-up period of 36 months. There were more cases of high-grade (3 or 4) late adverse events in the ENI group than in the non-ENI group (13% vs. 41%, respectively; p = 0.03). In cN0 cases without ENI, invasion of the pterygoid plates (57% vs. 90%; p < 0.01) and oral cavity (35% vs. 92%, with invasion vs without invasion, respectively; p = 0.02) was significantly correlated with a low 5-year regional recurrence-free rate. CONCLUSIONS: Patients with MCS and invasion of the pterygoid plates and oral cavity can be considered appropriate candidates for ENI.