Long-Term Survival after Primary Ewing's Sarcoma of the Skull with Intracranial Extension.

BACKGROUND AND OBJECTIVE: Primary Ewing's sarcoma of the skull is a very rare malignant neoplasm, predominantly occurring in children and adolescents. We describe here the clinical, neuroradiologic, and histopathologic features of a patient with primary Ewing's sarcoma of the skull and discuss the standards of therapy for this type of tumor. CLINICAL PRESENTATION: This 18-year-old male patient presented with a primary Ewing's sarcoma of the skull, involving the dura of the frontal and parietal lobes of the left cerebral hemisphere. He was treated with gross total surgical excision of tumor, skull reconstruction, chemotherapy, and irradiation. Twelve years after the surgery, the patient has no evidence of local recurrence or distant metastases. Radical surgical excision of the primary tumor with safety margins is thought to play a role in the favorable clinical course. CONCLUSION: The presented case is the longest surviving patient after treatment of primary Ewing's sarcoma of the skull bone. This rare type of tumor may allow better survival rates under adequate management than sarcoma elsewhere in the body.

[Primary intraosseous cavernous hemangioma of the cranium: a case report]. / Pervichnaya vnutrikostnaya kavernoznaya gemangioma kostei cherepa: obzor literatury i opisanie klinicheskogo nablyudeniya khirurgicheskogo lecheniya.

Primary intraosseous cavernous hemangioma (PICH) is a rare benign vascular tumor. This neoplasm is common in the spine and less common in skull. Toynbee J. first described this tumor in 1845. PICH of the cranium does not always have typical X-ray features and should be always differentiated with other more common skull lesions. Surgical resection is preferable since total resection is followed by favorable prognosis. We present a 65-year-old patient with asymptomatic tumor of the right parietal bone. CT revealed osteolytic lesion that required total resection and skull repair. Histopathological analysis revealed intraosseous cavernous hemangioma.

Endoscopic Approach for Resection of Frontal Forehead Osteoma: Technical Case Report Instruction.

Forehead osteomas are benign bone tumors. They are frequently associated with exophytic growth in the outer table of the skull, causing cosmetic disfigurement of the face.1-9 The objective of this study was to present the efficacy and feasibility of the endoscopic treatment of forehead osteomas by presenting a case report with details of the surgical technique. A 40-year-old female patient presented with aesthetic complains of a progressing bulge in the forehead. A computed tomography scan with 3-dimensional reconstruction showed bone lesions on the right side of the forehead. The patient underwent surgery under general anesthesia with no noticeable incision, which was planned 2 cm behind the hairline in the midline because the osteoma was close to the midline plane on the forehead (Video 1). A retractor coupled with a 4-mm channel for endoscopy and a 30-degree optic was used to dissect, elevate the pericranium, and locate the 2 bone lesions in the forehead. The lesions were removed using a chisel, endoscopic facelifting raspatory, and a 3-mm burr drill. The tumors were resected completely, resulting in good cosmetic outcomes. The endoscopic approach for treating forehead osteomas is less invasive and facilitates complete removal of tumors, which results in good cosmetic outcomes. Neurosurgeons should consider and add this feasible approach to enhance their surgical armamentarium.

Combined endoscopic endonasal & transoral approach to transpatial lesion involving the infratemporal fossa and masticator space: A case study and literature review / Abordaje endoscópico combinado endonasal y transoral a una lesión transespacial involucrando la fosa infratemporal y el espacio masticador: Reporte de caso y revisión de la literatura

Transpatial skull base lesions involving the infratemporal fossa (ITF) are challenging due to the complex neurovascular structures of the region. Open approaches have traditionally been utilized to access these spaces. We present a 55-year-old woman presented with a mesenchymal mass involving the left ITF and masticator space. A combined endoscopic endonasal transpterygoid approach was performed followed by an endoscopic transoral-transmandibular corridor to access and resect the tumor. The post-operative course was unremarkable with no recurrence during her follow-up. Combined endoscopic approaches for transpatial tumor resection offered sufficient exposure to access safely each space (AU)

Las lesiones de la base craneal con una distribución transespacial e involucrando la fosa infratemporal (ITF) representan un desafío debido a las complejas estructuras neurovasculares de dicha región. Los abordajes abiertos han sido empleados tradicionalmente para acceder a estas regiones. Presentamos el caso de una mujer de 55 años que consultó por una lesión mesenquimal localizada en la ITF izquierda y en el espacio masticatorio. Se realizó un abordaje combinado endoscópico transpterigoideo endonasal seguido de un abordaje endoscópico transoral-transmandibular para acceder y resecar el tumor. El curso postoperatorio fue anodino, y no ha presentado recidiva con 21 meses de seguimiento. Los abordajes endoscópicos combinados para la resección de lesiones tumorales, con una distribución transespacial involucrando la ITF, ofrecen una exposición suficiente para acceder de forma segura a cada espacio (AU)

Extradural hematoma from trauma to midline skull tumour.

We present a case of extradural hematoma resulting from a relatively minor closed injury over the vertex where a plasma cell tumour had invaded the superior sagittal sinus. The patient underwent an emergency craniotomy and evacuation of the hematoma. Hemostasis and prevention of recollection of the hematoma were hampered by the erosion of the sagittal sinus making its direct repair impossible. This was achieved by hitching up the dura lateral to the sinus to become its lateral wall reinforced by hemostatic agents. The patient made a full recovery. Malignant tumours invading the dural venous sinuses and eroding the skull can cause life-threatening intracranial bleeding after relatively minor trauma.

Giant Skull Tumor: A Benign Meningioma.

ABSTRACT: A 37-year-old male patient was complaining from painless forehead swelling, which started 5 years ago. Brain computed tomography scan and magnetic resonance imaging showed a large extradural mass compressing the both frontal lobes with skull bone infiltration, hyperostosis and enlargement. The patient was operated in 2 stages. In the first stage, the authors achieved separation for the tumor from the scalp and skull. In the second stage after 1 week, the authors continued with circumferential dural opening around the tumor and separation of the tumor from brain tissue. Duroplasty was performed by autologous fascia latta and skin flap was closed primarily. The specimen was received grossly as huge mass weighing 1530 g and measuring 39 × 16 × 12 cm. Histopathologic examination showed grade I meningioma extending beyond the skull to the surrounding soft tissue.

Pediatric Cases of Recurrent Skull Giant Osteoma Misdiagnosed as Fibrous Dysplasia.

ABSTRACT: Osteomas are benign mature bone tumors that typically arise in the skull. Osteomas larger than 3 cm in diameter are considered giant osteomas. Giant osteomas of the skull vault are very rare, especially in children; therefore, only a few cases have been reported in the literature. Although osteomas are usually asymptomatic, a large skull mass can cause headache, as well as esthetic disfigurement of the forehead. it can be misdiagnosed as other conditions, such as fibrous dysplasia, ossifying cephalhematoma, or other malignant bone tumors. Herein, the authors report 2 rare pediatric cases of giant osteomas mimicking fibrous dysplasia and their successful surgical excision. These cases showed good results without recurrence or complications on long-term follow-up after complete excision.

Primary Intraosseous Cavernous Hemangioma of the Cranium: A Systematic Review of the Literature.

Primary intraosseous cavernous hemangioma (PICH) is a rare, benign tumor of vascular origin, typically arising in the vertebral body. Its presence in the skull is exceedingly rare, with only a few cases being reported worldwide. We carried out the first systematic review of the literature, covering the epidemiology, clinical and imaging features, management, and prognosis of cranial PICH. The literature search revealed 51 studies with 77 patients; the mean age of the patients was 32.7 years with a female predominance of 1.4:1. The majority of cranial PICHs were located in the calvarium, primarily in the frontal and parietal regions, with only a few located in the skull base. The most common initial clinical manifestation was local growth or swelling, followed by a headache. Radiographically, PICHs represented osteolytic, intradiploic masses, which in many cases displayed trabeculations, leading to the so-called "honeycomb" or "starburst" pattern. After contrast administration, PICHs typically enhance. Tumor removal, with craniectomy or en bloc resection and subsequent skull reconstruction, was selected for calvarial PICHs, whereas a transsphenoidal approach, with only partial resection, was applied for clival/sella PICHs. Preoperative embolization, aiming to minimize intraoperative blood loss, was performed in the case of large tumors. At a mean follow-up of 39 months, no patient experienced tumor recurrence, even after subtotal resection. Owing to the benign nature of the tumor, maximal safe resection is recommended as the treatment of choice for patients with cranial PICH.

Two-step treatment of a giant skull vault hemangioma: A rare case report and literature review.

Skull vault hemangiomas are benign vascular tumours of the calvaria that are usually asymptomatic or present as firm, painless lumps. We present a case of a 59-year-old female with a giant intraosseous calvarial hemangioma that was admitted in our department with a palpable mass over the left frontoparietal region, personality changes and impaired emotional and cognitive functions. The patient was treated with a two-step approach involving endovascular and surgical treatment, and suffered two rare, but recognized complications, a contrecoup intracerebral haemorrhage and valproate-induced stupor and parkinsonism. At the 6-month follow-up, the patient had complete recovery with a good neurological outcome.

Cranioplasty With Customized Craniofacial Implants and Intraoperative Resizing for Single-Stage Reconstruction Following Oncologic Resection of Skull Neoplasms.

BACKGROUND: Craniectomies requiring skull reconstruction are indicated following oncological resection of masses involving the underlying brain and/or skull. Immediate cranioplasties have previously been performed using suboptimal hand-bending or molding techniques using "off - the - shelf" products. Today with computer - aided design, customized craniofacial implants have become widely available for personalized reconstruction of resected bone and soft tissue. We present here the largest series to date of single stage reconstruction using alloplastic biomaterials in consecutive patient series with oversized customized implants. METHODS: A single-surgeon, retrospective, 8-year study was conducted on all consecutive patients undergoing single stage cranioplasty with prefabricated implants using a myriad of biomaterials. All outcomes were analyzed in detail and compared with previous studies utilizing similar alloplastic implants. RESULTS: In total, 56 patients underwent resection of skull neoplasms and subsequent cranioplasty reconstruction using customized implants. The most common neoplasms were meningiomas (39%). The most common complications seen among patients were dehiscence - (7%), and extrusion of implant - (3.5%). There was no significant difference in the incidence of postoperative complications between patients who had postoperative chemotherapy/radiotherapy versus those that did not (22.2% versus 13.1%, P = 0.39). One-year follow-up revealed acceptable cranial contour and symmetry in all 56 cases. CONCLUSIONS: This is a consecutive case series of prefabricated single-stage cranioplasty, following resection of brain tumors with bone extension or skull bone neoplasm, demonstrating excellent results with regards to safety and patient satisfaction. There are several advantages such as comprehensive resection and reconstruction plan using 3D models, shorter operative time, and better restoration of complex anatomy.

Epidemiological Profile of 96 Intracranial Tumors Treated in a Single Reference Center

Objectives The present study aims to categorize the prevalence of intracranial tumors surgically treated at the neurosurgery service of Hospital Universitário Evangélico Mackenzie (HUEM) between 2016 and 2018. Material and Methods This survey included patients surgically treated due to primary or metastatic intracranial neoplasia between 2016 and 2018 at a referral center in the city of Curitiba. These patients were analyzed for epidemiological, histopathological, and topographic data, and they underwent an assessment of the outcome at the time of hospital discharge. Results Atotal of 96patientsmet the inclusion criteria. Themost prevalent tumorwas the glioma, with 39.6% of the sample, with glioblastoma being themost prevalent histological type. Brainmetastases andmeningiomas represented, respectively, 21.9%and 18.8%of the total. There was a predominance of supratentorial and intra-axial tumors in our sample. Conclusion Glioma was the most commonly found tumor, directly associated with high morbidity and mortality. The development of new and more effective drugs with action directed at themolecular level of intracranial tumorsmay be the path to a longer survival and improvement in the quality of life of these patients.

Cranial sonolucent prosthesis: a window of opportunity for neuro-oncology (and neuro-surgery).

INTRODUCTION: Ultrasound (US) is a versatile technology, able to provide a real-time and multiparametric intraoperative imaging, and a promising way to treat neuro-oncological patients outside the operating room. Anyhow, its potential is limited both in imaging and therapeutic purposes by the existence of the bone shielding. To enhance the spectrum of uses, our group has designed a dedicated US-translucent cranial prosthesis. Herein, we provide the proof of concept of a long-term US-based follow-up and a potential bedside therapeutic exploitation of US. METHODS: The prosthesis was first implanted in a cadaveric specimen to record any issue related to the cranioplasty procedure. Hence, the device was implanted in a patient undergoing surgery for a multi-recurrent anaplastic oligodendroglioma. US multiparametric scans through the device were acquired at 3, 6, 9, and 30 months after the procedure. RESULTS: The prosthesis could be modeled and implanted through ordinary instruments, with no concerns over safety and feasibility. Trans-prosthesis multiparametric US imaging was feasible, with image quality comparable to intraoperative US. Long-term follow-up in an outpatient setting was possible with no adverse events. Trans-prosthesis mechanical interaction with microbubbles was also feasible during follow-up. CONCLUSIONS: This report provides the first proof of concept for a potential breakthrough in the management of neuro-oncological patients. Indeed, through the implantation of an artificial acoustic window, the road is set to employ US both for a more dynamic long-term follow-up, and for US-guided therapeutic applications.

Synchronous Reconstruction of the Jaw Using a "One and a Half"-Barrel Fibular Bone Graft for Ossifying Fibroma Patients.

BACKGROUND: Ossifying fibroma is one of the common benign tumors that affect the appearance and functions of the jaw. Ossifying fibroma may exhibit a wide range of biological behaviors, leading to deformities involving the jaw and other secondary facial deformities. Hence, to improve the function of the jaw and the patient's general facial appearance (bearing in mind each patient's facial shape and, or appearance), the authors thus, however, used a ''one and a half"-barrel fibular bone graft to achieve the ideal height and radian of the bone graft. CASE PRESENTATION: Between July 2017 and January 2021, the authors retrospectively collected and analyzed clinical and surgical data from 39 patients who had undergone operations in our hospital. Twenty patients were operated on using our new surgical method, whereas 19 patients received conventional or debulking operation. Clinical factors associated with the operation were assessed, including classification of the jaw defects, perioperative complications, and postoperative outcomes. RESULTS: All the flaps ultimately survived. According to the postoperative satisfaction survey, patients who underwent reconstruction were satisfied with their postoperative facial appearance, with an average of 8.5 out of 10. Based on the preoperative clinical data, 26 patients had suitable bone grafts for dentures to improve their oral function. CONCLUSIONS: A ''one and a half''-barrel fibular bone graft effectively improves the facial appearance of patients and as well as provides an appropriate height and radian for the bone graft.

Extracranial temporal bone paragangliomas: Re-defining the role of otologic surgery within the scope of function-preserving multimodal concepts.

INTRODUCTION: The aim of this study was to present our concept in the management of extracranial temporal bone paragangliomas and demonstrate the outcome after primary surgical management of the middle ear component, with an individualized indication for adjuvant radiotherapy. MATERIALS AND METHODS: The records of all patients treated for extracranial jugulotympanic paragangliomas by means of primary surgical management between 2010 and 2021 were studied retrospectively. RESULTS: Twenty-nine patients made up our study sample (mean age 58.8 years). 15 cases were managed solely by means of surgery. Out of the remaining 14 cases with reduction of the middle ear component, adjuvant irradiation was performed in 11 cases, whereas a wait-and-scan strategy was adopted at the patient's request in three cases. No further growth was detected in our study cases. CONCLUSION: Our protocol seems to be associated with an acceptable quality of life and a satisfactory oncologic outcome.

Endolymphatic Sac Tumour: A Case Report and Review of the Literature

Introduction Endolymphatic sac tumor (ELST) is a slow-growing, low-grade, locallyinfiltrative tumor arising from the endolymphatic sac/duct, which is located in the posterior part of the petrous temporal bone. It may be sporadic in origin, or may be associated with Von-Hippel Lindau (VHL) syndrome. Case description A 40-year-old female patient with an ELST without VHL syndrome who was treated successfully by microsurgical extirpation of the tumor. Discussion We discuss the radiological features and the histopathology of this rare tumor and review the relevant literature. Conclusion The case herein reported adds to the previously-reported cases of this rare tumor.

Surgical Approaches to Tumors of the Occipito-Cervical, Subaxial Cervical, and Cervicothoracic Spine: An Algorithm for Standard versus Extended Anterior Cervical Access.

OBJECTIVE: To propose a surgical approach algorithm for the tumors of the cervicothoracic spine. METHODS: All patients operated for vertebral column tumors involving the occipito-cervicothoracic spine were reviewed. Oncologic characteristics and surgical approach were gathered. Approach was classified by the use of staging and trajectory (posterior, transnasal, transoral, transmandibular, transcervical, transsternal). Angle of attack was defined for the occipitocervical junction tumor as the angle inscribed by the inferior mandibular plane and line connecting the superior tumor pole and mandibular angle. For lesions extending below the thoracic inlet, angle of attack was that inscribed by the plane of the thoracic inlet and the line connecting the jugular notch and inferior tumor pole. RESULTS: In total, 115 patients were included (mean age 56.7 years, 64 [56%] male, average size 26.5 cm3, 39 [34%] primary tumors). Sixty-nine (60%) of patients had single-stage procedures (57 [49.6%] posterior-only, 12 [10.4%] anterior-only), 35 (30.4%) had 2-stage procedures, and 11 (9.6%) had 3- or 4-stage approaches. Lesions requiring a combined transmandibular-transcervical approach all involved the C2 and C3 levels and had a significantly steeper angle of attack (42.5 ± 9.5 vs. 6.1 ± 13.3°; P = 0.01) and greater superior tumor extent above the inferior plane of the mandible (3.69 ± 2.18 vs. 0.33 ± 0.78; P = 0.002). Lateral tumor extent, tumor size, nor inferior angle of attack differed significantly between approach groups. CONCLUSIONS: Here, we present a preliminary decision-making algorithm for the management of vertebral column tumors of the cervicothoracic spine. Based on this single-center experience, we suggest which patients, assessed via a combination of tumor histology and regional anatomy, may benefit from extended anterior surgical access.

Safety of Contemporary Resorbable Fixation Systems for Craniofacial Reconstruction in Pediatric Patients.

BACKGROUND: Resorbable hardware allows adequate strength for maintaining the relative position of the craniofacial skeleton during osseous healing, and allowing subsequent growth in pediatric patients. The purpose of this study was to determine the complication profile of the latest copolymer of resorbable plates for craniofacial reconstruction in pediatric patients. METHODS: Retrospective query of the operative billing record was performed for use of the DePuy Synthes Rapidsorb Fixation System at the authors' tertiary children's hospital from 2015 to 2019. Three hundred twenty-five patients were randomly selected for analysis of complications within the first postoperative year. RESULTS: Median age at craniofacial reconstruction was 3.0 years, with 127 procedures (39.9 percent) performed for an intracranial mass and 141 procedures (44.3 percent) performed for craniosynostosis. Overall, 7.9 percent patients had a postoperative wound complication. The most common complications were dehiscence (3.8 percent), hematoma/seroma (3.5 percent), and infection (2.5 percent). There were no instances of extrusion, plate fracture, or screw loosening. Hardware complications in the temporoparietal region were more likely to occur at incision sites (p = 0.001), whereas wound complications at the frontal region were more likely to occur away from incision sites (p < 0.001). There were no differences in complications between plates secured with resorbable screws or with the resorbable injectable polymer system (p ≥ 0.161 for all). Radiotherapy was significantly implicated in development of clinical infection (p = 0.001), culture-positive infection (p < 0.001), readmission (p = 0.007), reoperation (p = 0.003), and plate removal (p = 0.007). CONCLUSION: Resorbable cranial hardware has an overall favorable complication profile for craniofacial reconstruction in pediatric patients undergoing surgical intervention for craniosynostosis or intracranial mass resection.

Multiple myeloma complicated by skull plasmacytoma discovered after head injury.

Plasmacytoma is a malignant tumor originating from the plasma cells of the bone marrow. Those discovered after a head injury is rare. We report a case of a 48-year-old female who complained of scalp mass without other symptoms after head injury. Meningioma was considered preoperatively based on imaging findings, and surgical resection was performed. Postoperatively, multiple myeloma complicated by skull plasmacytoma was diagnosed by histopathology and systematic examinations in succession. When evaluating a head mass that appeared after a head injury, plasmacytoma should be considered at times. Osteolytic changes and biconvex form on imaging are beneficial to differentiation.

Programmed Death-Ligand 1 Expression and Tumor-Infiltrating Lymphocytes in Temporal Bone Squamous Cell Carcinoma.

OBJECTIVES/HYPOTHESIS: The tumor immune microenvironment in temporal bone squamous cell carcinoma (TBSCC), including the programmed death-ligand 1 (PD-L1) expression and tumor-infiltrating lymphocytes (TILs), has not been established. STUDY DESIGN: Retrospective cohort study. METHODS: We performed immunohistochemistry analyses to retrospectively analyze 123 TBSCC cases for PD-L1 expression and TILs and their prognostic significance. We also evaluated the prognostic correlations between these immunomarkers and the therapeutic responses to chemoradiotherapy (CRT). RESULTS: PD-L1 expression (≥1%) was detected in 62 (50.4%) TBSCC cases and was significantly associated with worse prognosis: progression-free survival (PFS), P < .0001; overall survival (OS), P = .0009. A high density of CD8+ TILs was significantly associated with better prognosis (PFS, P = .0012; OS, P = .0120). In contrast, a high density of Foxp3+ TILs tended to be associated with an unfavorable prognosis (PFS, P = .0148; OS, P = .0850). With regard to the tumor microenvironment subtypes defined by CD8+ TILs and PD-L1 expression, the CD8low /PD-L1+ group showed significantly worse prognosis. Among the 36 neoadjuvant CRT-treated cases, PD-L1 expression was significantly associated with worse OS (P = .0132). Among the 32 CRT-treated cases without surgery, a high density of CD8+ TILs tended to be more highly associated with complete response to CRT compared to a low density of CD8+ TILs (P = .0702). CONCLUSIONS: These results indicate that the evaluation of the tumor immune microenvironment may contribute to the prediction of prognoses and the selection of an individualized therapeutic strategy for patients with TBSCC. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2674-2683, 2021.