Skull base plasmacytoma in young patients aged below 40 years: Radiological perspectives and clinical outcomes.

BACKGROUND: Plasmacytoma of the skull base is a rare manifestation of plasma cell neoplasm with only a few cases documented in literature involving young adults. Plasmacytoma can be an isolated solitary lesion or a secondary manifestation of multiple myeloma (MM). In this study, we report the clinical and radiological characteristics, management, and outcomes of patients under the age of 40 who presented with skull base plasmacytoma and associated neurological manifestations. Additionally, we share our experience in treating a rare case of skull base plasmacytoma diagnosed during pregnancy, in which the patient exhibited a favorable response to myeloma treatment initiated after delivery. CASE SERIES: Four patients were identified, comprising one pregnant female and three male patients, with a median age of 36 years (range 33-37 years). The main presenting symptoms were headache, dizziness, and cranial nerve palsy. All patients received underwent systemic myeloma therapy and radiotherapy with three patients also underwent autologous stem cell transplantation (ASCT). Notably, all patients achieved complete remission. CONCLUSION: Skull base plasmacytoma represents a rare manifestation of plasma cell neoplasms, underscoring the importance of considering it in the differential diagnosis of skull base lesions to ensure early intervention and avoid potential serious complications. Throughout our series, the cornerstone of therapy involved radiotherapy, systemic myeloma therapy, and ASCT, all of which elicited a favorable response in every case.

[Transoral approach in surgery for chordomas extending into craniovertebral junction: a systematic review of the literature]. / Transoral'nyi dostup v khirurgii khordom, rasprostranyayushchikhsya v oblast' kraniovertebral'nogo perekhoda: sistematicheskii obzor literatury.

To date, treatment of chordomas involves maximal tumor resection followed by proton therapy. Various approaches are used depending on location of tumor (transcranial and through natural anatomical openings (nose, mouth), as well as their combinations). Although transoral approach has been introduced into neurosurgical practice for a long time, it is routinely used in patients with chordoma only in certain hospitals in the world. OBJECTIVE: To analyze postoperative outcomes in patients with chordomas of skull base and craniovertebral joint after transoral surgery. MATERIAL AND METHODS: We analyzed literature data devoted to patients with chordomas of skull base and craniovertebral joint after transoral surgery or another approach combined with transoral access. Among 111 primary articles, we selected 38 manuscripts including description of 109 patients with skull base chordoma who underwent transoral surgery or combination of approaches including transoral one. RESULTS: Gross total resection was achieved in 45.9% (n=50) of cases including 1 patient after en bloc resection. Subtotal resection was carried out in 28.4% of cases, partial - in 24.8%, biopsy - in 0.9% of cases. The complication rate in this group was 30%. The most common events were swelling of the tongue (10%) and diastasis of posterior pharyngeal wall sutures (8.2%) that required redo surgery. CSF leakage and meningitis were rare (1.8% and 3.6%, respectively). CONCLUSION: Transoral access allows for gross total resection of midline tumors with low incidence of severe complications. Combination of transoral and transcranial approaches is advisable to increase extent of resection.

Microsurgical resection of a samii type D jugular foramen schwannoma via the carotid triangle without removal of bony structure: how I do it.

BACKGROUND: Samii Type-D jugular foramen schwannomas (JFSs) are the most challenging for neurosurgeons because of anatomical complexity. Various neurosurgical approaches have been described to gain access to JF. METHODS: We present a female with incidental diagnosis of the Type-D JFS. Complete radical resection was achieved via the carotid triangle approach without any bony structure removal. And the patient was discharged asymptomatic and without new-developed neurological deficits. CONCLUSIONS: The carotid triangle is a secure and appropriate approach for some cases of selected Type-D JFSs. However, the specific indications of this approach should be further explored and investigated.

Interdisciplinary Management of Skull Base Tumors. / Interdisziplinäre Behandlung von Schädelbasistumoren.

OBJECTIVE: Endoscopic endonasal skull base surgery has gained acceptance worldwide. Comparative analysis has demonstrated that endoscopic skull base surgery may have advantages for many pathologies of the anterior skull base, e. g., sinonasal malignant tumors; pathologies of the central skull base, e. g., pituitary adenomas, craniopharyngiomas; well-selected cases of planum sphenoidale and tuberculum sellae meningiomas; or for clival lesions, e. g., chordomas, chondrosarcomas, or selected meningiomas. Over the past three decades, interdisciplinary surgical teams, consisting of otolaryngologists and neurosurgeons, have provided detailed anatomical knowledge, suggested new approaches or modifications of established surgical techniques, and offered continued surgical education. METHOD: A review of pertinent literature was conducted with an emphasis on interdisciplinary endoscopic surgery of skull base lesions. RESULTS: Based on the authors̓ surgical experience in two different interdisciplinary endoscopic skull base centers, the authors classify approaches for endoscopic endonasal skull base surgery, describe indications, and key anatomic landmarks for common pathologies, and highlight surgical techniques to avoid complications. CONCLUSION: Interdisciplinary endonasal endoscopic surgery combines surgical expertise, improves resection rates for many pathologies, and minimizes morbidity by reducing the incidence of surgical complications.

Gamma knife radiosurgery for clival metastasis: case series and systematic review.

PURPOSE: Clival metastatic cancer is rare and has limited literature to guide management. We describe management of clival metastasis with Gamma Knife radiosurgery (GKRS). We augment our findings with a systematic review of all forms of radiation therapy for clival metastasis. METHODS: Records of 14 patients with clival metastasis who underwent GKRS at the University of Pittsburgh Medical Center from 2002 to 2023 were reviewed. Treatment parameters and clinical outcomes were assessed. A systematic review was conducted using evidence-based guidelines. RESULTS: The average age was 61 years with male predominance (n = 10) and average follow-up of 12.4 months. The most common primary cancers were prostate (n = 3) and lung (n = 3). The average time from cancer diagnosis to clival metastasis was 34 months. The most common presenting symptoms were headache (n = 9) and diplopia (n = 7). Five patients presented with abducens nerve palsies, and two presented with oculomotor nerve palsies. The median tumor volume was 9.3 cc, and the median margin dose was 15 Gy. Eleven patients achieved tumor control after one procedure, and three with progression obtained tumor control after repeat GKRS. One patient recovered abducens nerve function. The median survival from cancer diagnosis and GKRS were 49.7 and 15.3 months, respectively. The cause of death was progression of systemic cancer in six patients, clival metastasis in one, and unknown in four. The systematic review included 31 studies with heterogeneous descriptions of treatment and outcomes. CONCLUSION: Clival metastasis is rare and associated with poor prognosis. GKRS is a safe, effective treatment for clival metastasis.

Free temporalis muscle fascia graft in dural reconstruction following surgical resection of intermediate and malignant skull base tumors: A 10-year experience from a single center.

BACKGROUND: Data from patients with post-ablative dural defects reconstructed using a free temporalis muscle fascia graft (FTFG) after resection of anterior or central skull base tumors were retrospectively analyzed. METHODS: The primary predictor and outcome variables were the reconstructive methods for dural repair and postoperative cerebrospinal fluid (CSF) leakage rate, respectively. RESULTS: Eighty patients were included, and 94 postoperative dural reconstructions were performed using FTFG. The postoperative CSF leakage rate was 3.19%. The postoperative CSF leakage rates did not significantly differ between open and endonasal endoscopic surgeries (1.92% vs. 4.88%; p > 0.05). In cases completed using the endonasal endoscopic approach, the postoperative CSF leakage rate was significantly associated with the intraoperative CSF leak flow (p < 0.05). CONCLUSIONS: Post-ablative dural defect reconstruction using FTFG resulted in low postoperative CSF leakage and complication rates comparable to those of free fascia lata graft from available literature.

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.

Combined simultaneous endoscopic endonasal and transcranial surgery using high-definition three-dimensional exoscope for malignant tumors of the anterior skull base.

BACKGROUND: Advanced surgical interventions are required to treat malignancies in the anterior skull base (ASB). This study investigates the utility of endoscopic endonasal and transcranial surgery (EETS) using a high-definition three-dimensional exoscope as an alternative to traditional microscopy. METHODS: Six patients with carcinomas of varying histopathologies underwent surgery employing the EETS maneuver, which synchronized three distinct surgical modalities: harvesting of the anterolateral thigh flap, initiation of the transnasal technique, and initiation of the transcranial procedure. RESULTS: The innovative strategy enabled successful tumor resection and skull base reconstruction without postoperative local neoplastic recurrence, cerebrospinal fluid leakage, or neurological deficits. CONCLUSION: The integration of the exoscope and EETS is a novel therapeutic approach for ASB malignancies. This strategy demonstrates the potential of the exoscope in augmenting surgical visualization, enhancing ergonomics, and achieving seamless alignment of multiple surgical interventions. This technique represents a progressive shift in the management of these complex oncological challenges.

[Endoscopic Surgery for Skull Base Tumors].

With the development of endoscopic and peripheral instruments, endonasal or transcranial endoscopic surgery for skull-base tumors has become more common. Preoperative simulation makes it relatively easy to understand the anatomical relationship between skull base tumors and the surrounding vital structures, which vary with each case. This may lead to the avoidance of complications and an improvement in the removal rate. Especially in cases of skull base tumors where multiple surgical approaches are possible, the three-dimensional model can be used to confirm the surgical field for each approach and consider the most appropriate. With the development of endovascular treatment and radiotherapy, experience in craniotomy has decreased. Young neurosurgeons need to develop skills to learn as efficiently as possible from their limited experience. Therefore, it is extremely useful to provide an environment that allows for easier preoperative simulations.

[Brain tumor surgery in adults.] / Felnottkori agydaganatok sebészete.

Despite the advanced medical and radiation therapy, the role of surgical resection of brain neoplasms still remains indisputable. The maximal safe resection of benign brain tumors may result in complete recovery of the patient. Surgery of malignant tumors can resolve mass effect, improve the neurological condition of the patient providing the possibility for further complex oncotherapy based on molecular level histopathology results. The advances in technical and multidisciplinary environment of brain tumor surgery facilitate more radical and safer resection resulting in better outcomes and preservation of quality of life, even in case of tumors which were considered inoperable until recently. In this review we present the recent technical innovations used in brain tumor surgery and discuss the surgical strategy of the most common tumor types (gliomas, meningiomas, cranial nerve tumors and brain metastases). The surgical management of complex skull base tumors, pituitary tumors, as well as neuro-endoscopic surgery and pediatric brain tumors are discussed in other papers of this special issue.

[Endoscopic transnasal resection of clival meningiomas]. / Rezul'taty endoskopicheskogo transnazal'nogo udaleniya meningiom oblasti skata.

BACKGROUND: Surgical treatment of ventral and ventrolateral meningiomas of posterior cranial fossa is difficult in modern neurosurgery. This is due to peculiarities of approach to these areas and concentration of critical structures (cranial nerves and great vessels). Currently, endoscopic transnasal approach to these meningiomas allows partial, and in some cases, total resection. However, this technique is not widespread. OBJECTIVE: To analyze the world literature data on postoperative outcomes in patients with clival and petroclival meningiomas after endoscopic transnasal resection. MATERIAL AND METHODS: We analyzed 22 articles representing treatment of 61 patients with clival and petroclival meningiomas. RESULTS: Total or near-total resection was achieved in 22.9% of cases, subtotal resection - 40.9%, partial resection - 26.2% (data were not provided in other cases). Even partial and subtotal resection leads to significant regression of symptoms. CONCLUSION: Endoscopic transnasal surgery is a full-fledged alternative to transcranial approaches in surgical treatment of clival meningiomas. It is also an additional option for patients with petroclival meningiomas after ineffective transcranial approaches. Transnasal tumor shrinkage and devascularization lead to brainstem decompression, regression of hydrocephalus and baseline clinical symptoms.

Proton beam therapy for clival chordoma: Optimising rare cancer treatments in Australia.

With the anticipated launch of the Australian Bragg Centre for Proton Therapy and Research (ABCPTR) in Adelaide, Australia, proton therapy will become a significant addition to existing cancer treatment options for Australians. The anticipated benefits will be particularly evident in rare cancers such as clival chordomas, a challenging tumour entity due to the anatomical relationship with critical structures, and proven radio-resistance to conventional radiation therapy. The article synthesises key findings from major studies and evaluates the current evidence supporting various management strategies for clival chordomas. It also considers the influence of institutional volume and multidisciplinary team management on patient outcomes and outlines how high-quality care can be effectively delivered within the Australian healthcare system, emphasising the potential impact of proton therapy on the treatment paradigm of clival chordomas in Australia.

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.

[The efficacy and safety analysis of endoscopic resection for infratemporal fossa benign mass].

Objective: To investigate the efficacy and safety of endoscopic resection of infratemporal fossa mass and to determine the indications for surgery. Methods: A retrospective case series study was conducted, including a total of 29 patients who underwent endoscopic surgery to treat infratemporal fossa mass in the Department of Rhinology of Beijing Tongren Hospital, Capital Medical University, from April 2008 to December 2021. Ten males and 19 females were included in the study, with age of (46.5±13.7) years. Pre-and post-operative sinus CT, sinus or nasopharyngeal enhanced MRI were evaluated, respectively. The main outcome measurements were the total resection of mass and the incidence of surgery-related complications. Results: Among the 29 cases of infratemporal fossa mass, 22 were schwannomas, 3 were cysts, 2 were neurofibromas, 1 was pleomorphic adenoma and 1 was basal cell adenoma. Preoperative imaging showed well-defined lesion boundaries, and postoperative pathology confirmed the benign nature of all cases. The endoscopic transnasal approach was used in 28 patients, while the combination of the transnasal approach and the transoral approach was used in 1 patient. Complete tumor removal was achieved in all cases with a 100% resection rate. The average follow-up time was 38 months (7-168 months), and no tumor recurrence was observed. Conclusions: The Endoscopic transnasal approach is a safe and effective surgical approach for the treatment of benign tumors or masses in the infratemporal fossa.

Extent of Surgical Resection as a Predictor of Tumor Progression in Skull Base Chordomas: A Multicenter Volumetric Analysis.

INTRODUCTION: Skull-base chordomas are aggressive tumors with a propensity for recurrence/progression. Even with standard of care (SoC), 5-year recurrence rates are variable (19%-54%). This high recurrence/progression rate correlates with increased morbidity and mortality. We sought to analyze a multicenter cohort of skull base chordomas to identify predictors of progression in patients receiving SoC. METHODS: The [Blinded]-Neurosurgery data registry was queried for skull base chordomas treated from 2008-2020. Patients with the histopathologic diagnosis of chordoma were included. The cohort was composed of patients with preoperative and postoperative magnetic resonance imaging. Tumor volume and radiologic characteristics were obtained from axial T2 sequences using a Digital Imaging and Communications in Medicine viewer. Survival analysis was performed using Kaplan-Meier method, and time-to-event multivariate regression was performed to identify independent predictors of progression. RESULTS: The cohort included 195 patients, of which 66 patients met inclusion criteria; median age was 44, and 28 (42%) were females. Fifty-four (82%) received SoC, 7 (11%) resection only, and 5 (8%) radiotherapy only. Median preoperative and postoperative tumor volumes were 11.55 cm3 (0.33-54.89) and 0.34 cm3 (0-42.52), respectively. Recurrence rate with SoC was 37%. Postoperative tumor volume (P = 0.010) correlated with progression. A postoperative volume of >4.9 cm3 (P = 0.044), ≤81.3% of tumor resection (P = 0.02), and lower-clivus location (P < 0.005) correlated with decreased time to progression. CONCLUSIONS: Skull base chordomas can be challenging to resect. Even though maximal resection and radiotherapy improve rate of tumor progression, many of these lesions eventually recur. We have identified a postoperative tumor volume of ≥4.9 cm3 and extent of resection of ≤81.3% in this cohort as predictors of progression in patients receiving SoC.

[EXTENDED ENDONASAL ENDOSCOPIC APPROACH IN TREATING PITUITARY ADENOMAS AND SKULL BASE TUMORS].

INTRODUCTION: The endoscopic endonasal approach (EEA) is a rapidly growing, minimally invasive discipline applied to a broad set of skull base tumors. The introduction of the endoscopic endonasal approach for the management of lesions of the skull base has produced a paradigm shift in the way these complicated lesions are managed. The extended endonasal approach provides the most direct route to the anterior cranial base including sella, cribriform plate, planum sphenoidale, suprasellar cistern, clivus and foramen magnum. Transsphenoidal microscopic pituitary surgery has long been considered the gold standard in surgical treatment of pituitary tumors. Extended endonasal endoscopic pituitary surgery has come into prominence over the last two decades as a superior alternative to microscopic surgery. Gaining experience in this approach has allowed the use of EEA for the surgical treatment of more complex pathologies such as meningiomas, craniopharyngiomas and more.

Unravelling the role of immune cells and FN1 in the recurrence and therapeutic process of skull base chordoma.

BACKGROUND: Skull base chordoma is a rare and aggressive tumour of the bone that has a high likelihood of recurrence. The fundamental differences in single cells between primary and recurrent lesions remain poorly understood, impeding development of effective treatment approaches. METHODS: To obtain an understanding of the differences in single cells between primary and recurrent chordomas, we performed single-cell RNA sequencing and T-cell/B-cell receptor (BCR) sequencing. This allowed us to delineate the differences between the two types of tumour cells, tumour-infiltrating lymphocytes, myeloid cells, fibroblasts and B cells. Copy number variants (CNVs) were detected and compared between the tumour types to assess heterogeneity. Selected samples were subjected to immunohistochemistry to validate protein expression. Fluorescence in situ hybridisation experiments, Transwell assays and xenograft mouse models helped verify the role of fibronectin 1 (FN1) in chordoma. RESULTS: Promoting natural killer (NK) cell and CD8_GZMK T-cell function or inhibiting the transformation of CD8_GZMK T cells to CD8_ZNF683 T cells and promoting the transformation of natural killer T (NKT) cells to NK cells are promising strategies for preventing chordoma recurrence. Additionally, inhibiting the M2-like activity of tumour-associated macrophages (TAMs) could be an effective approach. Antigen-presenting cancer-associated fibroblasts (apCAFs) and dendritic cells (DCs) with high enrichment of the antigen-presenting signature were enriched in primary chordomas. There were fewer plasma cells and BCR clonotypes in recurrent chordomas. Remarkably, FN1 was upregulated, had more CNVs, and was more highly secreted by tumours, macrophages, CD4 T cells, CD8 T cells and fibroblasts in recurrent chordoma than in primary chordoma. Finally, FN1 enhanced the invasion and proliferation of chordomas in vivo and in vitro. CONCLUSION: Our comprehensive picture of the microenvironment of primary and recurrent chordomas provides deep insights into the mechanisms of chordoma recurrence. FN1 is an important target for chordoma therapy.