Comparative overall survival analysis of chordomas of the base of the skull from the Surveillance, Epidemiology, and End Results (SEER) program between 2000 and 2020.

Chordomas of the skull base are rare, slow growing, locally invasive cancers with limited long-term survival analysis reported in the literature. We seek to provide comparative survival analysis of patients on a long-term (20-year) basis using population-level data. The Surveillance, Epidemiology, and End Results (SEER) program was queried for cases of chordoma relegated to the base of the skull, diagnosed between 2000 and 2020. Demographic, disease, and treatment information were analyzed using Cox proportional hazards and log-rank comparisons. 630 patients with chordoma of the skull base were identified. Age ≤ 49 years at diagnosis was associated with increased five-, 10-, and 20-year overall survival (hazard ratio (HR) = 0.39, 0.33, and 0.30, respectively; p < 0.001 for all). Treatment with surgery and adjuvant radiotherapy was associated with increased five-, 10-, and 20-year survival (HR = 0.71, 0.79, and 0.79, respectively; p < 0.001 for all). On univariate analysis, widowed patients had decreased survival (20-year overall survival = 34.8% [15.3%-34.8%] compared to married patients (74.4% [68.1%-80.8%]. Surgery remains the primary treatment associated with increased survival among patients with chordoma of the skull base, with adjuvant radiotherapy serving a complimentary role. Demographic factors such as marital status are also associated with changes in survival.

Postoperative trigeminal neuropathy outcomes following surgery for tumors involving the trigeminal nerve.

OBJECTIVE: To observe the evolution and outcomes of postoperative trigeminal neuropathy following surgery of tumor involving the trigeminal nerve. METHODS: A prospective observational study was conducted between October 2018 and February 2019 involving 25 patients with tumors confirmed to involve the trigeminal nerve during surgery by senior author. Pre- and postoperative trigeminal nerve function status and clinical data were recorded. RESULTS: This study included 18 cases of meningioma and seven of trigeminal schwannoma. Among the meningioma cases, 55.6% of the patients reported facial sensory dysfunction before surgery, 33.3% presented ocular discomfort, and 5.6% had masticatory muscle atrophy. Postoperatively, all patients experienced facial paresthesia, 94.4% complained of eye dryness, and one (5.56%) exhibited keratitis. Additionally, one patient (5.56%) showed new-onset masticatory weakness. During follow-up, 50.0% of patients reported improvement in facial paresthesia, and one (5.56%) experienced deterioration. Eye dryness resolved in 35.3% of patients, and keratitis remission was observed in one patient. However, one patient (5.56%) developed neurotrophic keratitis. Overall, 55.6% of patients displayed mild masticatory weakness without muscle atrophy. In the cases of schwannoma, 28.6% of patients had facial paresthesia before surgery, 42.9% showed ocular discomfort, and one (14.3%) complained of masticatory dysfunction. Postoperatively, 85.7% of patients reported facial paresthesia and eye dryness, with one patient (16.7%) experiencing keratitis. During follow-up, 66.7% of patients demonstrated improvement in facial paresthesia, 28.6% showed eye dryness remission, and one patient (16.7%) recovered from keratitis. However, one patient (16.7%) developed new-onset neurotrophic keratitis. One patient (16.7%) experienced relief of masticatory dysfunction, but 42.9% reported mild deterioration. Another patient (14.3%) had facial anesthesia that had not improved. CONCLUSION: Postoperative trigeminal neuropathy is a common complication with a high incidence rate and poor recovery outcomes after surgery for tumors involving the trigeminal nerve. When trigeminal nerve damage is unavoidable, it is essential to provide a multidisciplinary and careful follow-up, along with active management strategy, to mitigate the more severe effects of postoperative trigeminal neuropathy.

Long-term outcomes of stereotactic radiosurgery for skull base tumors involving the cavernous sinus.

PURPOSE: Stereotactic radiosurgery (SRS) is an effective and less invasive therapeutic option for cavernous sinus (CS) tumors. However, its long-term effectiveness and neurological outcomes have yet to be fully elucidated. We aimed to examine the long-term outcomes of SRS for CS tumors. METHODS: Overall, a cohort of 113 patients with benign CS tumors, including 91 with meningioma, 14 with trigeminal schwannoma (TS), and eight with cavernous hemangioma, treated with SRS at our institution from 1990 to 2018, was included. Tumor control and functional preservation/recovery were evaluated in detail. RESULTS: The median post-SRS follow-up period was 77 months (interquartile range, 39-177). Progression-free survival (PFS) was 97% at 5 years, 89% at 10 years, and 87% at 15 years for the entire cohort; 96% at 5 years and 87% at 10 years for meningiomas; and 100% at 10 years for the other tumors. No significant difference was observed between meningiomas and non-meningiomas (log-rank test, p = 0.107). Improvement in cranial nerve (CN) function was observed in 35 (27%) patients. TSs tended to show CN improvements more often than meningiomas did (total improvements, 62% vs. 23%; p = 0.004; eye movement function, 100% vs. 20%; p = 0.002). CN deterioration or development of new CN deficits was observed in 11 (10%) patients. CONCLUSION: SRS provides good tumor control and acceptable long-term outcome with sufficient preservation of CN function in patients with benign CS tumors.

[The DNA methylation profiling in the study and treatment of patients with meningiomas of the craniovertebral junction]. / Znachenie analiza metilirovaniya DNK v issledovanii i lechenii patsientov s meningiomami oblasti kraniovertebral'nogo perekhoda.

The paper presents the experience of using DNA methylation status in patients with meningiomas of the craniovertebral junction area in a neurosurgical clinic. A clinical case of combined treatment of a patient with meningioma of the craniovertebral junction and the choice of tactics based on the result of DNA methylation analysis of meningioma are described.

[Rosai-Dorfman intracranial histiocytosis: case report and literature review]. / Intrakranial'nyi gistiotsitoz Rozai­Dorfmana: sluchai iz praktiki i obzor literatury.

Histiocytosis is a group of idiopathic diseases accompanied by metabolic disorders and accumulation of metabolic products in histiocytes. Isolated Rosai-Dorfman histiocytosis of central nervous system is observed in less than 5% of cases. The authors report treatment and follow-up of a patient with intracranial Rosai-Dorfman disease. There were symptoms of lesion of the left cerebellopontine angle and epileptic seizures. Preoperative MRI identified two tumors (posterior cranial fossa on the left and right-sided parasagittal neoplasm). The authors carried out total resection of supratentorial tumor, after 3 weeks - subtotal resection of tumor in posterior cranial fossa. No recurrence after total resection was observed. Irradiation of infratentorial tumor with a total focal dose of 50 Gy after 6 months resulted tumor shrinkage throughout 12 months. Radiotherapy with the same dose was repeated throughout subsequent 12-month follow-up period due to progression of this focus. This treatment had a positive effect, but new skull base foci occurred. The authors emphasize the effectiveness of total resection and lower efficiency of subtotal excision combined with radiotherapy.

Quality of life and olfactory function after suprasellar craniopharyngioma surgery-a single-center experience comparing transcranial and endoscopic endonasal approaches.

The endoscopic endonasal approach to suprasellar craniopharyngiomas has become popular as alternative to transcranial approaches. However, the literature lacks data regarding quality of life and olfactory function. The assessment of the long-term quality of life and olfactory function of all patients harboring a suprasellar craniopharyngioma who underwent surgery in our department has been done. Patient characteristics and perioperative data were gathered in a prospectively maintained database. At the last follow-up visit, the olfactory function and the quality of life (ASBQ, SNOT-22) as well as visual and pituitary function were assessed. Thirteen and 17 patients underwent surgery via a transcranial (T) and endonasal (E) route, respectively. No differences were seen in ASBQ, SNOT-22, and olfactory function between T and E, but in E were more full-time worker and less obesity. CSF leaks occurred in 15% of T and 29% of E (p = 0.43). Patients from group E had a superior visual outcome which was most pronounced in the visual field. The degree of new anterior and posterior pituitary gland deficiency after surgery and in the follow-up was lower in group E. The general and sinonasal quality of life and the olfactory function are equal in E and T. E is associated with a superior visual outcome, lower rates of diabetes insipidus, and lower rates of obesity, but has a higher risk for postoperative CSF leaks.

An endoscopic endonasal approach to craniopharyngioma via the infrachiasmatic corridor: a single center experience of 84 patients.

OBJECT: The infrachiasmatic corridor is the most important surgical access route for craniopharyngiomas and was identified and used in clinical series. The aims of this study were to describe the characteristics that assist dissection and resection rates in endoscopic surgery of solid, cystic, and recurrent cases and their importance in the infrachiasmatic corridor in endoscopic surgery. METHODS: One hundred operations on 84 patients with pathologically identified craniopharyngioma were included in the study. The MRI findings were evaluated, and the location of the lesions was classified as (1) infrasellar; (2) sellar; or (3) suprasellar. In the sagittal plane, we measured the longest diameter of cystic and solid components and the height of chiasm-sella. Images were assessed for the extent of resection and were classified as gross total resection. This was deemed as the absence of residual tumor and subtotal resection, which had residual tumor. RESULTS: The infrasellar location was reported in 7/84 (8.3%) patients, the sellar location in 8/84 (9.5%), and the suprasellar location in 69/84 (82.1%) patients. The narrow and high chiasm-sella were observed in 28/69 (40.5%) and 41/69 patients (59.4%), respectively. The mean distance of the chiasm-sella was 9.46± 3.76. Gross total tumor resection was achieved in 60/84 (71.4%) and subtotal tumor resection was performed in 24/84 (28.6%) patients. The results revealed that suprasellar location (OR: 0.068; p = 0.017) and recurrent cases (OR: 0.011; p<0.001) were negative predictive factors on GTR. Increasing the experience (OR: 42,504; p = 0.001) was a positive predictor factor for GTR. CONCLUSION: An EETS approach that uses the infrachiasmatic corridor is required for skull base lesions extending into the suprasellar area. The infrachiasmatic corridor can determine the limitations of endoscopic craniopharyngioma surgery. This corridor is a surgical safety zone for inferior approaches.

Intracranial chordoma: radiosurgery, hypofractionated stereotactic radiotherapy and treatment outcomes.

BACKGROUND: The aim of the study was to assess the results of stereotactic radiosurgery and hypofractionated stereotactic radiotherapy (SRS/SRT) for skull base chordomas. MATERIALS AND METHODS: Twenty-three patients aged 12-75 were treated with SRS/SRT due to skull base chordoma. In 19 patients SRS/SRT was a part of the primary therapy, while in 4, a part of the treatment of recurrence. In 4 patients SRS/SRT was used as a boost after conventional radiotherapy and in 19 cases it was the only irradiation method applied. Patients were irradiated to total dose of 6-35 Gy and median total equivalent dose of 52 Gy. RESULTS: During median follow-up of 39 months, 4 patients died. One-, two- and five-year OS was 95%, 89% and 69%, respectively. In nine patients, progression of the disease was diagnosed during study period. One-, two- and five-year progression free survival (PFS) from the end of radiotherapy was 81%, 59% and 43%, respectively. Radiotherapy was well tolerated and only two patients in our group experienced moderate treatment-related toxicity. CONCLUSION: SRS/SRT alone or in combination with surgery is a safe and effective method of irradiation of patients with skull base chordomas. High EQD2 is necessary to achieve satisfactory treatment results.

Outcomes of adolescents and young adults treated for brain and skull base tumors with pencil beam scanning proton therapy.

BACKGROUND: The use of proton therapy (PT) in adolescents and young adults (AYAs) is becoming increasingly popular. This study aims to assess the outcomes and late toxicity consequences in AYAs (15-39 years) with brain/skull base tumors treated with pencil beam scanning proton therapy. METHODS: One hundred seventy six AYAs treated curatively at the Paul Scherrer Institute (PSI) were identified. Median age was 30 years (range 15-39) and median prescribed dose was 70.0 Gy (relative biological effectiveness [RBE]) (range 50.4-76.0). The most common tumors treated were chordomas/chondrosarcomas (61.4%), followed by gliomas (15.3%), and meningiomas (14.2%). RESULTS: After a median follow up of 66 months (range 12-236), 24 (13.6%) local only failures and one (0.6%) central nervous system (CNS) distant only failure were observed. The 6-year local control, distant progression-free survival, and overall survival were 83.2%, 97.4%, and 90.2%, respectively. The 6-year high-grade (≥grade [G] 3) PT-related late toxicity-free survival was 88.5%. Crude late toxicity rates were 26.2% G1, 37.8% G2, 12.2% G3, 0.6% G4, and 0.6% G5. The one G4 toxicity was a retinopathy and one G5 toxicity was a brainstem hemorrhage. The 6-year cumulative incidences for any late PT-related pituitary, ototoxicity, and neurotoxicity were 36.3%, 18.3%, and 25.6%; whilst high-grade (≥G3) ototoxicity and neurotoxicity were 3.4% and 2.9%, respectively. No secondary malignancies were observed. The rate of unemployment was 9.5% pre-PT, increasing to 23.8% post-PT. Sixty-two percent of survivors were working whilst 12.7% were in education post-PT. CONCLUSIONS: PT is an effective treatment for brain/skull base tumors in the AYA population with a reasonable late toxicity profile. Despite good clinical outcomes, around one in four AYA survivors are unemployed after treatment.

[Treatment of petroclival meningiomas: current state of the problem]. / Lechenie petroklival'nykh meningiom: sovremennoe sostoianie problemy.

Petroclival meningiomas (PCMs) are benign, slowly growing tumors. Surgery still remains the main treatment option for them. The desire for total resection of large extended PCMs often leads to the development or worsening of persistent neurological deficits. This paper presents a review of the world literature devoted to petroclival meningiomas. We discuss the issues of PCM classification, biology, diagnosis, and selection of the optimal algorithm for their treatment.

[Use of navigation in skull base surgery]. / Primenenie navigatsii v khirurgii osnovaniia cherepa.

The review briefly presents the history of development of navigation systems in neurosurgery. The idea of the existing principles underlying the navigation systems used in neurosurgery is given. Currently, the basic principles of navigation are optical and electromagnetic. Studies are presented comparing the accuracy of various navigation systems. Optical navigation demonstrates greater accuracy compared to electromagnetic, but both methods demonstrate a submillimeter error in the experiment. The history of use of navigation in the surgery of the skull base is analyzed in detail, the most relevant areas of use of navigation within the surgery of the skull base are considered: craniofacial reconstruction, endoscopic endonasal surgery, surgery of common tumors of the skull base affecting the infratemporal, pterygopalatine fossa, temporomandibular joint. Indications for the use of navigation, limitations of the methodology are explained.

Craniofacial resection of malignant tumors of the anterior skull base: a case series and a systematic review.

BACKGROUND: Craniofacial resection (CFR) is still considered as the gold standard for managing sinonasal malignancies of the anterior skull base (ASB), while endoscopic approaches are gaining credibility. The goal of this study was to evaluate outcomes of patients who underwent CFR at our institution and to compare our results to international literature. METHOD: Retrospective analysis of all patients undergoing CFR between 1995 and 2017, and systematic literature review according to the PRISMA statement. RESULTS: Forty-one patients with sinonasal malignancy (81% with stage T4) of the ASB were included. There was no operative mortality. Complications were observed in 9 cases. We obtained 100% follow-up with mean observation of 100 months. Disease-specific survival rates were 90%, 74%, and 62% and recurrence-free survival was 85% at two, 72% at five, and 10 years follow-up, respectively. CFR as primary treatment, en bloc resection, and resection with negative margins correlated to better survival. Recursive partition analysis identified the latter as the most important prognostic factor, regardless of surgical technique. The relative risk of non-radicality was significantly higher after piecemeal resection compared to en bloc resection. Compared to 15 original articles, totaling 2603 patients, eligible for review, the present study has the longest follow-up time, the second highest 5-year OS, and the third highest 5-year DSS, despite having a higher proportion of patients with high-stage disease. CONCLUSION: CFR in true en bloc fashion can still be considered as the treatment of choice in cases of advanced-stage sinonasal malignancies invading the ASB.

The use of dehydrated amniotic membrane allograft for augmentation of dural closure in craniotomies and endoscopic endonasal transphenoidal surgeries.

BACKGROUND: Primary watertight dural closure is the preferred method of postcraniotomy dural repair. However, even when ideal technique is implemented, postoperative infection, cerebrospinal fluid (CSF) leaks, pseudomeningoceles, and dural scarring are possible complications. For this reason, materials that augment the dura's ability to create a watertight seal, prevent disease transmission, and inhibit inflammatory response are sought. Dehydrated amniotic membrane (DAM) allograft appears to fulfil these requirements as it has several beneficial properties that aid wound healing, including promotion of epithelialization, scar tissue prevention, and inhibition of bacterial growth. We provide the literature's first description of the use of DAM allograft to supplement dural closures for craniotomies and transsphenoidal surgeries. METHODS: We conducted a pilot study, retrospectively reviewing our institution's database of craniotomies and transsphenoidal surgeries that utilized DAM to augment dural closure. RESULTS: One hundred fifty-five cases, including 102 new craniotomies for supratentorial lesions, one re-do craniotomy for supratentorial recurrent glioma, 18 craniotomies for infratentorial lesions, 1 craniotomy for anterior skull base schwannoma, 32 transphenoidal surgeries, and 1 combined craniotomy and transnasal endoscopic surgery, used DAM allograft to augment dural closure. Only one complication occurred (0.6% complication rate), which was a superficial wound infection requiring washout without craniectomy. No CSF leaks occurred. CONCLUSIONS: This pilot study demonstrates that dehydrated amniotic membrane allograft can be safely utilized as an adjunct during dural closures for craniotomies and transsphenoidal surgeries.

[Facial nerve injury in neurosurgery: a rehabilitation potential of botulinum therapy]. / Porazhenie litsevogo nerva pri neirokhirurgicheskikh operatsiiakh: reabilitatsionnyi potentsial botulinoterapii.

Surgical treatment of posterior cranial fossa and cerebellopontine angle tumors is associated with a risk of facial nerve dysfunction. The causes for facial muscle paresis include nerve compression by the tumor, destruction of the nerve structure by the tumor growing from nerve fibers, nerve injury during surgical removal of the tumor, etc. The first 3 months after facial nerve injury are a potential therapeutic window for the use of botulinum toxin type A (BTA). During this period, the drug is introduced both in the healthy side to improve the facial symmetry at rest and during mimetic movements and in the affected side to induce drug-induced ptosis. Post-paralytic syndrome develops 4-6 months after facial nerve injury. At this stage, administration of BTA is also an effective procedure; in this case, drug injections are performed on the affected side at small doses and symmetrically on the healthy side at doses doubling those for the affected side. BTA injections are mandatory in complex treatment of facial muscle paralysis.

Clival paragangliomas: a report of two cases involving the midline skull base and review of the literature.

Head and neck paragangliomas are rare neuroendocrine tumors that arise from paraganglion cells of the parasympathetic nervous system. Paragangliomas arising from the midline skull base have only rarely been reported. Surgery is the mainstay of treatment and adjuvant radiation is often recommended. These tumors can rarely secrete metanephrines and normetanephrines which can complicate operative management. Here we present two cases of clival paragangliomas with unique clinical presentations and review the previous literature on skull base paragangliomas.

Pathologic aspects of skull base tumors.

Skull base tumors form a highly heterogeneous group. As there are several structures in this anatomical site, a large number of different primary malignancies might develop, as well as a variety of secondary (metastatic) tumors. In this article, the most common malignancies are presented, along with a short histopathologic description. For some entities, an immunohistochemical profile is also given that should be helpful in proper diagnosis. As many pathologic diagnoses nowadays also include genetic studies, the most common genetic abnormalities in skull base tumors are presented.

[Neurosurgery in a patient on dual antiplatelet therapy. Case report and the review of the literature]. / Neirokhirurgicheskoe vmeshatel'stvo u patsienta, nakhodiashchegosia na dvoinoi dezagregantnoi terapii. Opisanie sluchaia i obzor literatury.

INTRODUCTION: A neurosurgical intervention in a patient on dual antiplatelet therapy is a serious challenge for both the neurosurgeon and anesthesiologist.. MATERIAL AND METHODS: The article describes a clinical case of a successful urgent neurosurgical intervention (ventriculoperitoneostomy for obstructive hydrocephalus caused by a large meningioma of the posterior surface of the petrous pyramid) in a patient on dual antiplatelet therapy (DAT) due to a recently placed coronary stent.. CONCLUSION: Given a high risk of coronary stent thrombosis, the surgery was performed in the presence of ongoing DAT. There were no intracranial hemorrhagic complications, but subcutaneous hemorrhagic complications developed. The article discusses the features of managing similar patients whose number is growing.

Giant prolactinoma presenting as a skull base tumor with erosion of the cervical vertebrae: pronounced responsiveness to dopamine agonist treatment.

Giant prolactinomas are rare and usually associated with symptoms attributable to hypopituitarism and compression of juxtasellar structures such as the cranial nerves of the cavernous sinus and the optic chiasm. Occasionally, they masquerade as skull base tumors with atypical symptoms. We describe a patient who presented with a low-energy trauma in the neck region that led to the initial diagnosis of a large skull base tumor eroding the cervical vertebrae. After stabilizing surgery, the patient responded to dopamine agonist therapy with normalization of serum prolactin levels and pronounced reduction in tumor volume.

Skull base surgery for malignant tumors: The 2nd international collaborative study (1995-2015).

BACKGROUND: The current study presents the effort of a global collaborative group to review the management and outcomes of malignant tumors of the skull base worldwide. PATIENTS AND METHODS: A total of 28 institutions contributed data on 3061 patients. Analysis evaluated clinical variables, survival outcomes, and multivariable factors associated with outcomes. RESULTS: The median age was 56 years (IQR 44-67). The open surgical approach was used in 55% (n = 1680) of cases, endoscopic resection was performed in 36% (n = 1087), and the combined approach in 9.6% (n = 294). With a median follow-up of 7.1 years, the 5-year OS DSS and RFS were 65%, 71.7% and 53%, respectively. On multivariable analysis, older age, comorbidities, histology, dural/intracranial involvement, positive margins, advanced stage, and primary site were independent prognostic factors for OS, DSS, and RFS. Adjuvant RT was a protective prognostic factor. CONCLUSION: The progress across various disciplines may have contributed to improved OS and DSS in this study compared to previous reports.

A Rare Tumor Causing Optic and Oculomotor Nerve Compression: Clivus Plasmacytoma Case Report.

Plasmacytomas rarely affect the skull base and may be found as an isolated lesion or as a part of multiple myeloma. The typical feature of plasmacytomas is aggressive bone destruction in the skull. It is often confused with the chordoma of the clivus. The most common location for skull-base plasmacytomas is the nasopharynx. The most commonly affected cranial nerve in clivus tumors is the abducens nerve. In our 64-year-old male case, a plasmacytoma was detected in the clivus. There was ptosis and decreased vision due to optic nerve and oculomotor nerve involvement due to the plasmacytoma. Radiotherapy was applied for the treatment.