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Neurosurgical Review Jan 2024Clear cell meningiomas are a rare histological subtype of World Health Organization (WHO) grade II meningioma. Despite its relatively low frequency, clear cell... (Review)
Review
Clear cell meningiomas are a rare histological subtype of World Health Organization (WHO) grade II meningioma. Despite its relatively low frequency, clear cell meningioma has attracted considerable attention because of its unique pathological characteristics, clinical behavior, and challenging management considerations. The purpose of our systematic review is to provide clinicians with a better understanding of this rare disease. PubMed was searched for articles in the English language published from 1988 to 2023 June. The keywords were as follows: "clear cell meningioma," "clear cell" and "meningioma." We analyzed clinical manifestations, radiological manifestations, pathological features, comprehensive treatment strategies, and prognosis to determine the factors influencing recurrence-free survival (RFS). Recurrence-free survival curves of related factors were calculated by the Kaplan‒Meier method. The log-rank test and Cox univariate analysis were adopted to assess the intergroup differences and seek significant factors influencing prognosis and recurrence. Fifty-seven papers met the eligibility criteria, including 207 cases of clear cell meningioma (CCM), which were confirmed by postoperative pathology. The fifty-seven articles involved 84 (40.6%) males and 123 (59.4%) females. The average age at diagnosis was 27.9 years (range, 14 months to 84 years). Among the symptoms observed, headache, neurologic deficit, and hearing loss were the most commonly reported clinical manifestations. Most tumors (47.8%) were located in the skull base region. Most tumors showed significant enhancement, and homogeneous enhancement was more common. A total of 152 (74.1%) patients underwent gross total resection (GTR), and 53 (25.9%) patients underwent subtotal resection (STR). During the follow-up, the tumor recurred in 80 (39.4%) patients. The log-rank test and the Cox univariate analysis revealed that tumor resection range (GTR vs. STR) and adjuvant treatment (YES vs. NO) were significant predictors of recurrence-free survival (RFS). Clear cell meningioma is a rare type of meningioma with challenging diagnosis and therapy. The prognosis of this disease is different from that of regular meningiomas. Recurrence remains a possibility even after total tumor resection. We found that the surgical resection range and adjuvant treatment affected the recurrence period. This finding provides significant guidance for the treatment of clear cell meningioma.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Female; Humans; Infant; Male; Middle Aged; Young Adult; Central Nervous System; Headache; Meningeal Neoplasms; Meningioma
PubMed: 38183517
DOI: 10.1007/s10143-023-02251-6 -
Cancers Dec 2023This systematic review aims to characterize ongoing clinical trials and therapeutic treatment options for chordoma, a rare notochordal remnant tumor that primarily... (Review)
Review
This systematic review aims to characterize ongoing clinical trials and therapeutic treatment options for chordoma, a rare notochordal remnant tumor that primarily affects the cranial base, mobile spine, and sacrum. While radical surgical resection remains the cornerstone for chordoma management, unique technical challenges posed by its proximity to critical neurovascular structures confer a tendency towards disease recurrence which often requires additional treatment modalities. In an attempt to better understand the current treatment landscape, a systematic review was designed to identify clinical trials directed at chordoma. A total of 108 chordoma trials were identified from four clinical trial databases; fifty-one trials were included in the final analysis, of which only 14 were designated as completed (27.5%). Aggregate data suggests most chordoma interventions are repurposed from other neoplasms that share common molecular pathways, with a recent emphasis on combination therapeutics within and across drug classes. Naturally, the publication and dissemination of clinical trial results remain a concern ( = 4, 28.6%), highlighting the need for enhanced reporting and transparency measures. Active clinical trial efforts are quite promising, with a renewed focus on novel biotherapeutic targets and deciphering the natural history, as well as survivorship of this complex disease.
PubMed: 38136345
DOI: 10.3390/cancers15245800 -
Journal of Otolaryngology - Head & Neck... Dec 2023Juvenile Nasopharyngeal Angiofibroma (JNA) is a fibrovascular tumor of the nasopharynx that classically presents in adolescent males. The reported mean age of onset is... (Review)
Review
BACKGROUND
Juvenile Nasopharyngeal Angiofibroma (JNA) is a fibrovascular tumor of the nasopharynx that classically presents in adolescent males. The reported mean age of onset is between 13 and 22 years old [1-6]. Significant androgen stimulation is hypothesized to explain the strong predisposition for JNA to present in young adolescent males. However, considerable variability in age at diagnosis exists with rare involvement of very young patients incongruent with typical male pubertal growth patterns.
OBJECTIVE
The purpose of this systematic review is to identify cases of early-onset JNA (EOJNA), (defined as age < 10 years) in the literature and to examine the disease characteristics and treatments used in this patient group. A case of a 7 year old boy with EOJNA at our institution is also described and presented.
METHODS
We searched Embase, Cochrane database and MEDLINE from 1996 to February 2021 for studies that reported cases of EOJNA. Relevant clinico-demographic data, disease severity and treatment outcomes were recorded and analyzed using descriptive statistics. We compared our findings with reported means for JNA in all ages.
RESULTS
We identified 29 studies containing a total of 34 cases of EOJNA. The vast majority (31/34) of patients were males and the mean age of diagnosis was 8.15 years old. The most common presenting symptoms were nasal obstruction (65.2%) and epistaxis (60.9%). Patients were most commonly Radkowski stage II (39.4%) and III (39.4%). Primary treatment modalities included open surgery (66.7%), endoscopic surgery (24.2%), and radiotherapy (9.1%). Recurrence was evident in 30%. Radkowski stage and type of treatment did not differ significantly within the EOJNA group (p = 0.440 and p = 0.659, respectively).
CONCLUSION
This systematic review suggests that rare cases of EOJNA have distinct disease characteristics. Patients in this cohort appeared to have more advanced disease and higher recurrence rates when compared with reported averages. We hope that this review prompts increased clinical awareness of this potentially more aggressive subtype of JNA. As more cases of EOJNA are reported, a more powered statistical analysis of this cohort would be feasible.
Topics: Adolescent; Humans; Male; Young Adult; Adult; Child; Female; Angiofibroma; Nasopharyngeal Neoplasms; Epistaxis; Treatment Outcome; Nasal Obstruction; Retrospective Studies
PubMed: 38115030
DOI: 10.1186/s40463-023-00687-w -
Journal of Stomatology, Oral and... Jun 2024This systematic review aimed to evaluate the dental prosthetic rehabilitation (DPR) in patients after jaw reconstruction with fibula free flap. Four databases were...
This systematic review aimed to evaluate the dental prosthetic rehabilitation (DPR) in patients after jaw reconstruction with fibula free flap. Four databases were searched from January 2000 to January 2023. Of the 2507 studies identified, 36 observational studies were included. Cancer was the most common surgical indications for jawbone resection with 58.3 % of cases followed by benign tumours which representing 24 %. The DPR rate was estimated at 51.6 % across the studies (ranging from 38 % to 55 % depending on the benign or malignant nature of the tumors). Implant-supported prostheses represented 58.9 % of cases of which 66.9 % were fixed and 33.1 % were implant-stabilized overdentures. Virtual surgical planning (VSP) was used in 20 % of studies and aimed to improve the position of the grafted fibula, quality, and aesthetics of DPR and to decrease ischemia and the operating time. One in two authors performed DPR 12 months after jaw reconstruction. If implant survival rate reached 93 % in non-irradiated fibula, it fell to 38 %, 55 %, and 77 % if implantation occurred in the 12, 17, and 24 months after radiotherapy, respectively. Various parameters should be better investigated in further studies including the typology of the prostheses (implant-supported vs removable), the use of VSP, and the optimal time for DPR taking into account the characteristics of the tumor, the size of bone defect, and the need for external irradiation therapy.
Topics: Humans; Fibula; Free Tissue Flaps; Dental Prosthesis, Implant-Supported; Plastic Surgery Procedures; Mandibular Reconstruction; Jaw Neoplasms; Bone Transplantation
PubMed: 38072231
DOI: 10.1016/j.jormas.2023.101735 -
Child's Nervous System : ChNS :... Apr 2024Non-chordomatous bony tumors of the clivus are extremely rare. Site, extent, and aggressiveness of tumor limits the extent of resection. It poses challenge to the...
BACKGROUND
Non-chordomatous bony tumors of the clivus are extremely rare. Site, extent, and aggressiveness of tumor limits the extent of resection. It poses challenge to the neurosurgeons due to the complexity of anatomy. There is paucity of literature exclusively on non-chordomatous bone tumors of the clivus in young adults.
OBJECTIVES
To analyze the clinical presentation, imaging findings, surgical approach, complications, and outcome of primary clival bony tumors in young adults.
METHODS
We retrospectively reviewed children and young adults with primary clival bony tumors excluding chordoma who underwent surgical resection between years 2010 and 2023 in our center. We analyzed the demographic details, imaging findings, operative variables, perioperative complications, length of stay, complications, and outcome at latest follow-up.
RESULTS
The mean age was 17.5 ± 1.73 years (range 16 to 19 years). Headache was the presenting complaint in all four patients (100%). The mean duration of symptom was 7.25 ± 3.2 months (range 5 to 12 months). The tumor was localized in clivus in all four patients (100%). The mean length of stay in hospital was 30.5 ± 13.48 days (range 11 to 40 days). All patients underwent surgical treatment. Surgical approaches used were anterior approach in four patients (100%). Gross total excision was performed in one patient (25%), sub-total excision was performed in two patients (50%), and tumor decompression was performed in one patient (25%). Of these, three were designated as having benign tumors and one had a malignant tumor. There was no perioperative mortality. There was one mortality (25%) on 2 months follow-up due to tumor progression. Three patients (75%) had improved symptomatically at latest follow-up. Two patients (50%) received adjuvant chemoradiotherapy. The mean follow-up was 38 ± 39.29 months (range 2 to 72 months).
CONCLUSION
Non-chordomatous bony tumors of the clivus are rare and often underestimated. Surgery is the treatment of choice. Tumor consistency and adhesion to critical neurovascular structures precludes gross total resection. Various approaches are in the armamentarium. Approach to be decided based on the expertise of the neurosurgeon to achieve safe maximal resection. Multidisciplinary approach is mandatory for streamlined management. Adjuvant therapy is decided based on the residual tumor following surgery.
Topics: Young Adult; Child; Humans; Adolescent; Adult; Follow-Up Studies; Retrospective Studies; Chordoma; Bone Neoplasms; Cranial Fossa, Posterior; Skull Base Neoplasms; Treatment Outcome
PubMed: 38051310
DOI: 10.1007/s00381-023-06237-z -
Neurosurgical Review Nov 2023The purpose of this study was to systematically review studies in the literature to assess the superiority between microsurgery and radiosurgery regarding the efficacy... (Meta-Analysis)
Meta-Analysis
The purpose of this study was to systematically review studies in the literature to assess the superiority between microsurgery and radiosurgery regarding the efficacy in improving petroclival meningioma (PCM)-related trigeminal neuralgia (TN). PubMed, Embase, Web of Science, and Cochrane clinical trial databases were systematically searched from the inception until December 08, 2022. The overall proportion of patients with improved TN after treatment in all six included studies was 56% (95% confidence interval [CI], 35-76.9%). Higgins I statistics showed significant heterogeneity (I = 90%). Subgroup analysis showed that the proportion of improved TN was higher in the microsurgery group than that in the radiosurgery group (89%; 95% CI, 81-96.5% vs. 37%, 95% CI, 22-52.7%, respectively, p < 0.01). Subgroup analysis (for studies that documented the number of posttreatment Barrow Neurological Institute scores 1 and 2) revealed that the proportion of pain-free without medication after treatment was higher in the microsurgery group than that in the radiosurgery group (90.7%; 95% CI, 81-99.7% vs. 34.5%, 95% CI, 21.3-47.7.7%, respectively, p < 0.01). Based on the results of this meta-analysis, we concluded that microsurgery is superior to radiosurgery in controlling PCM-related TN.
Topics: Humans; Trigeminal Neuralgia; Meningioma; Treatment Outcome; Radiosurgery; Microsurgery; Skull Base Neoplasms; Meningeal Neoplasms; Retrospective Studies
PubMed: 38012480
DOI: 10.1007/s10143-023-02225-8 -
Journal of Plastic, Reconstructive &... Jan 2024Nasal dermoid cysts are surgically treated using external incision, open rhinoplasty, transnasal endoscopy, or combined approaches. It is unclear how these approaches... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Nasal dermoid cysts are surgically treated using external incision, open rhinoplasty, transnasal endoscopy, or combined approaches. It is unclear how these approaches differ with regard to the incidence of adverse events.
METHODS
We conducted a systematic review of studies on the surgical management of midline nasal dermoids. Following data abstraction, we carried out a series of single-arm meta-analyses to estimate summary risks of recurrence and combined adverse events (recurrence, revision, infection, or readmission) according to the surgical approach.
RESULTS
Forty-three eligible studies published between 1958 and 2020 reported on 439 cases of nasal dermoid cysts. Treatment approaches included external incision (25 studies), rhinoplasty (15 studies), and transnasal endoscopy (5 studies). To our knowledge, no study has compared outcome incidence between the surgical approaches. External incision had the lowest summary incidence of both recurrence (1.78% [95% CI: 0.57%, 3.65%]) and combined adverse events (4.94% [95% CI: 2.72%, 7.77%]). Rhinoplasty had a higher incidence of recurrence (4.81% [95% CI: 0.91%, 11.6%]) and combined adverse events (8.32% [95% CI: 2.77%, 16.5%]), and transnasal endoscopy had the highest incidence of recurrence (the only reported adverse event; 7.89% [95% CI: 0%, 28.9%]).
CONCLUSION
Our results suggest that the incidence of adverse events was lowest among patients who were subjected to external incision for nasal dermoid removal. Incidence was higher for patients who underwent rhinoplasty and the highest for patients who underwent transnasal endoscopy. Future work on this topic should include well-designed prospective studies that compare rates of adverse events and cosmetic outcomes between surgical approaches.
Topics: Humans; Dermoid Cyst; Prospective Studies; Nose Neoplasms; Rhinoplasty; Endoscopy
PubMed: 37983980
DOI: 10.1016/j.bjps.2023.10.114 -
Neurosurgical Review Oct 2023Perioptic meningiomas, defined as those that are less than 3 mm from the optic apparatus, are challenging to treat with stereotactic radiosurgery (SRS). Tumor control... (Meta-Analysis)
Meta-Analysis Review
Perioptic meningiomas, defined as those that are less than 3 mm from the optic apparatus, are challenging to treat with stereotactic radiosurgery (SRS). Tumor control must be weighed against the risk of radiation-induced optic neuropathy (RION), as both tumor progression and RION can lead to visual decline. We performed a systematic review and meta-analysis of single fraction SRS and hypofractionated radiosurgery (hfRS) for perioptic meningiomas, evaluating tumor control and visual preservation rates. Using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we reviewed articles published between 1968 and December 8, 2022. We retained 5 studies reporting 865 patients, 438 cases treated in single fraction, while 427 with hfRS. For single fraction SRS, the overall rate of tumor control was 95.1%, with actuarial rates at 5 and 10 years of 96% and 89%, respectively; tumor progression was 7.7%. The rate of visual stability was 90.4%, including visual improvement in 29.3%. The rate of visual decline was 9.6%, including blindness in 1.2%. For hfRS, the overall rate of tumor control was 95.6% (range 92.1-99.1, p < 0.001); tumor progression was 4.4% (range 0.9-7.9, p = 0.01). Overall rate of visual stability was 94.9% (range 90.9-98.9, p < 0.001), including visual improvement in 22.7% (range 5.0-40.3, p = 0.01); visual decline was 5.1% (range 1.1-9.1, p = 0.013). SRS is an effective and safe treatment option for perioptic meningiomas. Both hypofractionated regimens and single fraction SRS can be considered.
Topics: Humans; Meningeal Neoplasms; Meningioma; Optic Nerve; Radiosurgery; Treatment Outcome
PubMed: 37897519
DOI: 10.1007/s10143-023-02197-9 -
Journal of Neuro-oncology Oct 2023Despite their precarious behavioral classification (benign and low grade on histopathology yet behaviorally malignant), great strides have been taken to improve... (Meta-Analysis)
Meta-Analysis Review
Assessing survival outcomes and complication profiles following surgical excision and radiotherapy as interventions for skull base chordoma: a systematic review of operative margins and surgical approaches.
INTRODUCTION
Despite their precarious behavioral classification (benign and low grade on histopathology yet behaviorally malignant), great strides have been taken to improve prognostication and treatment paradigms for patients with skull base chordoma. With respect to surgical techniques, lateral transcranial (TC) approaches have traditionally been used, however endoscopic endonasal approaches (EEA) have been advocated for midline lesions. Nonetheless, due to the rarity of this pathology (0.2% of all intracranial neoplasms), investigations within the literature remain limited to small retrospective series. Furthermore, radiotherapeutic treatments investigated to date have proven largely ineffective.
METHODS
Accordingly, we performed a systematic review in order to profile surgical and survival outcomes for skull base chordoma. Fixed and random-effect meta-analyses were performed for categorical variables including GTR, STR, 5-year OS, 10-year OS, 5-year PFS, and 10-year PFS. Additionally, we pooled eligible studies for formal meta-analysis to compare outcomes by surgical approach (lateral versus midline). Statistical analyses were performed using R Studio 'metafor' package or Cochrane Review Manager. Furthermore, meta-analysis of pooled mortality rates and sub-analyses of operative margin and surgical complications were used to compare midline versus lateral approaches via the Mantel-Haenszel method. We considered all p-values < 0.05 to be statistically significant.
RESULTS
Following the systematic search and screen, 55 studies published between 1993 and 2022 reporting data for 2453 patients remained eligible for analysis. Sex distribution was comparable between males and females, with a slight predominance of male-identifying patients (0.5625 [95% CI: 0.5418; 0.3909]). Average age at diagnosis was 42.4 ± 12.5 years, while average age of treatment initiation was 43.0 ± 10.6 years. Overall, I value indicated notable heterogeneity across the 55 studies [I = 56.3% (95%CI: 44.0%; 65.9%)]. With respect to operative margins, the rate of GTR was 0.3323 [95% CI: 0.2824; 0.3909], I = 91.9% [95% CI: 90.2%; 93.4%], while the rate of STR was significantly higher at 0.5167 [95% CI: 0.4596; 0.5808], I = 93.1% [95% CI: 91.6%; 94.4%]. The most common complication was CSF leak (5.4%). In terms of survival outcomes, 5-year OS rate was 0.7113 [95% CI: 0.6685; 0.7568], I = 91.9% [95% CI: 90.0%; 93.5%]. 10-year OS rate was 0.4957 [95% CI: 0.4230; 0.5809], I = 92.3% [95% CI: 89.2%; 94.4%], which was comparable to the 5-year PFS rate of 0.5054 [95% CI: 0.4394; 0.5813], I = 84.2% [95% CI: 77.6%; 88.8%] and 10-yr PFS rate of 0.4949 [95% CI: 0.4075; 0.6010], I = 14.9% [95% CI: 0.0%; 87.0%]. There were 55 reported deaths for a perioperative mortality rate of 2.5%. The relative risk for mortality in the midline group versus the lateral approach group did not indicate any substantial difference in survival according to laterality of approach (-0.93 [95% CI: -1.03, -0.97], I = 95%, (p < 0.001).
CONCLUSION
Overall, these results indicate good 5-year survival outcomes for patients with skull base chordoma; however, 10-year prognosis for skull base chordoma remains poor due to its radiotherapeutic resistance and high recurrence rate. Furthermore, mortality rates among patients undergoing midline versus lateral skull base approaches appear to be equivocal.
Topics: Female; Humans; Male; Adult; Middle Aged; Retrospective Studies; Chordoma; Cranial Fossa, Posterior; Prognosis; Skull Base Neoplasms; Head and Neck Neoplasms; Treatment Outcome
PubMed: 37880419
DOI: 10.1007/s11060-023-04477-2 -
Medicina Oral, Patologia Oral Y Cirugia... Mar 2024Segmental surgical resection is a frequently indicated procedure to treat aggressive mandibular tumors. One of the most important complications derived from this...
BACKGROUND
Segmental surgical resection is a frequently indicated procedure to treat aggressive mandibular tumors. One of the most important complications derived from this technique is permanent paresthesia of the inferior alveolar nerve (IAN), which significantly affects the quality of life of patients who experience it. This could be avoided through maneuvers that preserve the IAN. The objective of this paper is to review the main techniques for IAN preservation and to present 2 cases with the technique used by the author.
MATERIAL AND METHODS
A systematic review was performed according to the PRISMA guidelines, apropos of two clinical cases reported in this study. The MEDLINE/PubMed and Scopus databases were searched. Several variables were considered and are presented in detail in the form of tables and figures. In addition, 2 case reports with NAI preservation techniques are presented.
RESULTS
13 articles were finally obtained for analysis. 127 patients were evaluated, reporting mandibular resections associated with various pathologies. Various surgical techniques were used, all with the same goal of maintaining the IAN. In most of the patients, the maintenance of sensitivity was achieved, which was verified with different methods.
CONCLUSIONS
Preservation of the IAN in maxillofacial surgical procedures where surgical resection of the mandibular bone has been performed is an alternative that has demonstrated successful results in terms of reducing postoperative sequelae and is currently positioned as a necessary and feasible procedure.
Topics: Humans; Quality of Life; Mandible; Mandibular Nerve; Mandibular Neoplasms; Tooth Extraction
PubMed: 37823290
DOI: 10.4317/medoral.26239