Esmée M Bosman, Max E Keizer, Jasper van Aalst, Martinus P G Broen, Alida A Postma, Astrid I P Vernemmen, Henricus P M Kunst, Yasin Temel
{"title":"Spontaneous Shrinking and Growing Skull Base Chordoma.","authors":"Esmée M Bosman, Max E Keizer, Jasper van Aalst, Martinus P G Broen, Alida A Postma, Astrid I P Vernemmen, Henricus P M Kunst, Yasin Temel","doi":"10.1055/a-2587-6573","DOIUrl":"https://doi.org/10.1055/a-2587-6573","url":null,"abstract":"<p><strong>Background: </strong>Chordomas are rare slow-growing tumors occurring in the axial skeleton and can demonstrate local aggressive behavior, typically extending from the median axis, compressing surrounding tissue. Complete surgical resection and adjuvant radiotherapy are the preferred treatments. We present an unusual case of a spontaneously shrinking and growing off-midline petroclival chordoma.</p><p><strong>Case description: </strong>A 23-year-old woman presented with right abducens nerve palsy. Computed tomography and magnetic resonance imaging (MRI) revealed an off-midline petroclival lesion compressing the abducens nerve with characteristics of a chondrosarcoma. Preoperative MRI indicated spontaneous lesion regression, and the abducens nerve showed clinical improvement. Hence, the planned surgery was canceled. During the wait-and-scan period, abducens nerve palsy recurred. MRI confirmed lesion growth and showed an intratumoral linear structure indicative of blood. Even though preoperative MRI again demonstrated shrinkage, the lesion was surgically removed. Despite the unusual presentation, histopathological examination diagnosed a conventional chordoma. A second surgery was required to remove the residual tumor, after which the patient received high-dose photon beam therapy.</p><p><strong>Conclusion: </strong>This article discusses the uncommon presentation and behavior of a petroclival chordoma, showing fluctuating cycles of off-midline growth and spontaneous regression. While intratumoral hemorrhage is hypothesized to explain this tumor behavior, the exact etiology needs further investigation. The case presented here emphasizes the importance of considering chordoma in the differential diagnosis despite an atypical disease course.</p>","PeriodicalId":44256,"journal":{"name":"Journal of Neurological Surgery Reports","volume":"86 2","pages":"e107-e111"},"PeriodicalIF":0.6,"publicationDate":"2025-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12064314/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144020462","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Mechanism of HDAC2 Inhibitors on Chronic Pancreatitis Pain.","authors":"Xiang-Tian Zeng, Wen-Hui Chen, Ding-Wen Zhong, Qi-Xin Dai, Yong He, Rong-Qiang Ye, Xiu-Lin Xiao, Yong-Hui Liao","doi":"10.1055/a-2561-8065","DOIUrl":"https://doi.org/10.1055/a-2561-8065","url":null,"abstract":"<p><strong>Background: </strong>Chronic pancreatitis (CP) is marked by persistent inflammation and fibrosis of the pancreas, often causing severe abdominal pain. The pain mechanism involves complex interactions between pancreatic inflammation and spinal nerve activity. Histone deacetylase 2 (HDAC2) is implicated in neural processes and pain modulation, making it a potential target for CP pain management.</p><p><strong>Aim: </strong>This study investigates HDAC2's role in CP pain and evaluates the effects of its inhibition in a CP rat model.</p><p><strong>Methods: </strong>CP was induced in male Sprague-Dawley rats using dibutyltin dichloride (DBTC). HDAC2 expression in spinal and pancreatic tissues was assessed through western blotting, quantitative Real-Time PCR, and enzyme-linked immunosorbent assay (ELISA). Pain sensitivity was evaluated using paw withdrawal tests. Co-cultures of AR42J pancreatic acinar cells and F11 spinal neurons were used to explore pancreatic-neural interactions. Chromatin immunoprecipitation (ChIP) and promoter assays examined HDAC2 transcriptional regulation.</p><p><strong>Results: </strong>HDAC2 expression was significantly elevated in CP rats, which also displayed increased pain sensitivity and higher inflammatory markers (interleukin [IL]-1β [IL-1β], tumor necrosis factor-α [TNF-α], IL-6, and chemokine ligand 2 [CCL-2]). HDAC2 inhibition reduced pain sensitivity and pancreatitis. Co-culture experiments revealed that pancreatic inflammatory mediators upregulate HDAC2 in neurons. ChIP identified Sp1 as a regulatory factor for HDAC2, with the extracellular signal-regulated kinase-Specific protein 1 (ERK-Sp1) pathway critical for its expression.</p><p><strong>Conclusion: </strong>HDAC2 is crucial in CP pain sensitization and inflammation. Its inhibition reduces pain and inflammation, offering potential for targeted pain management in CP.</p>","PeriodicalId":44256,"journal":{"name":"Journal of Neurological Surgery Reports","volume":"86 2","pages":"e99-e106"},"PeriodicalIF":0.6,"publicationDate":"2025-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12058284/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144014646","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Avraham E Adelman, Kartik Motwani, Nikita Chapurin
{"title":"Circulating Tumor DNA Testing for Detection and Surveillance of Sinonasal HPV-Associated Cancers.","authors":"Avraham E Adelman, Kartik Motwani, Nikita Chapurin","doi":"10.1055/a-2576-7496","DOIUrl":"https://doi.org/10.1055/a-2576-7496","url":null,"abstract":"<p><strong>Background: </strong>The incidence of human papilloma virus (HPV)-mediated head and neck (H/N) cancers has risen dramatically. While most HPV-associated H/N cancers are oropharyngeal squamous cell carcinoma (OPSCC), sinonasal squamous cell carcinoma (SNSCC) is the second most common. Recent studies highlight an increasing incidence of HPV-positive SNSCC. Circulating tumor HPV DNA (ctDNA) is a noninvasive tool that has become increasingly utilized to detect high-risk HPV genotypes in the setting of OPSCC, with recent studies reporting high sensitivity and specificity in both pretreatment detection and posttreatment surveillance in OPSCC. Only one study exists reporting its use for SNSCC and nasopharyngeal carcinoma, which was successful in pretreatment detection and identification of recurrence posttreatment.</p><p><strong>Case reports: </strong>We report two cases demonstrating the utility of ctDNA in HPV-mediated sinonasal malignancies. Case 1: 60-year-old male who presented with a large nasal cavity cancer. Pretreatment ctDNA testing yielded a positive tumor tissue modified viral (TTMV)-HPV DNA Score of 67, reflective of the normalized tumor tissue modified viral-HPV DNA fragments/mL of plasma, and pathology confirmed HPV+ SNSCC. Posttreatment surveillance with HPV ctDNA and endoscopy has shown no evidence of disease. Case 2 involves a 64-year-old male with HPV+ neuroendocrine carcinoma who developed recurrence. ctDNA testing, previously negative following initial treatment, scored 35 at recurrence, prompting salvage surgery and adjuvant chemoradiation.</p><p><strong>Conclusion: </strong>These cases, along with prior studies, underscore the potential of ctDNA as a diagnostic and surveillance tool for sinonasal malignancies. Further multi-institutional prospective trials with larger cohorts are needed to validate its role in detection and surveillance.</p>","PeriodicalId":44256,"journal":{"name":"Journal of Neurological Surgery Reports","volume":"86 2","pages":"e89-e91"},"PeriodicalIF":0.6,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12031431/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144052756","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Russel T Wagner, Jacopo Berardinelli, Amin B Kassam, Julian E Bailes, Melanie B Fukui, George C Bobustuc, Sammy Khalili, Neil S Mundi
{"title":"<b>Recurrent Glioblastoma Resection with Microvascular Free Flap Reconstruction and Associated GammaTile Implantation: A Personalized Approach with Oncologic and Reconstructive Integration</b>.","authors":"Russel T Wagner, Jacopo Berardinelli, Amin B Kassam, Julian E Bailes, Melanie B Fukui, George C Bobustuc, Sammy Khalili, Neil S Mundi","doi":"10.1055/a-2576-7559","DOIUrl":"https://doi.org/10.1055/a-2576-7559","url":null,"abstract":"<p><strong>Background: </strong>Glioblastoma multiforme (GBM), despite aggressive multimodal treatment comprising surgery followed by chemoradiation, is almost uniformly associated with inevitable recurrence and poor outcomes. In this clinical context, local radiation therapy-an emerging approach-has gained considerable attention over time for its potential to address the limitations of traditional treatment options for GBM. Multiple surgeries and adjuvant chemoradiation therapy can negatively impact the integrity of the scalp soft tissues and can compromise the ability to achieve primary closure over the surgical site. In these circumstances, complex reconstruction with free tissue transfer may be necessary.</p><p><strong>Methods: </strong>We report the case of a 37-year-old female patient with recurrent GBM and associated wound healing complications who underwent single-stage GammaTile surgically targeted radiation therapy combined with microvascular free flap scalp reconstruction.</p><p><strong>Results: </strong>Immediate free flap reconstruction over the site of GammaTile implantation did not result in any wound healing complications and did not compromise the viability of the transplanted tissue. This approach also provided immediate and localized radiation, possibly enhancing patient progression-free survival while reducing the likelihood of radiation-induced adverse effects.</p><p><strong>Conclusion: </strong>We report the first case of GammaTile implantation with immediate reconstruction of the overlaying soft tissue defect with a free flap. Despite the immediate local radiation produced by the tiles abutting the deep surface of the free flap, there were no complications noted in the vascularity of the transplanted tissue. This finding provides preliminary evidence supporting the safety of using free tissue transfer alongside GammaTile implantation for complex reconstruction.</p>","PeriodicalId":44256,"journal":{"name":"Journal of Neurological Surgery Reports","volume":"86 2","pages":"e92-e97"},"PeriodicalIF":0.6,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12031433/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144049120","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Use of a Septal Stapler to Secure a Septal Free Mucosal Graft to the Nasoseptal Flap Donor Site Following Endoscopic Endonasal Resection of a Pituitary Adenoma.","authors":"Jessa E Miller, Jakob L Fischer, Marilene B Wang","doi":"10.1055/a-2531-6140","DOIUrl":"https://doi.org/10.1055/a-2531-6140","url":null,"abstract":"<p><p>There are multiple techniques that can be utilized to reconstruct skull base defects following endoscopic endonasal pituitary surgery. The nasoseptal flap (NSF) is a vascularized reconstructive option that is commonly used to repair skull base defects. There are several factors that must be considered when deciding to perform a NSF. If a NSF is harvested from one side, the posterior septal mucosa on the contralateral side is often sacrificed and wasted during the posterior septectomy for the endoscopic endonasal pituitary approach. Harvesting a NSF from one side and a posterior septal free mucosal graft (FMG) from the contralateral side affords the surgeon multiple reconstructive options depending on the size of the defect and the presence of a CSF leak at the end of the tumor resection. After a NSF is performed, patients often have significant crusting along the exposed anterior cartilaginous septum that results from delayed remucosalization. If a contralateral posterior septal FMG is harvested, this can be secured to the caudal septal NSF donor site to help minimize postoperative sinonasal symptoms, principally caudal septal crusting which can result in nasal obstruction. In this operative video, we demonstrate the use of a septal stapler to secure a FMG to the anterior NSF donor site following endoscopic endonasal resection of a pituitary adenoma. The staples are dissolvable, and the septal stapler technique is more efficient than performing a quilting suture.</p>","PeriodicalId":44256,"journal":{"name":"Journal of Neurological Surgery Reports","volume":"86 2","pages":"e98"},"PeriodicalIF":0.6,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12132704/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144217194","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Will C Kaiser, Sanaa Hameed, Fauziyya Muhammad, David Barkyoumb, Christian El Amm, Zachary A Smith
{"title":"Application of Augmented Reality Navigation in Craniofacial Surgery for Fibrous Dysplasia.","authors":"Will C Kaiser, Sanaa Hameed, Fauziyya Muhammad, David Barkyoumb, Christian El Amm, Zachary A Smith","doi":"10.1055/a-2547-5400","DOIUrl":"https://doi.org/10.1055/a-2547-5400","url":null,"abstract":"<p><strong>Introduction: </strong>Fibrous dysplasia of the craniofacial bones, or craniofacial dysplasia (CFD), involves the replacement of normal bone with fibrous osseous tissue, resulting in asymmetry and distortion of the overlying soft tissue and irregular bone deposition. Treatment primarily involves surgical resection, and achieving symmetry by matching the contralateral unaffected side is crucial. However, surgical correction is challenging due to the lack of visualization of the normal contralateral structures and the need to precisely control resection depth. Although the application of an augmented reality navigation (ARN) system for CFD surgery has been documented, to our knowledge its specific use in identifying key neurovascular structures has not been reported.</p><p><strong>Methods: </strong>We present the application of an ARN system for the surgical management of an 18-year-old woman with CFD. The virtual plan was designed to visualize the extent of tumor, identify normal and abnormal vasculature, and guide the reconstruction of a normal anatomical contour.</p><p><strong>Results: </strong>ARN was successfully integrated into the surgical workflow and optimized operative planning, identification of tumor margins, avoidance of neurovascular structures, reconstruction, and symmetric recontouring. The ability to visualize structures in real time proved to be especially beneficial for making intraoperative adjustments.</p><p><strong>Conclusion: </strong>ARN has significant applications for CFD surgery by providing real-time, three-dimensional simulation, and precise overlay of patient-specific anatomy and pathology, facilitating safe resection, and providing a useful reconstruction guide. To our knowledge, this report presents the first detailed description of its utility in visualizing critical neurovascular structures, offering significant potential to enhance surgical safety and patient outcomes.</p>","PeriodicalId":44256,"journal":{"name":"Journal of Neurological Surgery Reports","volume":"86 2","pages":"e83-e88"},"PeriodicalIF":0.6,"publicationDate":"2025-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12020552/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144037843","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Russel T Wagner, Jacopo Berardinelli, Melanie B Fukui, Sammy Khalili, Neil S Mundi, Amin B Kassam, Stephen J Winkler
{"title":"Preoperative Administration of Amphotericin B in Orbital Mucormycosis Management: A Case Report.","authors":"Russel T Wagner, Jacopo Berardinelli, Melanie B Fukui, Sammy Khalili, Neil S Mundi, Amin B Kassam, Stephen J Winkler","doi":"10.1055/a-2558-6468","DOIUrl":"https://doi.org/10.1055/a-2558-6468","url":null,"abstract":"<p><p>This case report presents a 29-year-old male with diabetes mellitus who developed rhino-orbito-cerebral mucormycosis (ROCM) that was successfully treated with liposomal amphotericin B orbital injections. Despite emergent endoscopic debridement, the patient's disease progressed intracranially and intraorbitally, but he declined further surgical intervention. Subsequently, due to rapid acute vision loss, we initiated transcutaneous retrobulbar amphotericin B (TRAMB) injections. Following these injections, visual acuity, motility, and intraorbital fungal burden improved despite intracranial progression. This report highlights the benefits of TRAMB administration in aggressive fungal infections and explores the mechanisms behind its effectiveness, particularly in globe preservation. By targeting the infection in an area with a relatively robust blood supply, TRAMB reduces surgical difficulty and improves overall outcomes.</p>","PeriodicalId":44256,"journal":{"name":"Journal of Neurological Surgery Reports","volume":"86 2","pages":"e72-e76"},"PeriodicalIF":0.6,"publicationDate":"2025-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12020544/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144005224","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rita M Jalkh, Yara Yammine, Nader Zalaquett, Houssein Darwish, Zeina Korban
{"title":"Bilateral Endoscopic Endonasal Optic Nerve Decompression in an Infant with Osteopetrosis: A Case Report.","authors":"Rita M Jalkh, Yara Yammine, Nader Zalaquett, Houssein Darwish, Zeina Korban","doi":"10.1055/a-2554-2426","DOIUrl":"https://doi.org/10.1055/a-2554-2426","url":null,"abstract":"<p><strong>Background: </strong>Osteopetrosis is a rare genetic disorder characterized by abnormal bone density and structure, often leading to vision loss due to optic canal stenosis and consequent nerve compression. Early intervention is critical to prevent irreversible damage. This case report discusses the management of bilateral optic nerve compression in an infant with osteopetrosis.</p><p><strong>Case description: </strong>A 7-month-old male with a family history of osteopetrosis presented with hepatosplenomegaly. The infant was diagnosed with osteopetrosis based on radiological findings and genetic testing. Ophthalmologic examination and magnetic resonance imaging showed evidence of bilateral optic nerve compression. Endoscopic transcaruncular optic nerve decompression was not attainable The patient underwent a bilateral expanded endoscopic endonasal medial orbital wall and optic canal decompression.</p><p><strong>Conclusion: </strong>This is one of the few reported cases of endoscopic endonasal optic nerve decompression surgery on an infant. Endoscopic endonasal optic nerve decompression surgery is a viable and effective treatment option for optic nerve compression in infants with osteopetrosis, especially in cases where cost of surgery is a limiting factor for patients. This approach provides direct access to the optic canal with minimal morbidity, offering significant potential for visual recovery, and an improved quality of life. Our patient represents the youngest reported infant in the literature, demonstrating the potential for undergoing this surgical approach at the earliest possible age to aid with his prognosis.</p>","PeriodicalId":44256,"journal":{"name":"Journal of Neurological Surgery Reports","volume":"86 2","pages":"e65-e71"},"PeriodicalIF":0.6,"publicationDate":"2025-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12020546/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144023196","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Grace R Fassina, Emily E Nguyen, David Cho, Drake J Williams, Alexander R Evans, Panayiotis E Pelargos, Christopher S Graffeo
{"title":"The Question Mark Question: Wound Healing after Hemicraniectomy.","authors":"Grace R Fassina, Emily E Nguyen, David Cho, Drake J Williams, Alexander R Evans, Panayiotis E Pelargos, Christopher S Graffeo","doi":"10.1055/a-2561-7951","DOIUrl":"https://doi.org/10.1055/a-2561-7951","url":null,"abstract":"<p><strong>Introduction: </strong>The reverse question mark (RQM) incision is the conventional scalp flap technique incorporated during decompressive hemicraniectomy (DHC) operations. Recently, the retroauricular (RA) incision emerged as a possible alternative. We sought to assess the contemporary literature regarding postoperative outcomes following RQM or RA for DHC and subsequent cranioplasty.</p><p><strong>Methods: </strong>MEDLINE and Embase databases were queried using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies reporting outcomes after primary DHC and secondary cranioplasty for both RQM and RA were included in a meta-analysis of proportions performed using random-effects modeling.</p><p><strong>Results: </strong>Five manuscripts representing a total of 511 patients met the inclusion criteria. No difference was observed in the rate of primary surgical site infection (SSI) after RQM as compared with RA (OR = 1.64, 95% CI = 0.92-2.92). Similarly, based on those four studies reporting SSI data after cranioplasty, no difference was detected in secondary SSI rates between RQM and RA (OR = 1.93, 95% CI = 0.79-4.72). Patients who underwent primary RQM had increased odds of undergoing cranioplasty compared with patients who had received a primary RA (OR = 1.57, 95% CI = 1.03-2.39).</p><p><strong>Conclusion: </strong>This novel systematic review and meta-analysis reported postoperative outcomes after DHC using either the RQM or RA incision technique. No significant difference was noted in SSI after either the primary decompression or the secondary cranioplasty. These findings support the use of either technique in routine practice, as guided by surgeon preference or other clinical considerations, such as superficial temporal artery preservation for an unrelated future indication.</p>","PeriodicalId":44256,"journal":{"name":"Journal of Neurological Surgery Reports","volume":"86 2","pages":"e77-e82"},"PeriodicalIF":0.6,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12020538/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144001673","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Chao Li, James Fowler, Kishore Balasubramanian, Kar-Ming Fung, Piao Zhe, William W Wu
{"title":"Malignant Transformation of a Vestibular Schwannoma Without Previous Radiation Exposure: Illustrative Case and Literature Review.","authors":"Chao Li, James Fowler, Kishore Balasubramanian, Kar-Ming Fung, Piao Zhe, William W Wu","doi":"10.1055/a-2547-5320","DOIUrl":"10.1055/a-2547-5320","url":null,"abstract":"<p><strong>Background: </strong>Although malignant transformation of benign vestibular schwannoma (VS) preceded by irradiation has been well documented, few studies have demonstrated malignant transformation in the absence of radiation. Here, we present a rare case of the malignant transformation of a benign VS to a malignant peripheral nerve sheath tumor (MPNST) in the absence of prior irradiation. Additionally, we conducted a literature search to identify all other reported cases of MPNST arising from VS under similar conditions.</p><p><strong>Case presentation: </strong>A 75-year-old female presented to the hospital with a 1-month history of left-sided facial numbness, loss of taste on the left side of her tongue, severe dysarthria, and recent-onset cranial nerve VI and VII palsies. MRI of the brain with and without contrast demonstrated an enlarging cerebellopontine angle mass and signs of brainstem compression. The patient underwent a left retrosigmoid craniotomy and surgical resection. Pathology and immunohistochemistry sequencing findings were consistent for MPNST with rhabdomyoblastic differentiation (malignant triton tumor). An outside review of the case by a large academic institution concurred with the diagnosis. The patient did not report any previous history of irradiation.</p><p><strong>Conclusion: </strong>A total of 11 cases, including ours, have appropriate S-100 immunochemical reactivity to confirm malignant transformation. Due to the limited number of reported cases of MPNST arising from VS without prior irradiation, information regarding pathogenesis and pathological diagnosis is scarce. We provide valuable additions to the literature, including next-generation sequencing data, to identify potentially targetable genetic changes and help elucidate the pathogenesis of MPNST.</p>","PeriodicalId":44256,"journal":{"name":"Journal of Neurological Surgery Reports","volume":"86 2","pages":"e57-e64"},"PeriodicalIF":0.6,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11968137/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143796478","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}