Background: Malignant peripheral nerve sheath tumours (MPNSTs) are highly aggressive neoplasms with a marked propensity for local recurrence and metastatic spread. The management of MPNSTs continues to challenge pathologists and surgeons. As MPNSTs of the paranasal sinuses and the skull base are rare, prognostic factors and treatment modalities have not been consistently identified.
Patients And Methods: We present a case of MPNST of the anterior skull base and provide an overview of all MPNSTs reported since 1970, in which the tumour location was the anterior skull base or the paranasal sinuses.
Results: Literature review revealed 33 well-documented cases of MPNSTs in this anatomic location. These cases were analysed with emphasis on age, gender, affected site, therapy, outcome, presence of neurofibromatosis, local recurrence and metastases.
Conclusions: Despite multimodal therapy and advances in surgical techniques, the prognosis of MPNST located in the paranasal sinuses and the anterior skull base remains dismal. Outcome is mainly a function of local control by surgical resection. Adjuvant radiochemotherapy has shown no benefit. It may therefore be advisable to abstain from radiochemotherapy in order to improve chances for surgical intervention in case of recurrent disease. Close follow-up investigations are indispensable.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.jcms.2007.11.003 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Causal relationship between psoriasis and sudden deafness: a Mendelian randomization study.
Arch Dermatol Res
January 2025
Tianjin Medical University, Tianjin, 300102, China.
Objective: This study aims to investigate the genetic link between psoriasis and sudden sensorineural hearing loss (SSNHL).
Methods: From a genetic standpoint, this study further highlighted the connection between psoriasis and SSNHL. Single nucleotide polymorphisms (SNPs) connected to SSNHL could be found using a genome-wide association study from the IEU OpenGWAS project website.
Twenty years of experience with lesions involving the temporomandibular joint and adjacent structures complex.
Int J Oral Maxillofac Surg
January 2025
Department of Oral Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China; Shanghai Key Laboratory of Stomatology and Shanghai Research Institute of Stomatology, Shanghai, China; National Clinical Research Center of Stomatology, Shanghai, China. Electronic address:
With their close anatomical relationship, the temporomandibular joint (TMJ), mandibular ramus, skull base, ear, and infratemporal fossa make up a complex structure that may collectively be referred to as the 'TMJ and adjacent structures complex' (TASC). This study presents the neoplasms that may be encountered in the TASC region and their classification, which may be useful for the surgical treatment. Data of 1022 consecutive patients treated with resection of TASC neoplasms from May 2000 to October 2022 were reviewed.
View Article and Find Full Text PDFCoagulopathy at admission in traumatic brain injury and its association with hematoma progression: A systematic review and meta-analysis of 2411 patients.
Clin Neurol Neurosurg
December 2024
Student Research Committee, Gonabad University of Medical Sciences, Gonabad, Iran. Electronic address:
Progressive hemorrhagic injury (PHI) is a frequent complication of traumatic brain injury (TBI). This study aims to investigate the impact of coagulation factors (platelet [PLT], prothrombin time [PT], activated partial thromboplastin time [aPTT], international normalized ratio , fibrinogen [Fg], D-dimer [Dd], and fibrin [Fib]) at admission and PHI development through a comprehensive systematic review and meta-analysis based on PRISMA 2020 guideline. Databases including PubMed, Scopus, Web of Science, and Embase were searched up to March 2024.
View Article and Find Full Text PDFEvaluation of postoperative intracranial pressure in patients with radiological diagnosis of idiopathic intracranial hypertension.
Am J Otolaryngol
December 2024
Skull Base and Rhinology Department, Hospital Universitario Pedro Ernesto, Rio de Janeiro, Brazil; Skull Base and Rhinology Department, Policlinica de Botafogo, Rio de Janeiro, Brazil.
Objective: This study aimed to assess postoperative intracranial pressure in patients with clinical and/or radiological features of idiopathic intracranial hypertension (IIH) undergoing endoscopic endonasal surgery for primary cerebrospinal fluid (CSF) leak repair.
Methods: Data was prospectively collected from 9 patients diagnosed with CSF nasal leaks who underwent corrective endonasal surgery between January 1, 2021, and October 31, 2022. Postoperative intracranial pressure was measured via lumbar puncture at least one month after surgery.
Base of Skull & Spinal Canal Narrowing in an Adolescent with Autosomal Recessive Hypophosphatemic Rickets Type 2.
Calcif Tissue Int
January 2025
Department of Paediatric Endocrinology, Alder Hey Children's Hospital, Liverpool, UK.
Autosomal recessive hypophosphatemic rickets type 2 (ARHR2) is an uncommon hereditary form of rickets characterised by chronic renal phosphate loss and impaired bone mineralisation. This results from compound heterozygous or homozygous pathogenic variants in ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1), a key producer of extracellular inorganic pyrophosphate (PPi) and an inhibitor of fibroblast growth factor23 (FGF23). ENPP1 deficiency impacts FGF23 and increases its activity.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!