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Identification of the Determinants of Plexiform Neurofibroma Morbidity in Pediatric and Young Adult Neurofibromatosis Type 1 Patients: A Pilot Multivariate Approach.
Cancers (Basel)
January 2025
Sophia, Department of General Pediatrics, Erasmus MC, 3015 GD Rotterdam, The Netherlands.
Background: Plexiform neurofibromas (PNs) are histologically benign peripheral nerve sheath tumors associated with neurofibromatosis type 1 (NF1) and often lead to significant morbidity due to growth. Management includes watchful waiting, surgery for partial debulking, and, since recently, systemic treatment with MEK inhibitors. However, due to the scarcity of natural history studies, our understanding of the natural progression of PNs to guide clinicians in deciding in whom and when to intervene is scarce.
View Article and Find Full Text PDFSurgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review.
Life (Basel)
December 2024
Department of Thoracic Surgery and Transplantation, Pomeranian Medical University in Szczecin, Alfreda Sokołowskiego 11, 70-891 Szczecin, Poland.
Neurofibromatosis type 1 (NF1) significantly increases the risk of malignant peripheral nerve sheath tumors (MPNST), a rare and aggressive malignancy for which treatment is clinically challenging. This paper presents the case of a 24-year-old male with an NF1 who developed MPNST with lung metastases. Due to the limited effectiveness of systemic therapy in the treatment of MPNST, the patient underwent radical surgical resection and radiotherapy.
View Article and Find Full Text PDFTrigeminal malignant peripheral nerve-sheath tumor: Systematic review, and case report treated by proton-beam therapy.
Neurochirurgie
December 2024
CHU de Caen Normandie, Department of Neurosurgery, Caen F-14000, France; INSERM, UMR-S U1237, Physiopathology and Imaging of Neurological Disorders (PhIND), GIP Cyceron, Caen, F-14000, France.
Background: Malignant peripheral nerve-sheath tumor (MPNST) is rare, occurring in approximately 0.001% of the general population. Cases involving cranial nerves, and particularly the trigeminal nerve (V), are exceptionally rare, with only 36 cases reported in a literature review in 2013.
View Article and Find Full Text PDFStroke-Related Outcomes in Moyamoya Disease Compared with Moyamoya Syndromes: A Propensity-Matched Analysis.
World Neurosurg
December 2024
Neurosurgical Service, Harvard Medical School, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA; Harvard Medical School, Boston, Massachusetts, USA. Electronic address:
Article Synopsis
- - The study aimed to analyze stroke-related outcomes between Moyamoya syndrome (MMS) subgroups and Moyamoya disease (MMD) patients using propensity score matching (PSM) to provide direct comparisons.
- - Researchers analyzed a national database from 2011-2020, focusing on patients with MMD and specific comorbidities (sickle-cell disease, Down syndrome, and neurofibromatosis), and matched them to control for stroke risk factors.
- - Results indicated that patients with MMS related to sickle-cell disease had significantly lower rates of ischemic strokes, hemorrhagic strokes, and transient ischemic attacks compared to those with MMD only, while no significant differences were found in the MMS
Recurrent Solitary Neurofibroma Involving the Proximal Lacrimal Drainage System.
Ophthalmic Plast Reconstr Surg
November 2024
Solitary eyelid neurofibroma is a rare condition, and there have been no reported cases of solitary eyelid neurofibroma involving the proximal lacrimal drainage system. A 6-year-old male presented with recurrent painless swelling in the medial part of the left lower eyelid. The patient was misdiagnosed as eyelid polyp and underwent excision biopsy twice elsewhere.
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