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Pediatric Spinal Tumors
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Rajiv R. Iyer, Nir Shimony, Mohammad Hassan A. Noureldine, Eric Bouffet, George I. Jallo
The most common nerve sheath tumors in children are schwannomas and neurofibromas. A more aggressive and unusual entity, the malignant peripheral nerve sheath tumor, can also be seen and requires radical resection followed by adjuvant therapy.
Peripheral nerve disorders
Published in Michael Y. Wang, Andrea L. Strayer, Odette A. Harris, Cathy M. Rosenberg, Praveen V. Mummaneni, Handbook of Neurosurgery, Neurology, and Spinal Medicine for Nurses and Advanced Practice Health Professionals, 2017
Malignant—Malignant peripheral nerve sheath tumor (MPNST). This may form spontaneously, occur following radiation, or occur in patients with neurofibromas and NF-1. Suspect malignancy in patients with increasing pain, neurologic deficit, and rapid tumor growth. After definitive diagnosis, wide resection is preferred by many surgeons, often combined with radiation to produce a 5-year survival of about 50%. Do not perform aggressive resection based on intraoperative histology.
Neurofibroma
Published in Dongyou Liu, Tumors and Cancers, 2017
Soubhagya Ranjan Tripathy, Samer Hoz Saad Alameri, Mohamed Said Elsanafiry
Neurofibroma is a benign nerve sheath tumor. However, there is a risk of malignant transformation in PNF, leading to malignant peripheral nerve sheath tumor. Five-year survival approaches 90% following chemotherapy alone and can substantially delay or defer radiation in children with OPG.
Endovascular embolization of spontaneous massive hemorrhage of a facial plexiform neurofibroma: case report and literature review
Published in Brain Injury, 2022
Ying Jiang, Zheng Xu, Jin-Xiang Huang, Dan-Qing Yu, Cheng-Guang Huang
The NF1 is known as a genetic tumour-disposition syndrome associated with neoplasm in both nervous and other organ systems (17,18). Most of these tumours are histologically benign with an overall low incidence of malignancy (18). For example, the plexiform neurofibroma is the most common peripheral nerve tumour impacting patients with NF1 (19), whose incidence rate could be up to 50% (20). However, compared to the general population, patients with NF1 still show an average 15-years decrease in life expectancy (21–24). This significant mortality is mainly due to the relentless growth of malignant tumours in the nervous system. In the peripheral nerve system, 8%–13% of patients with NF1 will develop the malignant peripheral nerve sheath tumour (MPNST), which is a rare form of sarcoma and performs in a highly aggressive manner (19). Due to limited treatment options, the 5-year survival rate of MPNST is below 50% (19). In the central nervous system, NF1 individuals are at risk for glial neoplasm. The optic pathway glioma account for up to 80% of all intracranial tumour in children, whose incidence rate is approximately 15%–20% (25). However, the glioma outside of the optic pathways is the main cause of early death in NF1 adults (23,24). Glioblastoma is the most commonly seen non-optic glioma, whose incidence rate is approximately 5% (26,27). In most cases, the diagnosis of glioblastoma carries a poor prognosis and the overall survival period is about 50–60 weeks (28). In the current study, the patient suffered from a glioblastoma reoccurrence shortly after tumourectomy, which is consistent with the conclusion of literature.
Selumetinib: the first ever approved drug for neurofibromatosis-1 related inoperable plexiform neurofibroma
Published in Current Medical Research and Opinion, 2021
Sabyasachi Mukhopadhyay, Arpita Maitra, Shouvik Choudhury
Plexiform neurofibroma (PN) arises from multiple nerve fascicles and tends to grow along the length of a nerve. It occurs grossly in 30% cases of NF1 and present from birth usually1. It is the most common cause of morbidity and cosmetic impairment. PN can be both visible from the surface of the body, or may be internal with no evident superficial extension. Growth of PN can occur at any stage of life but mostly in childhood and during hormonal changes. The most alarming feature is the increased risk of transformation into malignant peripheral nerve sheath tumors (MPNST). Treatment remains a surgical challenge, with no established medical approach and surgery is not always possible also due to location, involvement of vital tissue, optimal timing, and incomplete removal etc3.
Triton tumor of the orbit
Published in Orbit, 2020
Atanu Barh, Bipasha Mukherjee, Kirthi Koka, Subramanian Krishnakumar
Peripheral nerve sheath tumors are derived from the pluripotent Schwann cells of the neurilemmal sheath.Many unusual histologic variants within an MPNST have been described, including osteoid, benign striated muscle, cartilage, nevus cells, malignant melanoma, malignant neuroepithelium, liposarcoma, angiosarcoma, and rhabdomyosarcoma (embryonal and pleomorphic).6 The exact pathogenesis of the rhabdomyosarcomatous differentiation within the substance of a peripheral nerve tumor is unclear. MTT was first described by Mason in 1932 as “nerve rhabdomyoma.” In 1973, Woodruff coined the name “malignant triton tumor” inspired by Locatelli’s experiment on triton salamanders capable of growing muscle and bone from nerve tissue.6 Locatelli proposed that endoneurial cells of “neuromas” may differentiate into muscle tissue under the organizing influence of motor nerve fibers. However, limb and muscle regeneration in tritons is not dependent upon motor nerve innervation as even aneurogenic forelimbs of young salamanders may regenerate.6 Later, Masson proposed the more acceptable theory that neoplastic Schwann cells can transform into striated muscle elements which can give rise to rhabdomyosarcomatous differentiation.6