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Neurofibromatosis type II (also known as MISME syndrome – multiple inherited schwannomas, meningiomas, and ependymomas) is a genetic condition that may be inherited or may arise spontaneously, and causes benign tumors of the brain, spinal cord, and peripheral nerves.
The primary Schwann cell differentiation and neoplastic proliferations are characteristics of peripheral nerve sheath tumors. For instance, the Schwann cell, which is the major neoplastic cell component of neurofibroma, [7] is cytologically distinguished by the expression of S-100 protein and wavy nuclear outlines.
A neurofibroma is a benign nerve-sheath tumor in the peripheral nervous system. In 90% of cases, they are found as stand-alone tumors ( solitary neurofibroma , solitary nerve sheath tumor [ 1 ] or sporadic neurofibroma [ 1 ] ), while the remainder are found in persons with neurofibromatosis type I (NF1), an autosomal-dominant genetically ...
Lipofibromatosis-like neural tumor (LPF-NT) is an extremely rare soft tissue tumor first described by Agaram et al in 2016. [1] As of mid-2021, at least 39 cases of LPF-NT have been reported in the literature.
Neurofibromatosis; Back of an elderly woman with neurofibromatosis type 1: Specialty: Neurosurgery, neurology, Neuro-oncology: Symptoms: Small lumps within the skin, scoliosis, hearing loss, vision loss [1]
In a 2008 review of the latter two categories, atypical and anaplastic-meningioma cases, the mean overall survival for atypical meningiomas was found to be 11.9 years vs. 3.3 years for anaplastic meningiomas. Mean relapse-free survival for atypical meningiomas was 11.5 years vs. 2.7 years for anaplastic meningiomas. [25]
A central nervous system primitive neuroectodermal tumor, often abbreviated as PNET, supratentorial PNET, or CNS-PNET, [1] is one of the 3 types of embryonal central nervous system tumors (medulloblastoma, atypical teratoid rhabdoid tumor, and PNET). [2]
The latter had, through the years, been variously described as bizarre cutaneous neurofibroma, myxoma of nerve sheath, and pacinian neurofibroma. [4] Clinically, neurothekeomas present as a solitary nodule of the skin. The most common sites of occurrence are the head and neck and the extremities.