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Micrograph of a schwannoma, a tumor seen in neurofibromatosis type II. HPS stain. Schwannoma of the N. Vestibularis Meningiomas in a person with NFII. The so-called acoustic neuroma of NF2 is in fact a schwannoma of the nervus vestibularis, or vestibular schwannoma. The misnomer of acoustic neuroma is still often used.
A unique subtype of schwannoma that typically develops in superficial (cutaneous or subcutaneous) sites and may be identified by its development pattern, which is plexiform (intraneuralnodular). [6] [7] The correlation with schwannoma predisposition disorders like NF2 and schwannomatosis, however, is minimal (approximately 5 percent of cases). [8]
Schwannomatosis is an extremely rare genetic disorder closely related to the more-common disorder neurofibromatosis (NF). Originally described in Japanese patients, [1] it consists of multiple cutaneous schwannomas, central nervous system tumors, and other neurological complications, excluding hallmark signs of NF.
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 inherited disease.
New and more precisely defined entities include malignant melanotic nerve sheath tumor (formerly known as melanotic schwannoma) and hybrid nerve sheath tumors. [4] [5] The majority of peripheral nerve tumors are benign tumors of the nerve sheath (usually schwannomas); on rare occasions, they are metastatic tumors or originate from the nerve cells.
Plexiform fibrohistiocytic tumor appears as a painless, slowly expanding soft tissue mass located in the dermis and subcutis. [2] The skin layer above is somewhat elevated and occasionally has a central depression. [3] The upper extremities were more commonly affected by plexiform fibrohistiocytic tumors.
Malignant schwannoma, [1] Neurofibrosarcoma, [1] and Neurosarcoma [1] Micrograph of malignant peripheral nerve sheath tumour with the typical herringbone pattern . H&E stain .
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