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Malignant melanotic nerve sheath tumor (previously known as melanotic schwannoma) is a rare aggressive peripheral nerve sheath tumor that typically develops in conjunction with spinal or visceral autonomic nerves, consisting uniformly of Schwann cells displaying melanocytic differentiation.
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.
Malignant schwannoma, [1] Neurofibrosarcoma, [1] and Neurosarcoma [1] Micrograph of malignant peripheral nerve sheath tumour with the typical herringbone pattern . H&E stain .
A nerve sheath tumor is a type of tumor of the nervous system (nervous system neoplasm) which is made up primarily of the myelin surrounding nerves.Nerve sheath tumors can be benign or malignant, and may affect both the peripheral and central nervous systems.
Micrograph of a psammomatous melanotic schwannoma with a psammoma body, as may be seen in Carney complex. H&E stain. Psammoma bodies may be seen in: Endosalpingiosis [10] Psammomatous melanotic schwannoma; Melanocytic nevus [11]
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.
Melanotic schwannoma: 8-18% Acromegaly: 10% Osteochondrotic myxoma <10% Multiple blue nevi: The LAMB acronym refers to lentigines, atrial myxomas, and blue nevi. [1]
Psammomatous melanotic schwannoma: Carney complex: Sclerotic fibroma: Cowden syndrome: Trichilemmoma: Cowden syndrome Bannayan–Riley–Ruvalcaba syndrome: Sebaceous adenoma: Muir–Torre syndrome: Sebaceous carcinoma: Muir–Torre syndrome: Syringofibroadenoma: Clouston syndrome Schöpf–Schulz–Passarge syndrome: Syringoma: Down syndrome ...
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