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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.
Definitive diagnosis is made by tumor biopsy. [12] Surgery is the most common method of treating peripheral nerve sheath tumors. [11] In malignant tumors, complete resection is the only known curative treatment (with a sufficiently wide margin or even amputation to improve prognosis). [12]
Symptoms may include: Swelling in the extremities (arms or legs), also called peripheral edema; the swelling often is painless. Difficulty in moving the extremity that has the tumor, including a limp. Soreness localized to the area of the tumor or in the extremity. Neurological symptoms. [3] Pain or discomfort: numbness, burning, or "pins and ...
A melanocytoma is a rare pigmented tumor that has been described as a variant of the melanocytic nevus [1] and is derived from the neural crest. [2] The term "melanocytoma" was introduced by Limas and Tio in 1972.
For asymptomatic or incidental schwannomas or neurofibromas, only continued imaging is typically indicated to assess whether tumor growth is occurring. [1] Surgical resection may be an option for tumors causing extensive radicular pain or other symptoms, and for tumors exhibiting aggressive behavior. [ 1 ]
A related category of melanocytic proliferation is superficial atypical melanocytic proliferations of uncertain significance (SAMPUS). This category, unlike MELTUMP, which implies as yet undetermined potential for metastases even after complete excision, signifies lesions without metastatic potential at time of excision but with potential to ...
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.
A diagnosis of melanoma is supported by the presence of the S-100 protein marker. HMB-45 is a monoclonal antibody that reacts against an antigen present in melanocytic tumors such as melanomas. It is used in anatomic pathology as a marker for such tumors.
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