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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 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.
Malignant schwannoma, [1] Neurofibrosarcoma, [1] and Neurosarcoma [1] Micrograph of malignant peripheral nerve sheath tumour with the typical herringbone pattern . H&E stain .
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. [ 8 ] [ 9 ] A variety of peripheral nerve cells, including axons, perineurial cells, fibroblasts, and varying inflammatory components such as mast cells ...
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]
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 ...
Carney complex and its subsets LAMB syndrome [1] and NAME syndrome [1] are autosomal dominant conditions comprising myxomas of the heart and skin, hyperpigmentation of the skin (lentiginosis), and endocrine overactivity.
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.