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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 schwannoma, [1] Neurofibrosarcoma, [1] and Neurosarcoma [1] Micrograph of malignant peripheral nerve sheath tumour with the typical herringbone pattern . H&E stain .
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.
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.
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 ...
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.
Antoni A area of schwannoma with Verocay bodies (one annotated by circle) Verocay bodies were first described by Uruguayan neuro-pathologist José Juan Verocay in 1910. It is a required histopathological finding for diagnosing schwannomas (tumors of Schwann cells).