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A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen). When the same type of tumor is found in the adrenal gland, they are referred to as a pheochromocytoma. They are rare tumors, with an overall estimated incidence of 1 in 300,000. [1]
A gangliocytic paraganglioma is a rare tumour that is typically found in the duodenum and consists of three components: (1) ganglion cells, (2) epithelioid cells (paraganglioma-like) and, (3) spindle cells (schwannoma-like). [1]
The 5th WHO classification delineates distinct types of tumors, some of them being further divided into subtypes, rendering the former terms entity and variant obsolete. When molecular diagnostics are not complete enough to allow precise classification, diagnosis should be designated by appending not otherwise specified (NOS).
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.
These tumors might lack a capsule or be less encapsulated than a typical schwannoma.More challenging to identify from MPNST are the infrequent plexiform schwannomas that develop in deep anatomical sites, such as soft tissue [9] or major peripheral nerves. [10] These tumors may exhibit enhanced cellularity and mitotic activity.
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.
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