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GP consist of three components (1) ganglion cells, (2) epithelioid cells (neuroendocrine-like), and (3) spindle cells (schwannoma-like). The microscopic differential diagnosis includes poorly differentiated carcinoma, neuroendocrine tumour and paraganglioma. [1] GPs may be sporadic or arise in the context neurofibromatosis type 1.
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 glomus tumor (also known as a "solitary glomus tumor" [1]) is a rare neoplasm arising from the glomus body and mainly found under the nail, on the fingertip or in the foot. [2]: 670 They account for less than 2% of all soft tissue tumors. [3] The majority of glomus tumors are benign, but they can also show malignant features. [4]
Ganglioneuroma (occasionally called a "ganglioma") is a rare and benign tumor of the autonomic nerve fibers arising from neural crest sympathogonia (undifferentiated cells of the sympathetic nervous system). [1] However, ganglioneuromas themselves are fully differentiated neuronal tumors that do not contain immature elements. [2]
Zellballen are separated into groups by segmenting bands of fibrovascular stroma, and are surrounded by supporting sustentacular cells. [1] A zellballen pattern is diagnostic for paraganglioma or pheochromocytoma. [2] Zellballen is German for "ball of cells". [3]
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