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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 .
Micrograph highlighting the sustentacular cells in a paraganglioma. S100 immunostain. A sustentacular cell is a type of cell primarily associated with structural support, they can be found in various tissues. [1]
The S100 proteins are a family of low molecular-weight proteins found in vertebrates characterized by two calcium-binding sites that have helix-loop-helix ("EF-hand-type") conformation. At least 21 different S100 proteins are known. [1] They are encoded by a family of genes whose symbols use the S100 prefix, for example, S100A1, S100A2, S100A3.
Neuroendocrine tumors (NETs) are neoplasms that arise from cells of the endocrine and nervous systems.They most commonly occur in the intestine, where they are often called carcinoid tumors, but they are also found in the pancreas, lung, and the rest of the body.
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]
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).
Paraganglioma (j) typically shows negativity for calcitonin and S100-positive sustentacular cells (inset) [9] Hyperfunctioning follicular adenoma typically shows follicles with papillary infoldings and bubbly, pale colloid with peripheral scalloping (a).
They are universally S-100 positive, which is a marker for cells of neural crest cell origin. Schwannomas of the head and neck are a fairly common occurrence and can be found incidentally in 3–4% of patients at autopsy. [4]