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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.
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.
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]
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 ]
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).
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.
6286 n/a Ensembl ENSG00000163993 n/a UniProt P25815 n/a RefSeq (mRNA) NM_005980 n/a RefSeq (protein) NP_005971 n/a Location (UCSC) Chr 4: 6.69 – 6.7 Mb n/a PubMed search n/a Wikidata View/Edit Human S100 calcium-binding protein P (S100P) is a protein that in humans is encoded by the S100P gene. Function The protein encoded by this gene is a member of the S100 family of proteins containing 2 ...