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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.
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).
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]
Furthermore, when pictured in the ideal clinical scenario (an older woman in her mid-50s), the spontaneous attacks of flushing, sweating, and a racing heart may be mistaken for pre-menopausal related hot flashes. Unmanaged pheochromocytoma is dangerous and can lead to serious, potentially fatal, complications, including stroke and hypertension ...
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 ]
The protein encoded by this gene is a member of the S100 family of proteins containing 2 EF-hand calcium-binding motifs. S100 proteins are localized in the cytoplasm and/or nucleus of a wide range of cells, and involved in the regulation of a number of cellular processes such as cell cycle progression and differentiation.