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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]
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.
Tumors of the paraganglionic tissues are known as paragangliomas, though this term tends to imply the nonchromaffin type, and can occur at a number of sites throughout the body. Chromaffin paragangliomas are issued from chromaffin cells, and are known as pheochromocytomas .
There is one report of widespread metastases of a malignant glomus tumor involving the skin, lungs, jejunum, liver, spleen, and lymph nodes. [11] Another report of a malignant glomus tumor (glomangiosarcoma) with metastases from the skin. [12] A malignant glomus tumor one arose from the kidneys. [10]
The organ of Zuckerkandl is of pathological significance in the adult as a common extra-adrenal site of pheochromocytoma though the most common extra-adrenal site is in the superior para-aortic region between the diaphragm and lower renal poles. [9] Extra-adrenal tumors account for around 25% of all cases of pheochromocytoma. [10]
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]
1.5.9 Gangliocytoma 1.5.10 Multinodular and vacuolating neuronal tumor 1.5.11 Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) 1.5.12 Central neurocytoma 1.5.13 Extraventricular neurocytoma 1.5.14 Cerebellar liponeurocytoma 1.6 Ependymal tumours 1.6.1 Supratentorial ependymoma 1.6.1.1 Supratentorial ependymoma, ZFTA fusion-positive