Paraganglioma

A paraganglioma ( syn. chemodectoma, chromaffin tumor ) is a mostly benign neuroendocrine tumor that arises from an autonomic ganglion ( Paraganglion ). Origin can be both the parasympathetic and the sympathetic nervous system. Depending on location, 10 to 40% of malignant paragangliomas and may lead to Tumor ( metastases). There are no reliable criteria that can predict malignant behavior. There is a family history, as the paraganglioma follows an autosomal dominant inheritance with maternal imprinting.

Localization

Depending on the affected ganglion differentiates:

• Carotid body - Karotisgabeltumor: at the carotid bifurcation
• Glomus jugular: the parasympathetic Paraganglion in the jugular fossa
• Glomus tympanic: in the middle ear, in the nerve tympanic intercalated Paraganglion
• Vagus nerve - vagal paraganglioma: the jugular foramen of the skull base
• Glomus aorticum ( mediastinal paraganglioma ) in the anterior mediastinum, pulmonary artery or aorta to
• Abdominal ganglia - retroperitoneal paraganglioma: on the abdominal aorta
• Internal organs - visceral paraganglioma: the internal organs (especially bladder )

Symptoms

Symptoms of a tympanic emanating from glomus paraganglioma may be unilateral hearing loss to deafness and a pulse-synchronous tinnitus. Late symptoms include bleeding from the affected ear and extent of the tumor in the posterior and middle cranial fossa paresis of cranial nerve VII (facial nerve ), IX (glossopharyngeal ), X ( vagus ), XI ( accessory nerve ) and XII ( hypoglossal nerve ).

Diagnosis

In magnetic resonance imaging with T2 weighting the tumors represent predominantly hyperintense to the spinal cord and other surrounding organ structures dar.

Nuclear medicine whole body imaging with a 18 -F dopa PET (positron emission tomography) with very high sensitivity is possible.

Macroscopically, they are predominantly round-oval, reddish brown to encapsulated entities that occasionally infiltrate the bone. Histologically they consist of cell bales, which are surrounded by a fine capillary network. Immunohistochemically, the cells contain chromogranin A and synaptophysin. To the cell bales are partly so-called Sustentakularzellen containing S100 protein.

Therapy

Treatment is by surgical removal. The surgery of intracranial glomus applies, because of the blood and wealth of infiltrative growth, difficult. In need of a neck dissection, the operation should be done with the help of a physician experienced in the neck surgery (eg endocrine surgeon or otolaryngologist ). The success of chemotherapy or radiation treatment for glomus tumors is controversial.