A paraganglioma ( syn. Chemodectoma , chromaffin tumor ) is a mostly benign, neuroendocrine tumor that arises from an autonomous ganglion ( paraganglion ). The origin can be both the parasympathetic and the sympathetic nervous system. Depending on the location, 10 to 40% of paragangliomas are malignant and can lead to tumor settlements ( metastases ). There are no reliable criteria that can predict malignant behavior. There is a familial accumulation, as the paraganglioma follows an autosomal dominant inheritance with maternal (maternal) imprinting .
Depending on the ganglion affected, a distinction is made:
- Glomus caroticum - carotid fork tumor : on the carotid fork
- Glomus jugulare : on the parasympathetic paraganglion in the jugular fossa
- Glomus tympanicum : Paraganglion embedded in the middle ear , in the nervus tympanicus
- Vagus nerve - vagal paraganglioma : on the jugular foramen of the base of the skull
- Glomus aorticum ( mediastinal paraganglioma ): in the anterior mediastinum , on the pulmonary artery or aorta
- Abdominal ganglia - retroperitoneal paraganglioma : on the abdominal aorta
- Internal organs - visceral paraganglioma : on the internal organs (especially the urinary bladder )
Symptoms of a paraganglioma emanating from the glomus tympanicum can include unilateral hearing loss or deafness and a pulse-synchronous ear noise. Late symptoms are bleeding from the affected ear and, if the tumor extends into the posterior and middle cranial fossa, paresis of the cranial nerves VII ( facial nerve ), IX ( glossopharyngeal nerve ), X ( vagus nerve ), XI ( accessory nerve ) and XII ( hypoglossal nerve ) .
Macroscopically, they are predominantly round-oval, red-brown encapsulated structures that occasionally infiltrate the bone. Histopathologically, they consist of balls of cells that are surrounded by a fine capillary network. Immunohistochemically, the cells contain chromogranin A and synaptophysin. Some so-called sustentacular cells, which contain S100 protein, lie around the cell balls.
Therapy is carried out by surgical removal. The operation of intracranial glomus tumors is considered difficult due to the abundance of blood and infiltrative growth. If a neck dissection is necessary , the operation should be carried out with the help of a doctor experienced in neck surgery (e.g. endocrine surgeon, ENT doctor or maxillofacial surgeon). Radiation therapy using "external beam radiation therapy" is a good alternative to risky surgery. The success of chemotherapy for glomus tumors is controversial.
www.alf3.urz.unibas.ch Pathohistology on PathoPic
- Charles Dupin et al .: Treatment of Head and Neck Paragangliomas With External Beam Radiation Therapy. Retrieved April 13, 2018 .
- M. Schirmer: Neurosurgery , 10th edition, ISBN 3-437-43600-7 , p. 222