Imaging differential diagnosis Epidemiology The relative prevalence of glomus jugulare with respect to other head and neck paraganglioma varies from publication to publication and depending on the definition of the terms jugulare, tympanicum and jugulotympanicum. Most agree however that they are more common than glomus vagale 3. These tumors are seen in adults, typically between 40 and 60 years of age, with a moderate female predilection 3. Clinical presentation Presentation depends on the degree of middle ear involvement.
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Mutations of SDHB play an important role in familial adrenal pheochromocytoma and extra-adrenal paraganglioma of abdomen and thorax , although there is considerable overlap in the types of tumors associated with SDHB and SDHD gene mutations.
The cut surface of a 3. It was surrounded by the heart, left lower lobe of the lung, aorta, esophagus, and diaphragm, and had been 1. Micrograph of a carotid body tumor with the characteristic Zellballen. The paragangliomas appear grossly as sharply circumscribed polypoid masses and they have a firm to rubbery consistency. They are highly vascular tumors and may have a deep red color.
On microscopic inspection, the tumor cells are readily recognized. Individual tumor cells are polygonal to oval and are arranged in distinctive cell balls, called Zellballen. With immunohistochemistry , the chief cells located in the cell balls are positive for chromogranin , synaptophysin , neuron specific enolase , serotonin , neurofilament and Neural cell adhesion molecule ; they are S protein negative.
The sustentacular cells are S positive and focally positive for glial fibrillary acidic protein. By histochemistry , the paraganglioma cells are argyrophilic, periodic acid Schiff negative, mucicarmine negative, and argentaffin negative.
While most are single, rare multiple cases occur usually in a hereditary syndrome. Paragangliomas are described by their site of origin and are often given special names: Carotid paraganglioma carotid body tumor : Is the most common of the head and neck paragangliomas. It usually presents as a painless neck mass, but larger tumors may cause cranial nerve palsies, usually of the vagus nerve and hypoglossal nerve. Organ of Zuckerkandl: A collection of paraganglia near the bifurcation of the aorta, comprising a small mass of neural crest-derived chromaffin cells.
Serves as a common origin of abdominal paragangliomas. The cranial nerves of the jugular foramen may be compressed, resulting swallowing difficulty, or ipsilateral weakness of the upper trapezius and sternocleiodomastoid muscles from compression of the spinal accessory nerve. These patients present with a reddish bulge behind an intact ear drum.
This condition is also known as the "Red drum". On application of pressure to the external ear canal with the help of a pneumatic ear speculum the mass could be seen to blanch. A deficient bony plate along the tympanic portion of the internal carotid artery aberrant ICA is a normal variant and can be mistaken with glomus jugulare.
They usually present as a painless neck mass, but may result in dysphagia and hoarseness. Pulmonary paraganglioma: These occur in the lung and may be either single or multiple. Classification[ edit ] Paragangliomas originate from paraganglia in chromaffin-negative glomus cells derived from the embryonic neural crest , functioning as part of the sympathetic nervous system a branch of the autonomic nervous system.
These cells normally act as special chemoreceptors located along blood vessels, particularly in the carotid bodies at the bifurcation of the common carotid artery in the neck and in aortic bodies near the aortic arch. Accordingly, paragangliomas are categorised as originating from a neural cell line in the World Health Organization classification of neuroendocrine tumors. In the categorization proposed by Wick, paragangliomas belong to group II.
Glomus Yugular o Timpánico
Doctors believe that acquired mutations cause the tumors, rather than hereditary genes. Glomus jugulare tumors form more often in women than in men, and in older adults. They can, however, form in anyone at any age. A physical examination is the first step towards diagnosing a glomus jugulare tumor. The symptoms, as well as an examination of the ear and throat area, can indicate that a tumor may be present.
Glomus Jugulare Tumor
Case 1 Case 1. Multiple lesions are slightly more common in males. A glomus jugulare tumor grows in the temporal bone of the skull, in an area called the jugular foramen. Fukushima journal of medical science. Glomus tumor Edit article Share article View revision history.