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| __NOTOC__ | | __NOTOC__ |
| {{Paraganglioma}} | | {{Paraganglioma}} |
| {{CMG}} {{AE}} {{AAM}} | | {{CMG}} {{AE}} |
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| ==Overview== | | ==Overview== |
| A '''paraganglioma''' is a rare [[neoplasm]] that can be found in the abdomen, thorax, and in the head and neck region. They are usually considered [[benign]] and complete surgical removal results in cure. However, in about 3% of cases they are [[malignant]] and have the ability to [[metastasis|metastasize]]. Paragangliomas are still sometimes called '''glomus tumors''' (not to be confused with [[glomus tumor]]s of the skin) and '''chemodectomas''', but paraganglioma is the currently accepted and preferred term.
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| Paragangliomas are found predominantly in the abdomen (85%) and the thorax (12%), and only 3% are found in the head and neck region. Most occur as single tumors. When they occur in multiple sites they are usually found as a part of a heritable syndrome such as [[multiple endocrine neoplasia]] types II-A and II-B and [[SDH]]-related mutations.
| | ==Historical Perspective== |
| ==Historical perspective== | | |
| Glomus tumor name formerly (and incorrectly) used paraganglioma.
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| ==Classification== | | ==Classification== |
| Paraganglioma may be classified into several subtypes based on having neural cell line and site of origin.
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| ==Pathophysiology== | | ==Pathophysiology== |
| On gross pathology, sharply circumscribed polypoid red vascular masses with firm to rubbery consistency are characteristic of paragangliomas. 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. These cell balls are separated by fibrovascular [[stroma]] and surrounded by
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| sustentacular cells.
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| ==Causes== | | ==Causes== |
| There are no established causes for paraganglioma, approximately 25% of cases are inherited.
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| | ==Differentiating Xyz from Other Diseases== |
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| ==Epidemiology and Demographics== | | ==Epidemiology and Demographics== |
| Paraganglioma is a rare disease, familial paragangliomas account for approximately 25% of cases and occur at earlier age.
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| ==Complication== | | ==Risk Factors== |
| Complication associated with paraganglioma my include [[Hoarseness]], [[Dysphagia]], [[Hearing loss]], and [[Facial paralysis]].
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| | ==Screening== |
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| | ==Natural History, Complications, and Prognosis== |
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| ==Diagnosis== | | ==Diagnosis== |
| ===Staging=== | | ===Diagnostic Study of Choice=== |
| There is no established system for the staging of paraganglioma.
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| ===Symptoms===
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| Symptoms of paraganglioma include [[dysphagia]], [[dizziness]], and hearing problems.
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| ===Physical Examination Finding===
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| Common physical examination findings of paragangliomas include painless neck mass, hearing loss, and red eardrum.
| | ===History and Symptoms=== |
| ===CT=== | | |
| | ===Physical Examination=== |
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| | ===Laboratory Findings=== |
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| | ===Electrocardiogram=== |
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| | ===X-ray=== |
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| | ===Echocardiography and Ultrasound=== |
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| | ===CT scan=== |
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| CT sacn is helpful in localization of paraganglioma.<ref name="cancergov"> National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_94_toc</ref>
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| ===MRI=== | | ===MRI=== |
| MRI is helpful in localization of paraganglioma which demonstrates a high signal mass.<ref name="cancergov"> National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_94_toc</ref>
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| ===Other Imaging Studies=== | | ===Other Imaging Findings=== |
| <sup>123</sup>I-metaiodobenzylguanidine (MIBG) scintigraphy coupled with CT imaging can be used for diagnosis of paragnglioma.
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| ===Other Diagnostic Studies=== | | ===Other Diagnostic Studies=== |
| [[Immunohistochemistry]] and [[histochemistry]] are used in the diagnosis of paraganglioma.
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| ==Treatment== | | ==Treatment== |
| ===Medical Therapy=== | | ===Medical Therapy=== |
| Pharmacologic medical therapies for paraganglioma include alpha blockers, beta blockers, and chemotherapy.
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| | === Interventions === |
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| ===Surgery=== | | ===Surgery=== |
| Surgery is the mainstay of treatment for paraganglioma.
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| | ===Primary Prevention=== |
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| | ===Secondary Prevention=== |
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| ==References== | | ==References== |
| | {{reflist|2}} |
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| {{reflist|2}}
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| {{Epithelial neoplasms}}
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