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| __NOTOC__ | | __NOTOC__ |
| {{Paraganglioma}} | | {{Paraganglioma}} |
| {{CMG}} {{AE}} {{AAM}} | | {{CMG}} {{AE}} |
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| ==Overview== | | ==Overview== |
| Paraganglioma is a rare [[neoplasm]] that can be found along the spinal cord in the abdomen, thorax, and head and neck. Paraganglioma is usually considered [[benign]]. However, in about 3% of cases they are [[malignant]] and have the ability to [[metastasis|metastasize]]. Symptoms of paraganglioma may include [[dysphagia]], [[dizziness]], [[tinnitus]] and [[hearing loss]]. Complete surgical removal results in cure. Paraganglioma is still sometimes called glomus tumor (not to be confused with [[glomus tumor]] of the skin) and chemodectomas, but paraganglioma is the currently accepted and preferred term.
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| Paraganglioma is found predominantly in the abdomen (85%), other sites include thorax (12%), and head and neck (3%). 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, II-B, and ''[[SDH]]'' related mutations. Symptoms of paraganglioma include [[dysphagia]], [[dizziness]], and hearing problems. 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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| ==Historical Perspective== | | ==Historical Perspective== |
| Glomus tumor name formerly (and incorrectly) used for paraganglioma.
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| ==Classification== | | ==Classification== |
| Paraganglioma may be classified into several subtypes based on the site of origin and presence of neural cells.
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| ==Pathophysiology== | | ==Pathophysiology== |
| On gross pathology, sharply circumscribed polypoid red vascular masses with firm to rubbery in consistency are characteristic findings 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 balls are separated by fibrovascular [[stroma]] and surrounded by 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 Paraganglioma From Other Diseases== | | ==Differentiating Xyz from Other Diseases== |
| Paraganglioma must be differentiated from [[carcinoid tumor]], neuroendocrine carcinoma, medullary carcinoma of the thyroid, middle ear [[adenoma]], and [[meningioma]].
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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 an earlier age.
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| ==Complication== | | ==Risk Factors== |
| Complication associated with paraganglioma may 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=== | | ===History and Symptoms=== |
| Symptoms of paraganglioma include [[dysphagia]], [[dizziness]], [[tinnitus]], and [[hearing loss]].
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| ===Physical Examination Finding=== | | ===Physical Examination=== |
| Common physical examination findings of paragangliomas include painless [[neck masses|neck mass]], [[hearing loss]], and [[tympanic membrane|red tympanic membrane]].
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| ===X-Ray=== | | ===Laboratory Findings=== |
| On abdominal or [[chest X-ray]], paragaglioma may be associated with hyperdense mass.
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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===
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| [[CT scan]] 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, MRI 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 the diagnosis of paragnglioma.
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| | ===Other Diagnostic Studies=== |
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| ==Treatment== | | ==Treatment== |
| ===Medical Therapy=== | | ===Medical Therapy=== |
| Pharmacological 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}} | | {{reflist|2}} |
| {{Epithelial neoplasms}}
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