Paraganglioma overview: Difference between revisions
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==Overview== | ==Overview== | ||
'''paraganglioma''' is a rare [[neoplasm]] that can be found in the abdomen, thorax, head and neck. They are usually considered [[benign]], 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. | |||
Paragangliomas are found predominantly in the abdomen (85% | Paragangliomas are found predominantly in the abdomen (85%, 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. 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> | ||
Surgery is the mainstay of treatment for paraganglioma. | Surgery is the mainstay of treatment for paraganglioma. | ||
==Historical perspective== | ==Historical perspective== | ||
Glomus tumor name formerly (and incorrectly) used paraganglioma. | Glomus tumor name formerly (and incorrectly) used paraganglioma. | ||
==Classification== | ==Classification== | ||
Paraganglioma may be classified into several subtypes based on having neural cell line and site of origin. | Paraganglioma may be classified into several subtypes based on having a neural cell line and site of origin. | ||
==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 | 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 | ||
sustentacular cells. | sustentacular cells. | ||
==Causes== | ==Causes== | ||
There are no established causes for paraganglioma, approximately 25% of cases are inherited. | There are no established causes for paraganglioma, approximately 25% of cases are inherited. | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
Paraganglioma is a rare disease, familial paragangliomas account for approximately 25% of cases and occur at earlier age. | Paraganglioma is a rare disease, familial paragangliomas account for approximately 25% of cases and occur at an earlier age. | ||
==Complication== | ==Complication== | ||
Complications associated with paraganglioma may include [[Hoarseness]], [[Dysphagia]], [[Hearing loss]], and [[Facial paralysis]]. | |||
==Diagnosis== | ==Diagnosis== | ||
===Staging=== | ===Staging=== | ||
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===CT=== | ===CT=== | ||
CT | 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> | ||
===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> | 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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==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
Pharmacological medical therapies for paraganglioma include alpha blockers, beta blockers, and chemotherapy. | |||
===Surgery=== | ===Surgery=== | ||
Surgery is the mainstay of treatment for paraganglioma. | Surgery is the mainstay of treatment for paraganglioma. | ||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
{{Epithelial neoplasms}} | {{Epithelial neoplasms}} | ||
Revision as of 14:32, 10 September 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]
Overview
paraganglioma is a rare neoplasm that can be found in the abdomen, thorax, head and neck. They are usually considered benign, complete surgical removal results in cure. However, in about 3% of cases they are malignant and have the ability to metastasize. Paragangliomas are still sometimes called glomus tumors (not to be confused with glomus tumors of the skin) and chemodectomas, but paraganglioma is the currently accepted and preferred term.
Paragangliomas are found predominantly in the abdomen (85%, 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. Symptoms of paraganglioma include dysphagia, dizziness, and hearing problems. MRI is helpful in localization of paraganglioma which demonstrates a high signal mass.[1] Surgery is the mainstay of treatment for paraganglioma.
Historical perspective
Glomus tumor name formerly (and incorrectly) used paraganglioma.
Classification
Paraganglioma may be classified into several subtypes based on having a neural cell line and site of origin.
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 sustentacular cells.
Causes
There are no established causes for paraganglioma, approximately 25% of cases are inherited.
Epidemiology and Demographics
Paraganglioma is a rare disease, familial paragangliomas account for approximately 25% of cases and occur at an earlier age.
Complication
Complications associated with paraganglioma may include Hoarseness, Dysphagia, Hearing loss, and Facial paralysis.
Diagnosis
Staging
There is no established system for the staging of paraganglioma.
Symptoms
Symptoms of paraganglioma include dysphagia, dizziness, and hearing problems.
Physical Examination Finding
Common physical examination findings of paragangliomas include painless neck mass, hearing loss, and red eardrum.
CT
CT scan is helpful in localization of paraganglioma.[1]
MRI
MRI is helpful in localization of paraganglioma which demonstrates a high signal mass.[1]
Other Imaging Studies
123I-metaiodobenzylguanidine (MIBG) scintigraphy coupled with CT imaging can be used for diagnosis of paragnglioma.
Other Diagnostic Studies
Immunohistochemistry and histochemistry are used in the diagnosis of paraganglioma.
Treatment
Medical Therapy
Pharmacological medical therapies for paraganglioma include alpha blockers, beta blockers, and chemotherapy.
Surgery
Surgery is the mainstay of treatment for paraganglioma.
References
- ↑ 1.0 1.1 1.2 National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_94_toc