Paraganglioma overview
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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, 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 the site of origin and having a neural cell line.
Pathophysiology
On gross pathology, sharply circumscribed polypoid red vascular masses with the firm to rubbery in 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.
Differentiating Paraganglioma From Other Diseases
Paraganglioma must be differentiated from carcinoid tumor, neuroendocrine carcinoma, medullary carcinoma of the thyroid, middle ear adenoma, and meningioma.
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, tinnitus and hearing loss.
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.
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