Paraganglioma overview

Jump to navigation Jump to search

Paraganglioma Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Paraganglioma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT Scan

MRI

Echocardiography and Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Paraganglioma overview On the Web

Most recent articles

cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Paraganglioma overview

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Paraganglioma overview

CDC on Paraganglioma overview

Paraganglioma overview in the news

Blogs on Paraganglioma overview

Directions to Hospitals Treating Paraganglioma

Risk calculators and risk factors for Paraganglioma overview

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 along the spinal cord, in the abdomen, thorax, head and neck. They are usually considered benign. However, in about 3% of cases they are malignant and have the ability to metastasize. Symptoms of paraganglioma may include dysphagia, dizziness, tinnitus and hearing loss. Complete surgical removal results in cure. 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%), other sites include thorax (12%), 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.[1] Surgery is the mainstay of treatment for paraganglioma.

Historical perspective

Glomus tumor name formerly (and incorrectly) used for paraganglioma.

Classification

Paraganglioma may be classified into several subtypes based on the site of origin and presence of neural cells.

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

Complication 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 tympanic membrane.

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 the 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. 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

Template:Epithelial neoplasms

Template:WikiDoc Sources