Paraganglioma classification

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 classification On the Web
Most recent articles
cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Paraganglioma classification All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Paraganglioma classification
CDC on Paraganglioma classification
Paraganglioma classification in the news
Blogs on Paraganglioma classification
Directions to Hospitals Treating Paraganglioma
Risk calculators and risk factors for Paraganglioma classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Pheochromocytomas and paragnagliomas (collectively referred to as PPGLs) are rare tumors that originate from chromaffin cells in the adrenal medulla (pheochromocytoma) and extra-adrenal neural ganglia (paraganglioma). These tumors can be biochemically active (producing catecholamines) or silent. PPGLs can also be classified as either sporadic or familial, with association extablished to several familial syndromes.

PPGLs can be either benign or malignant. However, the definition of malignancy is established by the presence of metastasis as benign and malignant tumors in this category are indistinguishable in their histological and biochemical characteristics. Extent of spread of tumor is another factor that classifies PPGLs, putting them into the categories of local, regional and metastatic.

Classification

There are several way to classify pheochromocytomas and paragangliomas (PPGLs), including:

PPGLs may be classified according to their site of origin into:

• Pheochromocytoma: tumor originates from chromaffin cells in the adrenal medulla. (80-85%)
• Paraganglioma: tumor originates from chromaffin cells in extra-adrenal neural ganglia. (15-20%) These can have a sympathetic origin (sympathetic chain in thorax, abdomen and pelvis) or a parasympathetic origin (in neck, head and skull base).

Both pheochromocytomas and paragangliomas may be classified into 3 subcategories based on spread:

• Local.
• Regional.
• Metastatic.

These 3 categories share the same histological and biochemical characteristics. Metastasis is considered the definition of malignancy in PPGLs. Non-metastatic PPGLs are considered benign.

[Disease name] may be classified into several subtypes based on:

• [Classification method 1]
• [Classification method 2]
• [Classification method 3]

OR

Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.

OR

If the staging system involves specific and characteristic findings and features:

According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].

OR

The staging of [malignancy name] is based on the [staging system].

OR

There is no established system for the staging of [malignancy name].