Paraganglioma classification

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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

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).

[Disease name] may be classified into [large number > 6] subtypes based on:

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

[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].