Paraganglioma pathophysiology

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

Overview

PPGLs arise from chromaffin cells in the adrenal medulla or in extra-adrenal neural ganglia. PPGLs are related to several hereditary syndromes and many of the sporadic cases present germline mutations. Chromaffin cells are involved in the production of catecholamines, hence, these tumors can be biochemically active.

Pathophysiology

Physiology

The normal physiology of [name of process] can be understood as follows:

Pathogenesis

OR

• [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
• The progression to [disease name] usually involves the [molecular pathway].
• The pathophysiology of [disease/malignancy] depends on the histological subtype.

Genetics

Genetics play an important part in PPGL pathogenesis.

[Disease name] is transmitted in [mode of genetic transmission] pattern.

OR

Genes involved in the pathogenesis of [disease name] include:

• [Gene1]
• [Gene2]
• [Gene3]

OR

The development of [disease name] is the result of multiple genetic mutations such as:

• [Mutation 1]
• [Mutation 2]
• [Mutation 3]

Associated Conditions

Conditions associated with [disease name] include:

• [Condition 1]
• [Condition 2]
• [Condition 3]

Gross Pathology

On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Microscopic Pathology

On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].