Paraganglioma
Key Facts
Terminology
Synonyms
Extraadrenal paraganglioma, carotid body tumor
Clinical Issues
Incidence
May occur at any age
More common in 3rd decade of life
May occur in anterior and posterior mediastinum
Symptomatology
Dyspnea
Headache
Hypertension
Diaphoresis
Part of multiple endocrine neoplasia (MEN) syndrome
Treatment and prognosis
In majority of cases, complete surgical resection is curative
Top Differential Diagnoses
Neuroendocrine carcinoma
Ectopic parathyroid tumor
Thymic carcinoma
Diagnostic Checklist
Zellballen growth pattern
Prominent cellular atypia
Macronuclei
Rare mitotic activity
 Mediastinal paraganglioma shows the typical nesting pattern of growth. Note the presence of a dilated vascular structure  . These findings are typical for paraganglioma. |
 Mediastinal paraganglioma shows the presence of marked nuclear atypia and macronuclei and a lack of mitotic activity. |
TERMINOLOGY
Synonyms
Extraadrenal paraganglioma, carotid body tumor
Definitions
Neuroendocrine neoplasm
ETIOLOGY/PATHOGENESIS
Etiology
These tumors may originate from normal paraganglia
CLINICAL ISSUES
Epidemiology
Incidence
Unusual tumors in mediastinal compartment
Age
May occur at any age
More common in 3rd decade of life
Gender
No gender predilection
Some studies have reported slight predominance in male patients
Site
Paragangliomas may occur in anterior and posterior mediastinum
Presentation
Chest pain
Cough
Difficulty in swallowing
Dyspnea
Headache
Hypertension
Diaphoresis
Part of the multiple endocrine neoplasia (MEN) syndrome
Some patients may be asymptomatic
Treatment
Complete surgical resection
Adjuvant therapy in selected cases
Prognosis
In majority of cases, complete surgical resection is curative
In some cases, paragangliomas behave aggressively with metastatic disease leading to death
MACROSCOPIC FEATURES
General Features
Soft, irregular
Tan to light brown
Size
Varies from 2 cm to > 10 cm in diameter
MICROSCOPIC PATHOLOGY
Histologic Features
So-called zellballen growth pattern
Nested pattern
Spindle cell pattern
Presence of large cells with macronuclei and bizarre shapes
Granular-like cells may be prominent
Melanin pigment may also be seen
Mitotic activity is rare
Extensive areas of hyalinization may be present
Ectatic vessels
DIFFERENTIAL DIAGNOSIS
Well-differentiated Neuroendocrine Carcinoma (Carcinoid Tumor)
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