Pheochromocytoma and Neuroblastoma

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Pheochromocytoma It is a relatively rare (usually not malignant) catecholamine producing tumor (both epinephrine and norepinephrine) of the adrenal medulla. 

a. Characteristics. Pheochromocytoma occurs mainly in adults and is generally found in the region of the adrenal gland but also is found in extra-adrenal sites. It occurs within families as part of the multiple endocrine neoplasia (MEN) type 2 syndrome.

b. Symptoms. It is associated with persistent or paroxysmal hypertension, anxiety, tremor, profuse sweating, pallor, chest pain, and abdominal pain.

c. Diagnosis. Increased urine VMA and metanephrine levels, inability to suppress catecholamines with clonidine, and hyperglycemia are common laboratory findings.

d. Treatment. Pheochromocytoma is treated by surgery or phenoxybenzamine (an -adrenergic antagonist).


Neuroblastoma 
It is a common extracranial neoplasm containing primitive neuroblasts of neural crest origin.

a. Characteristics. Neuroblastomas occur mainly in children. They are found in extra-adrenal sites usually along the sympathetic chain ganglia (60%) or within the adrenal medulla (40%). They metastasize widely.

b. Symptoms. Neuroblastoma is associated with opsoclonus (rapid, irregular movements of the eye in horizontal and vertical directions; “dancing eyes”).

c. Diagnosis. A neuroblastoma contains small cells arranged in Homer-Wright pseudorosettes. Increased urine VMA and metanephrine levels are found.

d. Treatment includes surgical excision, radiation, and chemotherapy.

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