History

Symptoms and signs of pheochromocytoma include the following:

Headache
Diaphoresis
Palpitations
Tremor
Nausea
Weakness
Pallor
Anxiety, sense of doom
Epigastric pain
Flank pain
Constipation
Weight loss

The classic history of a patient with a pheochromocytoma includes spells characterized by headaches, palpitations, and diaphoresis in association with severe hypertension. These 4 characteristics together are strongly suggestive of a pheochromocytoma. In the absence of these 3 symptoms and hypertension, the diagnosis may be excluded.

The spells may vary in occurrence from monthly to several times per day, and the duration may vary from seconds to hours. Typically, they worsen with time, occurring more frequently and becoming more severe as the tumor grows.

Pheochromocytomas occur in certain familial syndromes. These include multiple endocrine neoplasia (MEN) types 2A and 2B, neurofibromatosis (von Recklinghausen disease), and von Hippel-Lindau (VHL) disease, as well as others (see Etiology). In general, these hereditary cancer syndromes are inherited in an autosomal dominant manner.

Physical Examination

Clinical signs associated with pheochromocytomas include the following:

Hypertension: Paroxysmal in 50% of cases
Postural hypotension (from volume contraction)
Hypertensive retinopathy
Weight loss
Pallor
Fever
Tremor
Tachyarrhythmias ^[31]
Pulmonary edema
Cardiomyopathy
Diabetes mellitus ^[32]
Ileus

Sinus tachycardia (presenting as palpitations) is the most common cardiac rhythm abnormality in patients with pheochromocytoma, but more serious ventricular arrhythmias or conduction disturbances may also occur. Other cardiac manifestations include reversible dilated or hypertrophic cardiomyopathy; Takotsubo cardiomyopathy has gained increasing recognition.^{[31, 33]}

When pheochromocytoma occurs as part of a hereditary syndrome, other manifestations of the syndrome may be noted. In patients with neurofibromatosis, these include neurofibromas and café au lait spots. The latter are patches of cutaneous pigmentation that vary from 1-10 mm and can occur any place on the body; characteristic locations include the axillae and intertriginous areas (groin). The name café au lait refers to the color of the lesions, which varies from light to dark brown.

Differential Diagnoses

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Media Gallery

Axial, T2-weighted magnetic resonance imaging (MRI) scan showing large left suprarenal mass of high signal intensity on a T2-weighted image. The mass is a pheochromocytoma.
Abdominal computed tomography (CT) scan demonstrating left suprarenal mass of soft-tissue attenuation representing a paraganglioma.
Adrenalectomy specimen containing pheochromocytoma. Non-neoplastic adrenal cortex (yellow) surrounds a small tan-red tumor in the medullary region, representing a pheochromocytoma.
H and E, high power, showing classic "balls of cells" feature of a pheochromocytoma. Endocrine tumors such as a pheochromocytoma typically show some degree of nuclear pleomorphism ("endocrine atypia") which does not indicate malignancy.

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Pheochromocytoma Clinical Presentation

History

Physical Examination

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