Non-classical picture of pheochromocytoma

Arterial hypertension is the most common chronic disease in the world. Before a doctor who observes a patient with high blood pressure, there is always a question: what form of arterial hypertension in a patient is essential or secondary, as this affects the tactics of treatment and the prognosis of the disease.

To secondary endocrine hypertensions, in addition to hyperaldosteronism, hypertensive syndrome against the background of the Itenko-Cushing's disease, thyrotoxicosis, pheochromocytoma also applies. Pheochromocytoma (paraganglioma) - a tumor of chromaffin tissue that produces biologically active substances - catecholamines (epinephrine, norepinephrine, dopamine). The most common opinion is that the obligatory sign of pheochromocytoma is arterial hypertension with a crisis current and rise in blood pressure (BP) up to 240-260 mm Hg. St., accompanied by sweating, tachycardia, weight loss. We present our clinical observation, which shows that the disease can be manifested by moderate arterial hypertension with no severe crises, or may occur without increasing blood pressure.

Patient Kh., 51, entered the endocrinology department of the Republican Clinical Hospital (RKB) of the Republic of Tatarstan on 25.01.2012 with complaints of high blood pressure (BP) during physical exertion or at psychoemotional stress up to 160/90 mm Hg. St., accompanied by headaches of a pressing nature in the occipital region, sweating, tidal flare. At rest the blood pressure is 130/80 mm Hg. Art. Worried also aching pain in the lower limbs, coldness of the hands and feet, swelling late in the evening on the lower limbs, insomnia, constipation. The weight is stable.

Anamnesis of the disease. Persistent increase in blood pressure for 5 years. Adopted antihypertensive drugs: amlodipine 10 mg and lopaz 25 mg per day with a mild effect, blood pressure decreased to 140/80 mm Hg. Art. In 2010, primary hypothyroidism was identified, and L-thyroxine replacement therapy was prescribed at a dose of 100 mcg per day.

Considering the resistant course of arterial hypertension against the background of antihypertensive therapy, to eliminate the secondary nature of the increase in blood pressure at the place of residence, an examination was performed: ultrasound of the abdominal cavity organs, kidneys (pathology was not revealed). When computed tomography of the abdominal cavity was performed, the formation in the left adrenal gland was revealed (rounded, uniform with irregular contours, isodense volumetric 24x20 mm, density up to 34 units H). For the purpose of further examination and choice of treatment tactics, the patient is directed to a consultation of the endocrinologist in the RCB.

The condition on admission is satisfactory. Indicators of physical development: height - 154 cm, weight - 75 kg, BMI - 31.6 kg / m2. The constitution is hypersthenic. Skin and visible mucous membranes of physiological color, clean, moist. Subcutaneous fat is developed excessively, distributed evenly. Thyroid gland palpation is not enlarged, soft-elastic, mobile, painless. Peripheral lymph nodes are not enlarged. In the lungs, the breath is vesicular, there is no wheezing. BH - 18 per minute. Blood pressure - 140/90 mm Hg. Art. Heart rate - 76 beats per minute. Heart sounds are rhythmical, clear. The abdomen is soft, painless. The liver is not enlarged. Pasterness of the shins.

[1], [2], [3]

Diagnostics of the nonclassical picture of pheochromocytoma

Data of laboratory and instrumental research.

The general analysis of a blood: a hemoglobin - 148 g / l, iritr. - 5.15x1012, leukocytes - 6.9x109, n - 1%, with - 67%, lymph. 31%, mono. 1%, thrombocytes - 366000 in μl, ESR - 23 mm / h.

General analysis of urine: ud. Weight 1007, protein - otr., watering can. - meals in p / z, epit. Pl. - 1-2 in p / z.

Biochemical blood test: total bilirubin - 12,1 μmol / l (3,4-20,5 μmol / l), ALT - 18 units / l (0-55 units / l), AST - 12 units / l (5- 34 U / L), urea 4.4 mmol / L (2.5-8.3 mmol / L), creatinine 60 μmol / L (53-115 μmol / L), total protein 72 g / L ( 64-83 g / l), cholesterol - 6.6 mmol / l (0-5.17 mmol / l), potassium - 5.2 mmol / l (3.5-5.1 mmol / l), sodium - 141 mmol / l (136-145 mmol / l), chlorine - 108 mmol / l (98-107 mmol / l).

Hormones of the thyroid gland: TTG - 0,97 μIU / ml (0,3500-4,9400 μIU / ml), T4cv. - 1.28 ng / dl (0.70-1.48 ng / dL).

Glycemic profile: 800-4.5 mmol / l, 1100-5.0 mmol / l, 1300-3.9 mmol / l, 1800-5.8 mmol / l, 2200-5.5 mmol / l.

Ultrasound of the hepatobiliary system and kidneys: no pathology was detected.

ECG: Sinus rhythm with heart rate 77 ud. In min. Deviation of EOS to the right. PQ - 0,20 sec, tooth P - pulmonary type. Disturbance of conductivity along the right leg of the bundle.

Ultrasound of the thyroid gland. The volume is 11.062 ml. Isthmus 3,3 mm. Contours of the gland are wavy. Structure with indistinct hypoechoic sites up to 4 mm, diffusely-inhomogeneous. Echogenicity is normal. The intensity of blood flow in the CDC is normal. In the right lobe, an isoechogenic node 3.5 mm in diameter is closer to the lower pole, a weakly hyperechoic region of 4.8x4 mm is next to the back.

PCT of abdominal organs with contrast: in the region of the left adrenal gland, a rounded volumetric formation of 22x27 mm with a density of 27-31 HU is determined, with dense inclusions, with a reliable heterogeneous accumulation of contrast medium up to 86 HU.

Hormonal blood test: aldosterone - 392 ng / ml (norm 15-150 ng / ml), renin - 7.36 ng / ml / hour (norm 0.2-1.9 ng / ml / h), angiotensin - 1- 5.54 ng / ml (norm 0.4-4.1 ng / ml), cortisol - 11.1 μg / ml (norm 3.7-24.0 μg / ml); urine: normetanephrine - 3712.5 μg / day (30-440 μg / day norm), free metanephrenes - 25 μg / day (the norm is 6-115 μg / day).

On the basis of laboratory and instrumental methods of investigation, a diagnosis was established: "Volume formation of the left adrenal gland (hormone-active). Pheochromocytoma. Primary hypothyroidism, moderate severity, compensated. Arterial hypertension 2 degrees, 2 stages. CHF 1, FC 2. Risk 4. Obesity 1 degree, exogenously-constitutional genesis. "

Elevated urinary metanephrine levels, PKT data, characteristic of pheochromocytoma (for CT, a non-contrast density of pheochromocytoma is usually more than 10 units of Hounsfield - often more than 25 HU), pheochromocytoma is characterized by a round or oval shape, a clear contour, heterogeneous structure: fluid areas, necroses, calcinates, hemorrhages speak in favor of the established diagnosis. With a threefold increase in the urine of normetanephrine and double metanephrine, the presence of a chromaffin tumor is undoubted. Increasing the level of aldosterone, renin, angiotensin 1, most likely, has a secondary character, due to the presence of arterial hypertension in the anamnesis for 5 years.

[4], [5], [6], [7]

Treatment of the nonclassical picture of pheochromocytoma

The main task of drug therapy is preparation for surgical treatment. The drug of choice for preoperative preparation of patients with pheochromocytoma - selective prolonged a1-adrenoblocker - doxazosin (cardura). Doxazosin is dosed according to the hypotensive effect and the disappearance of hypovolemic manifestations (orthostatic test). In this patient, on the background of cardura intake, a stabilization of blood pressure was observed at a dose of 4 mg twice a day, a negative orthostatic test (130/80 mm Hg in the horizontal position and 125/80 mm Hg in the vertical position on day 13 of therapy cardura), which indicates the selection of adequate therapy. Achieving a negative orthostatic test during treatment is a criterion for the adequacy of preoperative preparation of a patient with pheochromocytoma, indicating sufficient replenishment of the circulating blood volume and blockade of a-adrenergic receptors.

At the time of hospitalization, the patient received an adequate dose of L-thyroxine 100 μg per day, so substitution therapy with thyroid hormones was decided to remain unchanged. A successful surgical removal of the hormone-active tumor of the left adrenal gland was carried out.

Histological conclusion: in the tumor material there are solid complexes of small and large polygonal and oval cells with a pale and partially empty cytoplasm and a small rounded nucleus surrounded by a thin stromal interlayer. The microscopic picture does not contradict the clinical diagnosis of pheochromocytoma.

With further observation, blood pressure was at stably normal values. Patient with improvement discharged home.

Thus, this patient had a non-classical picture of pheochromocytoma (no malignant hypertensive crises followed by development of hypotension, tachycardia, BP figures corresponded to the average degree of arterial hypertension, the presence of excessive body weight), which made it difficult to diagnose on the basis of clinical manifestations, which did not allow In the debut of the disease, put out a correct diagnosis and prescribe an adequate treatment.