Nonclassical picture of pheochromocytoma

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

In addition to hyperaldosteronism, hypertensive syndrome against the background of Itsenko-Cushing's disease, thyrotoxicosis, pheochromocytoma also belongs to secondary endocrine hypertension. Pheochromocytoma (paraganglioma) is a tumor of chromaffin tissue that produces biologically active substances - catecholamines (adrenaline, noradrenaline, dopamine). The most common opinion is that the obligatory sign of pheochromocytoma is arterial hypertension with a crisis course and an increase in arterial pressure (AP) to 240-260 mm Hg, accompanied by sweating, tachycardia, weight loss. We present our clinical observation showing that the disease can manifest itself as arterial hypertension of moderate severity with the absence of pronounced crises or proceed without an increase in arterial pressure.

Patient H., 51 years old, was admitted to the endocrinology department of the Republican Clinical Hospital (RCH) of the Republic of Tatarstan on 25.01.2012 with complaints of increased blood pressure (BP) during physical exertion or psychoemotional stress up to 160/90 mm Hg, accompanied by pressing headaches in the occipital region, sweating, and hot flashes. At rest, BP was 130/80 mm Hg. She also complained of aching pain in the lower extremities, cold hands and feet, swelling in the lower extremities towards evening, insomnia, and constipation. Her weight was stable.

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

Given the resistant course of arterial hypertension against the background of hypotensive therapy, in order to exclude the secondary nature of the increase in blood pressure at the place of residence, an examination was prescribed: ultrasound of the abdominal organs, kidneys (no pathology was revealed). During computed tomography of the abdominal organs, a formation was detected in the left adrenal gland (round, homogeneous with uneven contours, isodense volumetric, measuring 24x20 mm, with a density of up to 34 units H). For the purpose of further examination and selection of treatment tactics, the patient was referred to an endocrinologist at the RCH.

The patient's condition upon admission is satisfactory. Physical development indicators: height - 154 cm, weight - 75 kg, BMI - 31.6 kg/m2. Hypersthenic build. The skin and visible mucous membranes are of physiological color, clean, moist. Subcutaneous fat is excessively developed, distributed evenly. The thyroid gland is not enlarged by palpation, soft elastic, mobile, painless. Peripheral lymph nodes are not enlarged. In the lungs, vesicular breathing, no wheezing. RR - 18 per min. BP - 140/90 mm Hg. HR - 76 beats/min. Heart sounds are rhythmic, clear. The abdomen is soft, painless. The liver is not enlarged. Swelling of the shins.

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Diagnostics of a nonclassical pheochromocytoma.

Laboratory and instrumental research data.

Complete blood count: hemoglobin - 148 g/l, erythrocytes - 5.15x1012, leukocytes - 6.9x109, n - 1%, c - 67%, lymph. 31%, monocytocytes 1%, platelets - 366,000 in μl, ESR - 23 mm/h.

General urine analysis: specific gravity 1007, protein - negative, leukocytes - units in the field of view, epithelial cells - 1-2 in the field of view.

Blood biochemistry: total bilirubin - 12.1 μmol/l (3.4-20.5 μmol/l), ALT - 18 U/l (0-55 U/l), AST - 12 U/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).

Thyroid hormones: TSH - 0.97 μIU/ml (0.3500-4.9400 μIU/ml), free T4 - 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 detected.

ECG: Sinus rhythm with heart rate of 77 beats per minute. Deviation of the electrical axis to the right. PQ - 0.20 sec, P wave - pulmonary type. Conduction disorder along the right bundle branch of His.

Thyroid gland ultrasound. Volume 11.062 ml. Isthmus 3.3 mm. Gland contours are wavy. Structure with unclear hypoechoic areas up to 4 mm, diffusely heterogeneous. Echogenicity is normal. Blood flow intensity during color Doppler imaging is normal. In the right lobe, closer to the lower pole, an isoechoic node with a diameter of 3.5 mm is visualized, next to it behind is a weakly hyperechoic area of 4.8x4 mm.

CT scan of the abdominal organs with contrast: in the area 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 reliable heterogeneous accumulation of contrast agent up to 86 HU.

Hormonal blood test: aldosterone - 392 ng/ml (normal 15-150 ng/ml), renin - 7.36 ng/ml/hour (normal 0.2-1.9 ng/ml/hour), angiotensin - 1-5.54 ng/ml (normal 0.4-4.1 ng/ml), cortisol - 11.1 mcg/ml (normal 3.7-24.0 mcg/ml); urine: normetanephrines - 3712.5 mcg/day (normal 30-440 mcg/day), free metanephrines - 25 mcg/day (normal 6-115 mcg/day).

Based on laboratory and instrumental examination methods, the following diagnosis was established: "Space-occupying lesion of the left adrenal gland (hormonally active). Pheochromocytoma. Primary hypothyroidism, moderate severity, compensated. Arterial hypertension grade 2, stage 2. CHF 1, FC 2. Risk 4. Obesity grade 1, exogenous-constitutional genesis."

Increased levels of metanephrines in urine, CT data characteristic of pheochromocytoma (in CT, the non-contrast density of pheochromocytoma is usually more than 10 Hounsfield units - often more than 25 HU), pheochromocytoma is characterized by a round or oval shape, clear contour, heterogeneous structure: fluid areas, necrosis, calcifications, hemorrhages speak in favor of the established diagnosis. With a three-fold increase in normetanephrine in urine and a two-fold increase in metanephrine, the presence of a chromaffin tumor is beyond doubt. The increase in the level of aldosterone, renin, angiotensin 1 is most likely secondary, due to the presence of arterial hypertension in the anamnesis for 5 years.

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Treatment of a nonclassical pheochromocytoma.

The main objective of drug therapy is to prepare for surgery. The drug of choice for preoperative preparation of patients with pheochromocytoma is a selective prolonged alpha1-adrenergic blocker - doxazosin (cardura). Doxazosin is dosed based on the hypotensive effect and disappearance of hypovolemic manifestations (orthostatic test). In this patient, while taking cardura at a dose of 4 mg 2 times a day, stabilization of blood pressure and a negative orthostatic test (130/80 mm Hg in a horizontal position and 125/80 mm Hg in a vertical position on the 13th day of cardura therapy) were observed, 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 alpha-adrenergic receptors.

At the time of hospitalization, the patient received an adequate dose of L-thyroxine 100 mcg per day, so it was decided to leave thyroid hormone replacement therapy unchanged. A successful surgical removal of the hormonally active tumor of the left adrenal gland was performed.

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

During further observation, blood pressure was stable and normal. The patient was discharged home with improvement.

Thus, this patient had a non-classical picture of pheochromocytoma (there were no malignant hypertensive crises with subsequent development of hypotension, tachycardia, blood pressure values corresponded to the moderate degree of arterial hypertension, the presence of excess body weight), as a result of which diagnosis based on clinical manifestations was difficult, which did not allow the correct diagnosis to be made at the onset of the disease and adequate treatment to be prescribed.