Background: Paraganglioma is a rare neuroendocrine tumor from extra-adrenal chromaffin cells of the sympathetic paravertebral ganglia of thorax, abdomen, and pelvis. About 1520% of chromaffin-cell tumors are paragangliomas. In most cases paragangliomas produce catecholamines in large concentrations and the lack of treatment can lead to cardiovascular and cerebral catastrophes. That is why early diagnosis of this tumor is so important and, consequently, knowledge of its diagnostic criterion, too.
Clinical case: A 59-year-old man was presented to a cardiologist for rapid increasing of blood pressure (BP) (>220/120 mmHg), which was accompanied by severe headache and epigastric pain in September 2017. From anamnesis it is known: the disease began suddenly and had paroxysmal flow. The first attack lasted for about 3 minutes and finished itself. Then such attacks occured with different frequency (from 1 per month to several times per week) and were connected with physical activity. The cardiologist suspected diagnosis of pheochromocytoma. The result of urinary fractionated metanephrines measurement showed its levels higher than normal rage but did not confirm suspected diagnosis reliably. Then patient referred to a surgeon in May 2018. On the basis of patients complaints on epigastric pain during the attacks and the neck inflection of the gallbladder revealed by ultrasonography, it was decided to carry out cholecystectomy. A week after the operation the patient referred to the endocrinologist because of the continuing attacks. The diagnosis of pheochromocytoma was suspected again, the urinary fractionated metanephrines measurement was repeated, which revealed elevated normetanephrine level of 1008 μg/day (normal rage:<700 μg/day). A CT of retroperitoneal space revealed a spherical mass sized about 17×16×18 mm in paraaortal region under left renal vein. The retroperitoneal tumor was removed laparoscopically in August 2018. Pathological examination of the removed mass showed a paraganglioma with uncertain potential of biological behaviour. BP was normal and remained within range without the need of any antihypertensive medication until discharge from hospital. The patient is asymptomatic and normotensive during his follow-up visits.
Conclusion: This clinical case shows how a tumor with bright and specific symptoms can be revealed only in several years and after referring to several specialists. Also, this case highlights the importance of knowledge of diagnostic process of different diseases. In the present case the paraganglioma would have been revealed earlier if only the CT of retroperitoneal space had been done just after getting the results of free metanephrines, which were higher than normal.
18 - 21 May 2019
European Society of Endocrinology