Clinical and epidemiological characteristics of patients with pheochromocytoma and retroperitoneal paraganglioma

Introduction. Pheochromocytoma/paraganglioma (PPGL) is a rare hormonally active tumor of chromaffin tissue. Delayed diagnosis of this disease can lead to severe, even fatal, complications. Surgical treatment of PPGL without the necessary preoperative preparation can cause intraoperative uncontrolled hemodynamic impairment. In addition, since 2017, PPGL has been recognized as a malignant tumor due to the presence of metastatic potential. All these factors determine the need for early diagnosis of PPGL, which is based, first of all, on knowledge of the clinical picture by specialists in various fields.

Aim. To check and confirm the epidemiological characteristics of PPGL as well as the most common clinical symptoms in the whole group of PPGL and in subgroups of clinical forms; to form a risk group for screening for PPGL.

Materials and methods. There were enrolled 353 patients in the study. These patients were operated on at the St. Petersburg State University Hospital in the period from 2010 to 2022 inclusive. All the patients were verified PPGL diagnosis histologically. Clinical symptoms were assessed: a) retrospectively, by analyzing complaints and anamnesis from the initial examination in medical histories upon admission to the hospital for surgical treatment b) both in the entire group as a whole and in subgroups of clinical forms (paroxysmal, constant, mixed, asymptomatic). Statistical analysis was performed using the Python 3.11 programming language (Python Software Foundation, USA). descriptive statistics of quantitative characteristics are presented in the form of medians, first and third quartiles in me format [q1; q3], categorical features – in the form of absolute and relative frequencies in the format n (%). the frequency histogram was constructed using the Matplotlib 3.8.2 library.

Results. PPGL is most common in women over 40 years of age. The most common symptoms of PPGL are the following: a feeling of rapid heartbeat (55.95%), headache (41.07%), arterial hypertension (AH) in the form of hypertensive crises (55.65%), constant increase in blood pressure (38.39%), episodes of moderate increases in blood pressure (24.11%), sweating (30.65%), general weakness and fatigue (26.19%), hot flashes or chills (24.11%). These symptoms are formed into the criteria for screening for PPGL. In the asymptomatic clinical form – lower back pain is more common (14%); with paroxysmal – panic attacks (10%); with mixed – thirst and dry mouth (10%), and with constant form – the actual constant nature of hypertension (100%).

Conclusion. In a large sample of patients with a rare pathology, the most characteristic symptom complex was identified, characteristic of both PPGL and individual clinical forms. A risk group has been formed for screening for PPGL.

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