PHEOCHROMOCYTOMA AND PARAGANGLIOMA: CLINICAL PICTURE, DIAGNOSIS, TREATMENT, FOLLOW-UP AND PROGNOSIS - A LITERATURE REVIEW
Abstract
Introduction and aim: Pheochromocytoma and paraganglioma (PPGL) are tumors of chromaffin cells. The incidence of this disease is 2 to 8 cases per million. The peak incidence occurs in the 3rd to 5th decade of life. This article aims to review reports on the clinical picture, diagnosis, treatment, follow-up and prognosis of PPGL.
State of knowledge: Excess catecholamines secreted by the tumor can cause symptoms such as headache, sweating, hypertension, heart palpitations, and can also lead to organ damage. The diagnosis of PPGL includes the assessment of the clinical picture, biochemical tests, imaging studies, family history and genetic tests. The first-line test in the diagnosis is the determination of free methoxy-catecholamines concentrations in plasma. Surgical excision is the primary treatment for most localized PPGLs. To minimize the risk of perioperative cardiovascular complications in patients with pheochromocytoma and hormonally active paraganglioma, preoperative adrenergic receptor blockade is recommended. A minimum of 10 years of annual follow-up is recommended for every patient with a resected PPGL. When radical treatment is not possible, the therapeutic goals shift towards slowing down tumor progression and maintaining quality of life. The strongest predictor of a poor prognosis is the presence of metastases at diagnosis.
Conclusions: PPGLs are clinically challenging diagnoses. It is important to evaluate the diagnosis and treatment in a multidisciplinary team. Treatment should be tailored to each patient individually to protect against side effects and ensure a favorable prognosis. Further research is needed to increase the chances of early diagnosis and improve patient outcomes.
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