Pheochromocytoma

Abstract

Pheochromocytomas are rare tumors originating in the adrenal medulla. They may be sporadic or in the context of a hereditary syndrome. A considerable number of pheochromocytomas carry germline or somatic gene mutations, which are inherited in the autosomal dominant way. All patients should undergo genetic testing. Symptoms are due to catecholamines over production or to a mass effect. Diagnosis is confirmed by raised plasma or urine metanephrines or normetanephrines. Radiology assists in the tumor location and any local invasion or metastasis. All the patients should have preoperative preparation with α-blockers and/or other medications to control hypertension, arrhythmia, and volume expansion. Surgery is the definitive treatment. Follow up should be life-long.

Preoperative Management of Pheochromocytoma and Paraganglioma

“…With insufficient preoperative antihypertensive management or untreated hypovolemia, the hemodynamic instability during surgical treatment of PPGL may be lethal. Therefore, preoperative management, which includes hypertension control and expansion of blood volume, is extremely important (12,13). The main goal of preoperative management of PPGL is to normalize blood pressure and heart rate, restore effective circulating blood volume, improve metabolic condition, and prevent a patient from CA storm and hemodynamic instability during surgery (8).…”

“…It is proved that excessive CAs release results in perioperative cardiovascular instability in the patients with PPGL, so the administration of drugs which inhibit the CA biosynthesis may be beneficial for the treatment of PPGL (42). Metyrosine, a CA synthesis inhibitor, inhibits tyrosine hydroxylase, which catalyzes the conversion of tyrosine to dihydroxyphenylalanine (DOPA) ( Figure 1), the rate-limiting step of the CA synthesis pathway (12,43). Therefore, metyrosine has been used as one of the approaches for the management of PPGL, especially when phenoxybenzamine is unavailable in some areas, such as Italy.…”

Histopathological Analysis of Tumor Microenvironment and Angiogenesis in Pheochromocytoma

“…Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are relatively rare tumors originating from the adrenal medulla at proximately 2 to 9.1 per 1 million adults frequently associated with cardiovascular complications due to excessive catecholamine production (1)(2)(3). The difference between PHEOs and PGLs depends on the primary sites, i.e., intra-or extra-adrenal glands (4), and the former one termed as PHEOs (5).…”

Radiological diagnostics in patients with pheochromocytoma – do we need to prepare? Review of the literature

“…Pheochromocytomas (pheos) are chromaffin cell tumours derived from the neural crest and they are associated with catecholamine production 1 . Pheos are rare tumours, with an annual incidence of 2 to 9.1 per 1 million adults and may correspond up to 60% of all adrenal incidentalomas 2 . The majority of pheochromocytomas are benign but up to 25% of tumours may be malignant 3 .…”