From the Guidelines
Pheochromocytoma diagnosis and treatment involve a multidisciplinary approach. The diagnosis of pheochromocytoma is based on clinical presentation, endocrine assessment, and modern imaging techniques such as CT/MRI of the abdomen and chest CT, supplemented by isotope functional imaging like FDG-PET in selected cases 1.
Key Diagnostic Steps
- Endocrine assessment for excess hormone production
- Modern imaging techniques (CT/MRI, chest CT, and FDG-PET in selected cases)
- Histological diagnosis by an experienced pathologist using morphological, mitotic, and immunohistochemical parameters
Treatment Approach
- Surgical excision is the mainstay of treatment for localized and locally advanced pheochromocytoma, with the goal of achieving complete resection (R0 resection) 1
- Preoperative preparation is crucial to control blood pressure and prevent hypertensive crises, using medications such as phenoxybenzamine, doxazosin, or calcium channel blockers, with the addition of beta-blockers if necessary 1
- Intraoperative management involves careful monitoring and control of blood pressure, using medications such as magnesium sulfate, phentolamine, or nitroprusside as needed 1
- Postoperative care includes careful management of fluid administration to prevent hypotension, as well as monitoring for potential complications such as adrenal insufficiency 1
Additional Considerations
- Genetic screening should be considered in patients with pheochromocytoma, as certain mutations (e.g. SDHB) can increase the risk of malignant tumors 1
- Long-term follow-up is essential for patients with pheochromocytoma, with regular clinical, imaging, and biochemical assessments to monitor for recurrence or metastasis 1
From the Research
Diagnosis of Pheochromocytoma
- The diagnosis of pheochromocytoma depends mainly upon the demonstration of catecholamine excess by 24-h urinary catecholamines and metanephrines or plasma metanephrines 2
- Diagnostic screening includes measurement of catecholamines and their metabolites (metanephrines) in plasma and/or urine 3
- Pharmacological testing (e.g. clonidine suppression test) may be indicated in patients with moderately elevated catecholamines or when the diagnosis is still uncertain 3
- Several imaging techniques are applied to localize the tumor, including abdominal CT scan, magnetic resonance imaging (MRI), MIBG-scanning, and positron emission tomography (PET) based techniques 3, 2
Treatment of Pheochromocytoma
- Laparoscopic and adrenal sparing surgical intervention following preoperative alpha-blockade is the treatment of choice and usually curative 2
- Minimally invasive adrenalectomy (laparoscopic and retro-peritoneoscopic) allows earlier mobilization and recovery, reducing the risk of pulmonary infections and thromb-oembolic complications, and is associated with lower morbidity and mortality rates than traditional surgery 4
- Alpha 1 blocker therapy, alone or in combination with beta blockers, calcium antagonists, and plasma volume expansion, is the most commonly used preoperative treatment protocol 4
- In malignant pheochromocytomas, radiotherapy and chemotherapy are palliative treatment options 2
Genetic Aspects
- Approximately one out of four pheochromocytomas turn out to be hereditary entities, screening for genetic alterations is important 2
- Pheochromocytoma can be associated with certain genetic syndromes such as multiple endocrine neoplasia type 2 (MEN 2), neurofibromatosis (NF) and von Hippel-Lindau (VHL) syndrome 5
- Genetic testing will increasingly be the key factor in estimating the life-long risk for development of recurrent disease, contralateral disease or malignant dedifferentiation, thus influencing follow-up protocols 4