From the Guidelines
A paraganglioma is a rare tumor that develops from paraganglia, which are clusters of cells that arise from the autonomic nervous system, and can occur anywhere in the body, commonly in the head, neck, chest, abdomen, or pelvis, with approximately 10-30% being hereditary, linked to genetic mutations 1.
Definition and Characteristics
Paragangliomas form from chromaffin cells, which produce and release hormones like adrenaline (epinephrine) and noradrenaline (norepinephrine). When these tumors develop in the adrenal glands, they are specifically called pheochromocytomas. Many paragangliomas are non-cancerous (benign), but some can be malignant.
Symptoms and Diagnosis
Symptoms vary depending on location but often include high blood pressure, headaches, sweating, and rapid heartbeat if the tumor secretes hormones 1. The screening test of choice for pheochromocytoma/paraganglioma is measurement of circulating catecholamine metabolites, with plasma free or urinary fractionated metanephrines being the most sensitive and specific tests.
Treatment and Management
Treatment typically involves surgical removal of the tumor, sometimes preceded by medication to control blood pressure. Before surgery, patients usually need alpha-blockers like phenoxybenzamine or doxazosin for 1-2 weeks to prevent dangerous blood pressure spikes during tumor manipulation. Early diagnosis is important because untreated hormone-secreting paragangliomas can lead to life-threatening cardiovascular complications.
Key Considerations
It is essential to consider the diagnosis of paraganglioma in anyone with resistant hypertension, given the high morbidity and mortality of not treating these tumors, and the fact that one-third are inherited 1. Assessment of the family history should include specific inquiry regarding relatives with sudden death, and it should detail the spectrum of tumors associated with hereditary paraganglioma syndromes.
From the Research
Definition and Characteristics of Paraganglioma
- Paragangliomas are rare tumors that arise from extra-adrenal paraganglia 2
- They can be diagnosed in various clinical settings, including signs and symptoms related to catecholamine hypersecretion, mass effect symptoms, incidental finding on imaging, or family screening for hereditary paraganglioma 2
- Paragangliomas that hypersecrete catecholamines may cause signs and symptoms identical to those in patients with hyperfunctioning adrenal pheochromocytoma 2
Treatment and Management of Paraganglioma
- The treatment of choice for paraganglioma is surgical resection, with most tumors being benign and able to be excised totally 2
- Chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) can be used for palliative treatment of malignant paraganglioma and pheochromocytoma 3
- Metyrosine can be used as a therapeutic adjunct to decrease catecholamine production in select patients with paraganglioma and pheochromocytoma 4
- Emerging treatments for advanced or metastatic paraganglioma include radiolabeled MIBG, systemic chemotherapy, cytoreductive techniques, novel radiopharmaceuticals, radiotherapy, immunotherapy, and experimental therapies 5, 6