One-third of all paragangliomas (PCC/PGL) are hereditary(1).
According to various study populations, the overall 5-year survival rate of metastatic paraganglioma varies between 35-60%(2).
Prognosis Of Paragangliomas
A complete cure is achieved with the removal of paragangliomas and patients leading a normal life. The overall prognosis for benign tumors is excellent with a 5-year survival rate of pheochromocytomas >95%. However, the prognosis of metastatic tumors is poor with a 5-year survival rate of around 35-60%(2),(3).
Paragangliomas (PGL) are rare chromaffin cell tumors that develop along with the autonomic nervous system (sympathetic and parasympathetic nervous system) from the skull base to the pelvis. A paraganglioma found in the sympathetic tissue of the adrenal gland is termed as pheochromocytoma (PCC); whereas, in extra-adrenal location, it is known as sympathetic paragangliomas or parasympathetic head and neck paragangliomas (HNPGLs). The annual incidence of these tumors is around 2-10 per million cases. They can occur in all age groups but are mostly seen in 40-50 age groups with equal sex predilection. Nearly one-third of all these tumors are hereditary. About 80-85% of these tumors are benign with 10-15% being malignant. These malignant tumors can spread to lymph nodes, lungs, liver, and bone(1),(2).
Home Remedies For Paragangliomas
There are no particular home remedies for paragangliomas since it is a tumor that needs to be resected. However, patients with paragangliomas can eat a well-nourished diet to avoid any unwanted weight loss that is usually associated with the disease. Certain foods, such as nicotine, caffeine, and amine-rich foods should be avoided. Since people with paragangliomas may experience postural hypotension, it is advised they maintain proper fluid levels and drink enough water along with maintaining sitting posture and avoiding sudden standing attempts. Anxiety and stress should be avoided either by other activities or distractions to avoid precipitating symptoms.
The symptoms associated with paragangliomas are due to the excess production of catecholamines, size, and location of the tumor.
Hypertension either prolonged or paroxysmal is the most common sign along with spells of severe headache, excessive sweating and palpitations (the classical triad). These spells may last minutes to days, and vary in frequency or may occur spontaneously or triggered by some external stimulation. Other symptoms include nausea, vomiting, anxiety, weakness, hyperglycemia, cardiovascular complications (myocardial infarction, heart failure, cerebrovascular accidents, and sudden death). Paragangliomas of the head and neck region may produce symptoms of dysphagia, hoarseness, ringing in ears, hearing loss, cranial nerve palsy, cervical or neck masses(1).
Diagnosis Of Paragangliomas
The initial diagnosis of paragangliomas is done through biochemical testing for hypersecretion of catecholamines (epinephrine, norepinephrine, and dopamine) and tested for their metabolites (metanephrines, normetanephrine, and methoxytyramine). These metabolites can be found through plasma free metanephrines (MN) and/or 24-hour urinary excretion of fractioned MNs, which provide a sensitivity of >96%. Before testing nicotine, caffeine, amine-rich foods, and antihypertensive medications and tricyclic antidepressants should be avoided since they may give false-positive results. After the biochemical testing, the locations and staging of the tumor can be done with imaging techniques, which include MRI, CT scan, MIBG (I-metaiodobenzylguanidine) scintigraphy, and positron emission tomography (PET) scanning(1).
Management Of Paragangliomas
The treatment modality is surgical resection of the tumor with laparoscopy as the preferred method of surgery. To prevent surgical complications, pre-surgery medications need to be started, including alpha-blockers (doxazosin and phenoxybenzamine) and beta-blockers (atenolol and propranolol). The pretreatment reduces the perioperative mortality to below 1%. Postoperative complications include hypoglycemia and hypotension that subside within days. Postoperative follow up is necessary due to the possibility of recurrence and metastases since histologically benign tumors cannot be differentiated from malignant tumors. Head and neck paragangliomas can be managed with surgery and radiotherapy(1).
Malignancy is identified only when the distant spread has taken place with 10% of pheochromocytomas and 10-20% of sympathetic paragangliomas being malignant; head and neck paragangliomas are usually benign. Malignancy is usually associated with SDHB (succinate dehydrogenase type B), MAX (myc-associated factor X), and FH (fumarate hydratase) mutations. The curative options for malignant tumors are limited, palliative tumor resection, therapeutic doses of I-MIBG, and chemotherapy are employed.