Paragangliomas are rare tumors, with an overall estimated incidence of 1/300,000.(1)
When the same type of tumor is found in the adrenal gland, they are referred to as a pheochromocytoma.(1)
How Fast Do Paragangliomas Grow?
Paraganglioma is usually a benign tumor that is found in very a smaller number of cases and it consists of a special type of neuronal cell which is usually derived from neural crest cells and is responsible for the production of a variety of hormones. It is a neuroendocrine tumor associated with the production of proteinaceous hormones also known as catecholamines like epinephrine, norepinephrine, adrenaline, and noradrenaline, etc. It is a slow-growing tumor and usually remains up on the same site because most of them are not known to produce a metastatic tumor. It does not produce any symptoms in most of the cases and remains unknown until it produces some symptoms or become an incidental finding.
Those paragangliomas which get converted to a metastatic tumor are the only ones that grow at a moderate to fast pace. These tumors have to be removed very quickly because they are known to produce a lot of symptoms.
What Is The Survival Rate Of Paraganglioma?
Since it is a benign and asymptomatic tumor in most cases, so it does not produce a lot of problems for the patient. These paragangliomas can be divided based on the presence of parasympathetic or sympathetic neuronal cells. If the tumor consists of parasympathetic neurons then it is more likely to be present on the base of the skull near the ninth and tenth cranial nerve and is asymptomatic in almost 95 to 98 % of cases. If the tumor consists of sympathetic neurons then it is more likely to be present in the abdomen and pelvis and produce a lot of symptoms in about 20% cases.
Since it is a benign tumor, whenever it starts producing symptoms then it should be taken very seriously and treated immediately. Surgery is the first line of treatment and also the treatment of choice for any paraganglioma because it is almost a hundred percent curable if diagnosed in the earlier state (benign state) and symptoms also subside immediately. It carries a very good prognosis because of its non-recurrence nature.
If any of the paragangliomas especially the sympathetic one turns into a metastatic tumor and is found in various places in the body, it becomes very difficult to cure the tumor because it is almost impossible to remove all the tumors surgically. The other interesting and demoralizing fact about paraganglioma is, it is a neuroendocrine tumor that does not respond well to any type of chemotherapy or radiotherapy. So, it becomes very difficult for an oncologist to treat a metastatic paraganglioma.
Metastatic paragangliomas also carry a high risk of reoccurrence even after successful treatment for the first time because of the presence of some of the malignant cells at unknown locations. But overall paraganglioma is associated with a very good prognosis and a very low recurrence rate.(2)
It is a benign tumor and does not grow at a fast rate in most cases. It can defy all odds to become metastatic cancer and grow at a fast pace in a very small number of cases with the epidemiology of less than one such case per million.
Whenever it is associated with a low paced growth then there are very good chances of complete removal of the tumor surgically. The patient becomes also symptomless within few weeks but in some cases, it can be present at such a site that it is encasing a cranial nerve and becomes a very difficult case for removal. Overall, it carries a very good prognosis with a high success rate.