Malignant or Benign?

On the top of my most recent MRI scan results it states that I have "Metastatic Paraganglioma and SDHB genetic mutation". I do have the SDHB mutation (more on that later) but do not have "diagnosable" metastatic paraganglioma.

How do I know?

In the paraganglioma and pheochromocytoma world metastatic and malignant mean the same thing. It is actually the metastases of these tumours that is the only medically accepted indication of malignancy. It is impossible to tell the difference between a benign para and a malignant one just by looking at it through scanning technology or pathology. Pathologists and radiologist are continually trying to find ways to differentiate between malignant paraganglioma and benign. The only way to tell is by the position of the tumours.

(Note: Paraganglioma and Pheochromocytoma are the same tumour. The name differences simply show that "paras" are outside the adrenals, while "pheos" are paragangliomas of the adrenals)

While the paragangliomas I have had surgically removed have shown the malignant tendencies of tissue and vascular invasion, for paraganglioma this is not seen as an indication of malignancy. This gets very confusing for paraganglioma and pheochromocytoma patients (and doctors) as you may have a tumour which shows all the characteristics of malignancy, but are told that your tumours are benign. While most cancer definitions include the invasion and destruction of local healthy tissue, this does not apply to paraganglioma. It is only when the tumours invade organs, bone or distant lymph nodes, that they are called malignant.

For me, all of my tumours are in areas that have the sympathetic and para-sympathetic nervous system. As paraganglioma are tumours of these two nervous systems, they are considered primary tumours (benign) if they grow along these sites despite how many I may have. This means that they probably grew there as a results of cells behaving badly in the spot where they grow.

When the paraganglioma cells break off from these primary tumours and spread through either lymph fluid or blood, they tend to get caught in areas like lymph nodes, bones, liver and lungs. When this happens the term metastatic is used and the tumours are deemed malignant.

If they are benign are they life threatening?

"Benign" is a word that should not be associated with these tumours. "Benign" means "nice, friendly, not dangerous". Check out any dictionary. Tumours that can cause serious health issues and death should not be called "benign". Unfortunately, for many patients, people often see the term "benign" and decide that this kind of tumour is no more of a health risk than a pimple. This can extend to medical personnel who may then dismiss the patient as not having a serious health issue.

For many people with paraganglioma and pheochromocytoma the symptoms include huge amounts of adrenaline being pumped through their system. This can result in heart and organ damage and is life threatening. Some of the symptoms are severe hypertension, heart attack, stroke and death. Neck tumours, especial tumours attached to the vagal nerve, can cause choking and breathing issues. If the tumour is left too long, it can spread into the skull or cut off the vagas nerve which is part of the control centre for parasympathetic nervous system. There are obviously serious risks here. Tumours of any kind can grow to a point where they put pressure on surrounding nerves and organs and cause severe distress, pain and death.

Seemingly "benign" paraganglioma can suddenly turn metastatic/malignant and spread. While there is as yet no way to define why this happens the common links seem to be size, position and the SDHB genetic mutation. However, as with all things there are always exceptions.

While the benign/malignant discussion will continue until someone comes up with an answer, the best description we have is that it is "degenerative". For many, like me, it is a life-long, life threatening disease. And for many there is still no cure.