Most people probably have not heard of the term “pheochromocytomatosis.” Pheochromocytoma itself is already an odd word, let alone pheochromocytomatosis. The more I practice medicine, the more evident is to me that a disease’s presentation and natural history could be affected by medicine itself. Even 30 years ago, pheochromocytoma was still mostly suspected based on clinical symptoms; with the wide use of imaging, most cases of pheochromocytoma nowadays are found incidentally by imaging. Pheochromocytomatosis is likely a modern phenomenon associated with laparoscopic adrenalectomy.
A pheochromocytoma is surrounded by a fibrous capsule. During a surgical operation, the surgeon separates the pheochromocytoma from the normal tissue and organs and removes the tumor with the capsule intact, so that no tumor is left behind. That usually did not pose a problem in the era of open operation to remove the pheochromocytoma. During a laparoscopic operation, inexperience in surgical skills or accidents can result in rupture of the capsule and tumor cell spillage into the abdomen. The spilled tumor cells can form numerous tiny tumors that may grow to a few centimeters, which is called pheochromocytomatosis. It is not clear how often tumor capsule rupture occurs during adrenalectomy and how often pheochromocytomatosis occurs in those with tumor capsule rupture.
Once pheochromocytomatosis happens, it becomes an incurable problem. Pheochromocytomatosis, however, is indolent and may manifest itself years after the original operation. As pheochromocytomatosis is very rare, we are not sure how to best control it. Reoperation is usually done to remove visible gross tumors. Patients are then monitored. If the tumors grow again, MIBG radiotherapy or PRRT can be tried. If the tumors are still growing after the radiotherapies, somatostatin analogs and other agents may be used.
Hello Doctor pheo In April 2020, I had a paraganglioma surgery near my kidneys. I have been found to have the sdhb mutation and I do a 24 hour urine test every 6 months. what I was wondering was, when the first diagnosis was made, my values were diagnosed as noradrenaline 203 <80 metanephrine 31<261 and normatenaphrine 1536<390. The urine tests I did every 6 months were in constant range. Doctor, I was wondering if my metanephrine level was at the level of 10<261 in my 3 tests so far, but in my last test it was 210<261. Is it normal for it to rise this much in 6 months, all of my other values are below the range, only the metanephrine level has increased abnormally. It wasn't like this even when the first diagnosis was made, I wonder if you could help me, dear doctor, thank you.
ReplyDeleteDear Ramazan,
DeletePheo or para caused by SDHB mutation usually secretes normetanephrine as the main marker, which was the case for you. I would not worry about the metanephrine too much as of now, but would see how it will be in 6 months.
Dr. Pheo
Thank you for your answer, doctor. Well, I've read many articles that sdhb paragangliomas are bad and the rate of metastasis is 70% during their lifetime, can you give me information about them, I'm really afraid. Also, if you have any nutrition and advice, I am gladly waiting. thank you doctor
DeleteDear Ramazan,
DeletePheo or para caused by SDHB mutation indeed can be malignant. On the other hand, so long as you follow the surveillance program closely, the doctors should be able to detect metastasis early and manage it optimally. There is no specific nutrition intervention that would prevent metastasis. Living a healthy lifestyle is always a good idea.
Dr. Pheo
Hi Dr Pheo,
ReplyDeleteI’d like some advice on my situation if possible.
10 yrs ago I began experiencing constant terror, not anxiety but pure fear. I was put on a ton of pysch meds which I had adverse affects to and completely stopped 5 yrs ago. The medications I discovered actually increased my symptoms and after ceasing them I found the terror decreased but I was left with debilitating fatigue for many years and still feeling unwell. 4 months ago out of nowhere the intense terror returned. It’s like my body is stuck in fight or flight. I barely sleep, have lost 30 pounds, can barely eat, constant sweating and heart palps etc but it’s not from thought provoked anxiety, it’s like my body is being chemically altered. The thing is I don’t have high BP. Recent blood tests show very high cortisol levels. I’ve seen Drs, psychologists, natropaths and yet nothing helps. The symptoms are almost constant yet sometimes in the afternoon or evening like a switch it can all just stop only to start up again the next morning.
Any advice would be greatly appreciated
Renae
Dear Renae,
DeletePheo is possible. I recommend testing your plasma metanephrines to examine whether you have pheo.
Dr. Pheo
Hi Dr Pheo,
ReplyDeleteI have a few questions:
1 - Is it possible for someone with pheo to sometimes have healthy looking blood and 24h urine catecholamine results? For example, someone who has about one attack a day, but does not have an attack before or during those tests.
2 - Is heavy sweating supposed to happen during attacks? Or is it possible for someone with pheo to have ludicrous sweating at the slightest effort, but never during attacks?
3 - Is it possible to have had pheo since childhood?
Thanks for your involvement.
Dear Max333,
DeleteHere are my answers:
1. Yes for blood and urine catecholamines. That's why those are not the preferred tests. Blood or urine metanephrines are much better tests; they remain elevated whether there is an attack on the day of test or not, if the person has a pheo.
2. Sweating usually only happens during an attack.
3. It is possible.
Dr. Pheo
Hi Dr. Pheo,
ReplyDeleteThank you very much for this blog - It is quite challenging to find helpful information and answers, and I'm so grateful to have found this. I have known that something wasn't right with me for many years, but it was dismissed by my doctor so I began to believe that it was just in my head, and I need to power through - and that's exactly what I've been doing for 5+ years. Earlier this year, I discovered that I have extremely high BP during my 'spells', and this new development led my doctor to start bouncing me around from specialist to specialist to figure out what's going on, but initial testing came back normal and I started to feel uncomfortable with some of the additional tests and drugs that were being prescribed to me. I have suspected a pheo for several months now based on my own research, but nobody wanted to test me for it because my "symptoms weren't right". I finally self-referred to an endocrinologist who immediately requested blood work. My results: free metanephrine 6.3 nmol/L (<0.5), free normetanephrine 6.8 nmol/L (<0.9). I have a CT scheduled in a few weeks and have also started alpha blockers. My question for you... I am a very active person - I play several team sports and have a family trip including an amusement park visit coming up - are there things that I should be avoiding while I wait for my CT? I am guessing that I should probably take a break from contact sports and roller coasters, but was hoping to get your advice on the do's and don'ts for my current situation while I wait for next steps. Thank you!
Dear Unknown,
DeleteThanks for your compliments! I suggest you avoid contact sports and roller coaster etc before the tumor is found and taken care of.
Dr. Pheo
Thank you Dr. Pheo! Do you have any surgeons in Southern California that you would recommend?
DeleteDear Unknown,
DeleteDr. Michael Yeh at UCLA is an expert surgeon on pheo.
Dr. Pheo
Is it possible for someone to have high normetanephrines and hypotension at the same time? What other things aside from a pheo could cause that?
ReplyDeleteDear Anonymous,
DeleteSorry for the late response. It is possbile. The normetanephrine level is usually very high and the hypotension is usually preceded by hypertension. If the normetanephrine is only slightly high, the hypotension is unlikely caused by a pheo.
Dr. Pheo
Greetings, Dr. Pheo
ReplyDeleteI have one question, has the criteria for pheo changed? I know that we used to think blood work had to be about 4x the limit to be considered pheo...has that now changed to 2x?
Btw, I recently had a PET/Dotate scan which shows 2 small pheo's on my remaining bit of my left adrenal gland and my pancreas is lighting up.
Hope all is well and thank you so much.
Frances
Dear Frances,
DeleteNice to hear from you again. The specific levels of pheo markers that are considered "positive" should be determined with the tumor size and kind considered. For example, if one has a 1-cm adrenal tumor that looks like a pheo on imaing, even 1.5-fold high pheo marker level could indicate pheo diagnosis.
Pancreas often lights up even in people without a tumor. Where exactly is the uptake in the pancreas? Head, neck, body, or tail?
Best regards,
Dr. Pheo
Thanks for the quick reply. The dotate scan lights up in the distal body and tail of the pancreas. I normally wouldn't worry, it's just that another sister died recently of pancreatic cancer.
ReplyDeleteMy family certainly has had more than our fair share of cancer. It's a shame.
Thank you for all your kind help.
Frances
Dear Frances,
DeleteThe locations of the pancreas uptake in the DOTATATE PET is concerning for nuroendorine tumors in the pancreas or lymph nodes around the pancreas. They need to be addressed.
Best regards,
Dr. Pheo
Thank you. I go for an mri this afternoon.
ReplyDelete