One important feature of pheos is that they always grow. The growth speed of pheo, however, is only known for patients with von Hippel Lindau disease. For people with true sporadic pheo (~70% of all pheos), we have little knowledge on the growth speed of their pheos. The reason is that familial pheos can be monitored but sporadic pheos are usually resected once they are diagnosed. Only under two conditions, patient's choice of delaying surgery and omission of pheo testing of incidentally-identified adrenal mass, the growth speed of sporadic pheos can be assessed.
I recently took care of a patient with likely sporadic pheo. This patient's adrenal pheo increased from 0.6 x 1.2 cm to 3.0 x 3.6 cm in 6 years. This patient is among the few I know with recorded growth speed of a sporadic pheo.
The information on pheo growth speed is much needed to tell patients what they will expect of their tumor. I would like to do a survey of the growth speed of pheo, whether with genetic mutations or not. If you have a pheo that was imaged multiple times, please let me know the size of the tumor, the time interval in-between imaging, and whether you have a pheo syndrome (with mutations) or not. I thank you in advance.
Dr. Pheo
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My mother had her surgery to remove the pheo 4 days ago and because it was more than 3 months since the CT scan she was rescanned just before. They said there was no change in size (all I've been told is '5cm') but that they do this rescanning after one operation where they were surprised at the change in size of the pheo.
ReplyDeleteThe operation (laparascopic) was described as entirely uneventful and so was her time in High Dependency (24 hrs). Since she has been on the ward she has had an episode of pulse rate going up to 120, with irregular heartbeat, and this is now being treated plus she is back on an oxygen mask.
I'm not sure whether this is her body readjusting after the pheo came out?
Tachycardia (fast heart beat) after surgery is not uncommon and the causes are many. The most common reaction after pheo resection is hypotension (low blood pressure) and secondary tachycardia.
ReplyDeleteIf her blood pressure is normal, probably the tachycardia is not related to the "adjustment" to absence of pheo.
Dr. Pheo
I have the SDHB mutation, and my primary tumor was attached to my bladder. The mass was initially found when I was about 23 years old via CT scan, and measured at 4 cm. Although I was having episodic symptoms since I was 14, I went misdiagnosed (I was told it was a "pseudo-tumor" and to not worry since it wasn't growing) until my 3rd pregnancy. The tumor stayed at 4 cm for approx. 15 years, imaged at various times by CT and sonogram, and didn't start growing until the last pregnancy at 37 years old, when my BP was out of control at 16 weeks. 2 years later, I had a tumor at C2 that collapsed the vertabrae, and have had a dozen surgeries since to remove tumors and lymph nodes that metastisized. I currently have tumors in my thoracic spine, lungs, and neck. Hope this helps.
ReplyDeleteDear Pam,
ReplyDeleteThank you for sharing this info.
Dr. Pheo
Symptoms which I attributed to pheo started at the age of 19. Had a contrast CT at the age of 20 (in early 1980's) that didn't show anything... and like many pheo patients went undiagnosed for years. 8 years later had a 7.5cm pheo on an unenhanced CT.
ReplyDeleteIan's multiple pheos in his lungs are growing at a rate of 2mm every 6 months, currently approx 16mm from what I can work out. Hope this helps.
ReplyDeleteHe has sporadic disease, all the genetic tests were negative.
Thank you both, DJPheo and Jane.
ReplyDeleteDr. Pheo
9/24/07 1.1x1.3cm
ReplyDelete3/10/08 1.9x1.4cm
8/18/08 2.2x2.6cm
1/08/09 2.9x3.2
I also have multiple myeloma
Typing this from the report
History: Right Adrenal Gland lesion on outside hospital CT, History of plasmacytoma
Technique: 10 mCi-123 MIBG was administered IV. Planar images of the whole body and SPECT were obtained.
Correlation is made from an outside hospital CT Scan of 1/08/2009
Findings: There is a relativity round MIBG-avid lesion in the region of the right adrenal gland, correlated with the same lesion seen on CT scan 1/08/2008. No other abnormal foci of uptake are identified..Normal physiological uptake is seen within the liver.
Impression: Focus of uptake within the right adrenal gland as described, MIBG is generally a very specfic tracer for tumors of the neural crest. (Pheochromocytomas, Paragangliomas, PNET,neuroblastomas) In this age group pheochromocytoma is the most likey if consistent with the patients laboratory values. Other adrenal tumors are far less likely. In particular we could not find any report in the literature for MIBG avid adrenal plasmacytoma, which is a very rare entity..... I have been scheduled for surgery July 27th and I am not comfortable with this at all I am having a problem with the Dibenzyline too the endo is basing the pheo decision on my MIBG scan.. My 24 hour urine was normetanephrine was 2232 my methanephrine 837 and I think I did it wrong as far as what not to eat I didn't use caffeine I have read all your articles and I think I am right in wanting more tests but I will be told did you go to medical school etc. I fought the adrenal gland biopsy they wanted to do the endo last year and hes the one taking care of me now..
Dear Jane,
ReplyDeleteThere are two issues in your case. First, whether this tumor is pheo. Second, whether the tumor needs to be resected. The second issue is actually more important.
To address the first question, you can measure plasma metanephrines to make sure it is pheo or not. To address the more important second question, more need to be considered. I will check the Hounsfield units of the mass on the CT and see whether it is enhancing upon contrast administration.
The growth speed is little too fast for a benign adenoma. I tend to agree that it should be resected, regardless of whether it is a pheo or not. Lymphoma is another possibility, which also needs to be resected for clear diagnosis. If the decision is made to resect but the diagnosis of pheo is not very clear, I will treat it as if it is a pheo to be on the safer side. In other words, you will need preoperative preparation.
Can you tell me your age? Feel free to ask questions.
Dr. Pheo
I am 59 will be 60 on Feb 10 2010.. I have been on Dibenzyline for 2 weeks tomorrow 40mg total I go for pre surgery screening tomorrow my surgery is set for July 27th and I can tell you I am petrified. I have felt horrible on the dibenzyline almost debilitating and also my bp still is all over the place my pulse ranges from 90 to 109 I go back to my endo Wednesday for the last time.. I am going to ask him point blank if hes ever cared for a pheo patient I don't think he has..I am running out of time here how steady should your Bp stay while on this med? also one cardiologist gave me labetalol and said I will be given the diben too I have not had the labetalol my endo said its not always necessary he told me to increase my salt intake too and fluids I think I should have kept my appt with NYC The adrenal center but the traveling back and forth over 200 miles each way a couple of times a week.. Please any info would be helpful I will sign my name Janie as there is another Jane
ReplyDeleteDear Janie,
ReplyDeleteAfter you are treated with dibenzyline, it is reasonable to start labetalol to control the fast heart rate. It is also correct to increase your salt and fluid intake after you are treated with dibenzyline.
Dr. Pheo
I have conflicting Chromogranin A trends from two different laboratories and I don't know whether I have a fast growing paraganglioma or just a laboratory quality control problem. The assays were ordered by two different doctors at two different research hospitals. Recent CT and MIBG scans have found nothing. I understand that there is no universal calibration standard for chromogranin A assays and that different assays have different cross-reactivity with the chromogranin A fragments, but should I have wildly different trends? My chromogranin A has been assayed by both Quest Diagnostics and Mayo Clinic. The Mayo Clinic assays have a flat trend over the last three months. The only chromogranin A assay I had done prior to the removal of a pulmonary metastasis of my retroperitoneal paraganglioma about 650 days ago was not elevated. That assay was the second lowest value of six assays by Mayo Clinic over 800 days. The six Quest assays were over about the last 450 days have the last 3 assays elevated as much as almost 4 times the top of the reference range. I compared the two labs data by normalizing for the upper limit of the respective reference ranges. The are in excellent agreement between for 3 assays over a 200 days range even showing the same slight increase followed by a leveling off. Since then there is a huge divergence with the Mayo data continuing below the upper limit of the reference range and the Quest values skyrocketing to 3.7 times normal, before dropping back to 2.8 times normal. Is there a way to determine if the Quest assay has picked up a real problem that is not detected by Mayo's testing procedure? My doctor with more experience with paragangliomas says he doesn't trust the Mayo assay and always uses Quest.
ReplyDeleteDear Eve,
ReplyDeleteQuest chromogranin A assay has been excellent based on my own experience. I have not used Mayo lab for chromogranin A assay.
I would suggest measuring other markers such as neuron-specific enolase (NSE) and pancreastatin. These markers can at least show whether there is biochemical evidence of recurrence.
Dr. Pheo
I checked the Quest and Mayo Clinic lists of tests and didn't seen pancreastatin listed. Is it the same as the somatostatin test?
ReplyDeleteIt is not sandostatin. It is offered by a few labs. Just ask your doctor to contact his/her own hospital's lab.
ReplyDeleteDr. Pheo
Re growth rate
ReplyDeleteLatest scan (Sept 09) showed 2 new nodules in lungs and slight increase in the others (approx 2mm)since June 09 scan
NB. this is a non genetic pheochromocytoma.
Sutent (Sunitinib) 50mg daily started 29th Sept 2009
Not sure if this is the right place to post (please edit/ move as required) but - since starting Sutent Ian has been asymptomatic and all hormone levels have significantly decreased within 2 weeks of starting the drug. Noradrenalin dropped from 35000 to 24000! Side effects tolerable - fatigue, joint/muscle pain, flu like symptoms, loss of appetite & taste, hair thinned & whitened. BP & pulse now normal (120/70 & p70) whereas before 165/110 & p130 normal for Ian. Now awaiting CT/MRI to determine tumour size, at last count there were approx 16 spread across both lungs.
ReplyDeleteIan managed to walk around the block today - first time since April/May 2009 - endocrinologist delighted & amazed :)
That's great! I am very happy for Ian and you. Keep us posted.
ReplyDeleteDr. Pheo
Dr pheo I have slightly elevated BP and tachycardia runs around 100+.. I currently taking 60 mg proponolol, effexor and lorazepam... I did a 24 hour urine study and all levels within range except for one my doctor has scared me stating it is probably a Pheo... I am havingMRI tomorrow..is there a chance that my medications and diet affected the test..
ReplyDeleteDear Lisa,
DeleteThe medications may influence the results to a small degree. Diet usually does not interfere with pheo testing.
Dr. Pheo