Pheochromocytoma

[DreamLover]

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34yo Male, BMI 36, no other significant PM with a Right sided supra renal (adrenal) norepi secreting tumor. Alpha blocked with non selective alpha blocker (phenoxybenzamine) X 4 weeks.

No Changes on EKG for >1 week.
+ Orthostasis
SBP 116-156
Blood Glucose 85


How would you do this? Preop A-line, Midaz, heavy fentanyl induction, stick of esmolol, NTG, Phentolamine, Phenylephrine? CVL and fluids as needed?

Or would you be more on the conservative side...Esmolol, Phentolamine, Phenylephrine Infusions in line...TEE? I don't know of anyone still routinely floating PACs for these but would you?

Just asking as I had a pt similar to this recently...and as I have learned since starting my new job...everyone does everything differently

 

[pgg]

Haven't seen a pheo since I was a CA2 (not that long ago 😉) but

34yo Male, BMI 36, no other significant PM with a Right sided supra renal (adrenal) norepi secreting tumor. Alpha blocked with non selective alpha blocker (phenoxybenzamine) X 4 weeks.

No Changes on EKG for >1 week.
+ Orthostasis

So he's appropriately therapeutic on his alpha blocker ... my old notes say reasonable treatment endpoints are BP < 165/90, < 1 PVC / 5 min, no ST/T wave changes, and orthostatic hypotension not worse than 80/45 standing ... or development of side effects (reflex tachycardia, nasal congestion, nausea, or abdominal pain).

How would you do this? Preop A-line, Midaz, heavy fentanyl induction, stick of esmolol, NTG, Phentolamine, Phenylephrine? CVL and fluids as needed?

I don't know that you need to go heavy on the opiates. I thought manipulation of the tumor itself was the biggest risk for huge spikes in circulating catecholamines intraop, not pain. Enough anesthesia should be enough. Would have vasopressors inline and ready, including norepi, postop too.

PAC only if there was evidence of catecholamine myocarditis, which I thought typically improved if not resolved with appropriate weeks/months of preop alpha blocker treatment.

 

[DreamLover]

Would anyone place an epidural? I didn't but I've read about them.

Does Lap Vs Open change anything for anyone?

 

[DreamLover]

Haven't seen a pheo since I was a CA2 (not that long ago 😉) but

ha...same here...before last week anyway

 

[sevoflurane]

These patients are often volume depleted. Make sure you have a good full tank before starting the case. As mentioned, get ready to treat htn as you manipulate the tumor and get ready to treat hypotension once it's out. Many would get an echo pre-op. A surge of catecholamines can resemble Takotsubo Myocarditis and if you have issues an intraop TEE is easy and safe. I would not float a swan, but would go with good access + Aline. I've heard of magnesium infusions before the tumor is out. D/C afterward. Epidural is ok... but I would hesitate if urine/plasma catecholamines are excessively high even in pheo terms. Good chance to review MEN syndromes.

Sounds like fun! Do tell how it went.

 

[epidural man]

34yo Male, BMI 36, no other significant PM with a Right sided supra renal (adrenal) norepi secreting tumor. Alpha blocked with non selective alpha blocker (phenoxybenzamine) X 4 weeks.

No Changes on EKG for >1 week.
+ Orthostasis
SBP 116-156
Blood Glucose 85


How would you do this? Preop A-line, Midaz, heavy fentanyl induction, stick of esmolol, NTG, Phentolamine, Phenylephrine? CVL and fluids as needed?

Or would you be more on the conservative side...Esmolol, Phentolamine, Phenylephrine Infusions in line...TEE? I don't know of anyone still routinely floating PACs for these but would you?

Just asking as I had a pt similar to this recently...and as I have learned since starting my new job...everyone does everything differently

Nope. don't believe it. Pheo's are a myth. They stopped shortly after small pox disappeared.

 

[epidural man]

Nope. don't believe it. Pheo's are a myth. They stopped shortly after small pox disappeared.

And if you don't believe me that you can just ignore cancer, take a listen to this inspirational tale of Peter.

http://www.theonion.com/video/courageous-man-refuses-to-believe-he-has-cancer,14312/

 

[btbam]

.

 

[Idiopathic]

art line and two big IVs, no PAC, no CVL, no TEE (unless comorbid conditions suggest use would be beneficial). i like remifentanil in the OR for pain and to help with pressure, but if you dont have it then cardene/esmolol work well. propofol boluses are helpful as well in a pinch. consider phentolamine but ive never had to actually use it since most people are so alpha blocked. you may have to speed them up and volumevolumevolume after the tumor is out since they will be somewhat refractory to catecholamines.

the last two i did as an attending were prone. that was fun. i had to swan one guy because of his 30% EF and valvular disease. i wouldnt have done it if we were supine, but knew i couldnt get an echo in him prone.

 

[Stank811]

Just did a similar case three weeks ago.

63 yo F PMHx significant for MI 3/2011 resulting in CVICU stay with aortic ballon pump secondary to initial EF of 15%. Pt was stented with DES and most recent TTE pt EF was 55% with no significant valvular defects. After initial hospitalization pt required three more DES and was found to have failed so was on prasugrel and last stent was placed 09/2011. During all of this pt was noted to have episodes of severe HTN and on workup was noted to have NE secreting periaortic paraganglioma and L adrenal mass.

It was decided pt emergently needed resection of tumor. We instructed pt to continue both ASA and prasugrel. Pt BP was controlled preop with prazosin for several weeks and was then admitted night before for IV hydration.

Pt had two IV's, preop artline placed, CVL (no swan), we controlled BP with Nitroprusside gtt, nicardipine boluses, and phenylepherine gtt for lows. Esmolol gtt more for HR control the BP control. Pt BP did great with induction with 250 fentanyl, 150 propofol, and ROC. Pt did great, she had the expected HTN with manipulation of tumor and lows after removal but overall did well. Interestingly pt had ST depression in inferior leads with HR >85 and we had a hard time keeping HR below 70 with esmolol only and my staff thought it may be due to pt having NE secreting tumor resulting in increased ration of B2/B1 receptors similar to pt with CHF so we gave a little labetalol bolus and it worked well. Blood loss was only 500cc even though she was on dual antiplatelet therapy which surprised me. Overall was a great case.

 

[Robert Loblaw]

art line and two big IVs, no PAC, no CVL, no TEE (unless comorbid conditions suggest use would be beneficial). i like remifentanil in the OR for pain and to help with pressure, but if you dont have it then cardene/esmolol work well. propofol boluses are helpful as well in a pinch. consider phentolamine but ive never had to actually use it since most people are so alpha blocked. you may have to speed them up and volumevolumevolume after the tumor is out since they will be somewhat refractory to catecholamines.

the last two i did as an attending were prone. that was fun. i had to swan one guy because of his 30% EF and valvular disease. i wouldnt have done it if we were supine, but knew i couldnt get an echo in him prone.

you wouldn't want central access for rapid central administration of drugs? i have typically put central lines in simply for this reason, for either rapid intraoperative management or continued post-operative infusion of catechols...my two cents.

 

[Robert Loblaw]

I don't know that you need to go heavy on the opiates. I thought manipulation of the tumor itself was the biggest risk for huge spikes in circulating catecholamines intraop, not pain.

is not laryngoscopy considered a strong stimulus for massive catechol release? doesn't everyone have a story about how their friend's friend's friend's anesthesiologist diagnosed their friend's friend's friend's pheo because their BP went to 315/180 on induction/laryngoscopy?

 

[pgg]

is not laryngoscopy considered a strong stimulus for massive catechol release? doesn't everyone have a story about how their friend's friend's friend's anesthesiologist diagnosed their friend's friend's friend's pheo because their BP went to 315/180 on induction/laryngoscopy?

Sure, but I wrote "enough anesthesia should be enough" ... when I read "heavy fentanyl induction" in the OP, I was picturing something akin to the 500-750+ mcg fentanyl inductions of ye olde cardiac induction days. I might give 150 or 200 with induction and would expect that to be enough for a routine easy airway DL x1.

 

[W222]

55 yo M w/hx of cocaine/ETOH abuse, smoker, non-obstructive CAD and DM who presented to the ED one month prior to surgery c/o chest pain and was found to have a mass at about the T10 level abutting the SVC. His BP was through the roof and difficult to control during that admission so prompted them to look for other causes, found to have a pheo. Patient goes out on alpha blocker with plan to f/u in 6 weeks for surgery. Also, he is put on beta blocker a week before surgery.

Guy shows up for his surgery, admits to doing coke a week before. Surgeon agrees that if he tests positive for anything then we cancel. He swears the coke was a while back and his Utox is negative. We proceed.

Awake A-line and large bore IV, standard induction with a bolus of nitro/esmolol just prior to DL. We place a central line in case we need to start pressors post-op. Infusions: nitroprusside, esmolol, phentolamine, and phenylephrine. Everything went smoothly until the surgeon manipulated the tumor, BP at one point read >300/150. We were convinced the guy would stroke out or have an MI. After several minutes of insanely high BPs that would not respond to massive doses of phentolamine/nitroprusside the tumor was out.

Post-op, the guy woke up fine, Trp jumped slightly and he had a rhabdo that responded to hydration. Overall though he did well and was discharged on post-op day 5.

 

[btbam]

Awake A-line and large bore IV, standard induction with a bolus of nitro/esmolol just prior to DL. We place a central line in case we need to start pressors post-op. Infusions: nitroprusside, esmolol, phentolamine, and phenylephrine. Everything went smoothly until the surgeon manipulated the tumor, BP at one point read >300/150. We were convinced the guy would stroke out or have an MI. After several minutes of insanely high BPs that would not respond to massive doses of phentolamine/nitroprusside the tumor was out.

Post-op, the guy woke up fine, Trp jumped slightly and he had a rhabdo that responded to hydration. Overall though he did well and was discharged on post-op day 5.

😱

 

[RT2MD]

Just did a similar case three weeks ago.

63 yo F PMHx significant for MI 3/2011 resulting in CVICU stay with aortic ballon pump secondary to initial EF of 15%. Pt was stented with DES and most recent TTE pt EF was 55% with no significant valvular defects. After initial hospitalization pt required three more DES and was found to have failed so was on prasugrel and last stent was placed 09/2011. During all of this pt was noted to have episodes of severe HTN and on workup was noted to have NE secreting periaortic paraganglioma and L adrenal mass.

It was decided pt emergently needed resection of tumor. We instructed pt to continue both ASA and prasugrel. Pt BP was controlled preop with prazosin for several weeks and was then admitted night before for IV hydration.

Pt had two IV's, preop artline placed, CVL (no swan), we controlled BP with Nitroprusside gtt, nicardipine boluses, and phenylepherine gtt for lows. Esmolol gtt more for HR control the BP control. Pt BP did great with induction with 250 fentanyl, 150 propofol, and ROC. Pt did great, she had the expected HTN with manipulation of tumor and lows after removal but overall did well. Interestingly pt had ST depression in inferior leads with HR >85 and we had a hard time keeping HR below 70 with esmolol only and my staff thought it may be due to pt having NE secreting tumor resulting in increased ration of B2/B1 receptors similar to pt with CHF so we gave a little labetalol bolus and it worked well. Blood loss was only 500cc even though she was on dual antiplatelet therapy which surprised me. Overall was a great case.

Second year medical student here with a question.

It was my understanding that phenoxybenzamine is the usual agent for alpha blockade preop in a suspected pheochromocytoma. I didn't know the difference between phenoxybenzamine and prazosin until this post, which caused me to look it up. I get that phenoxybenzamine is (mostly) nonselective between alpha 1 and alpha 2, while prazosin is highly selective for alpha 1. I haven't had pharm yet, so I'm sorry if this question is a dumb one:

How does blocking alpha 2 help in the case of a pheo? I read that phenoxybenzamine inhibits reuptake of released norepinephrine by presynaptic adrenergic nerve terminals. I'm guessing that's the alpha 2 blockade? I would think that you would want to INCREASE reuptake... try and get some of the norepi out circulation. Is this flawed thinking?

Thanks for the discussion!

 

[narcusprince]

Second year medical student here with a question.

It was my understanding that phenoxybenzamine is the usual agent for alpha blockade preop in a suspected pheochromocytoma. I didn't know the difference between phenoxybenzamine and prazosin until this post, which caused me to look it up. I get that phenoxybenzamine is (mostly) nonselective between alpha 1 and alpha 2, while prazosin is highly selective for alpha 1. I haven't had pharm yet, so I'm sorry if this question is a dumb one:

How does blocking alpha 2 help in the case of a pheo? I read that phenoxybenzamine inhibits reuptake of released norepinephrine by presynaptic adrenergic nerve terminals. I'm guessing that's the alpha 2 blockade? I would think that you would want to INCREASE reuptake... try and get some of the norepi out circulation. Is this flawed thinking?

Thanks for the discussion!

You will probably get this in pharm. 90% of norepinephrine thats used is reuptaked in the presynaptic terminal. When you inhibit their reuptake you essentially decrease the supply of NE. Allowing some of the NE to be metabolized by COMT and MAO. Hence a catacholamine depleted state. Off the top of my head.

 

[RT2MD]

You will probably get this in pharm. 90% of norepinephrine thats used is reuptaked in the presynaptic terminal. When you inhibit their reuptake you essentially decrease the supply of NE. Allowing some of the NE to be metabolized by COMT and MAO. Hence a catacholamine depleted state. Off the top of my head.

Ahhh. I see. That kinda makes my question moot, then. 😎

Thanks for the reply. 👍

 

[Idiopathic]

you wouldn't want central access for rapid central administration of drugs? i have typically put central lines in simply for this reason, for either rapid intraoperative management or continued post-operative infusion of catechols...my two cents.

yeah, i think that i would like one but it wouldnt necessarily be a deal breaker.

 

[epidural man]

you wouldn't want central access for rapid central administration of drugs? i have typically put central lines in simply for this reason, for either rapid intraoperative management or continued post-operative infusion of catechols...my two cents.

Lots of reports of patients getting phenylephrine infusions during a long case, only to take the drapes down and seeing a black hand (or hand that will develop into a black one in the ensuing hours).

Tis no joke.

 

[DreamLover]

Lots of reports of patients getting phenylephrine infusions during a long case, only to take the drapes down and seeing a black hand (or hand that will develop into a black one in the ensuing hours).

Tis no joke.

Well, I did place a R IJ CVL for essentially the same reasons...possible need for vasopressor infusions. Plus, even though CVP is not an ideal measure...I liked the idea of noting the trend during the case and how it might change after ligation of the adrenal vein etc...to me, this is a case all about volume status.

I placed the standard pre-induction A-line, had premixed bags of phenylephrine, esmolol and SNP on my cart but not hooked in line. I also had syringes of Phenylephrine, NTG and Esmolol.

Luckily, he was a young healthy guy whose only issues were that he happened to have notice palpitations and increasing fits of rage where he felt like his "heart was going to explode." He was pretty stable through induction and CVL placement. MAPs fell from 90s to low 60s on induction --> responded to fluid. Only a few pressure swings with tumor manipulation...I do admit at one point right before they ligated the vein, his SBP was 230 while I had the sevo cranked and pushing a half stick of NTG (100mcg/cc)...then nice and controlled and very stable afterward without any of the feared post tumor hypotension. I just opened some colloids and he did great.

Funny thing was that the tumor was pretty big, and it was lap-so they had to get a bigger bag to fit the tumor (7X9cm) before they could pull it out...they had to keep extending their incision to be able to remove the bag and as the bag popped out of the belly, a large cyst on the tumor ruptured and spilled tons of norepi into the bag....thank God that cyst didn't rupture intraop!

 

[Idiopathic]

Lots of reports of patients getting phenylephrine infusions during a long case, only to take the drapes down and seeing a black hand (or hand that will develop into a black one in the ensuing hours).

Tis no joke.

are there really "lots of reports" of this?

 

[Dipipanone]

Anaesthesia & Intensive Care Medicine
Volume 12, Issue 10, October 2011, Pages 442-445
Endocrinology / Physiology


Endocrinology


Recognition and Management of Phaeochromocytoma

Rob Shawcross, John Moore



Rob Shawcross FRCA is a Specialist Trainee in Anaesthetics in the North West Region, UK. Conflicts of interest: none declared

John Moore MRCP FRCA is a Consultant in Anaesthetics and Intensive Care Medicine at Manchester Royal Infirmary, Central Manchester Foundation Trust, UK. Conflicts of interest: none declared
Available online 16 September 2011.


Abstract

Phaeochromocytoma is a rare catecholamine-secreting tumour with a high incidence of perioperative mortality if not managed appropriately. It benefits from an experienced structured medical, anaesthetic and surgical pathway. For anaesthetists successful and safe perioperative care requires knowledge of the pathophysiology of the condition combined with appropriate treatment strategies for preoperative preparation and management of acute release of catecholamines during periods of peri operative stress. Awareness of the perioperative presentation of phaeochromocytoma and associated signs and symptoms is important for all anaesthetists as the condition can present acutely during anaesthesia for other surgery. This has potentially a high mortality if not managed correctly.

 

[epidural man]

are there really "lots of reports" of this?

Ya know...I thought so, but I'm not seeing a lot come up on google scholar.

In my career, I have seen it happen twice, and my bro just told me about one that happened at his hospital.

Maybe it happens more often in the unit.

 

[Arch Guillotti]

Ya know...I thought so, but I'm not seeing a lot come up on google scholar.

In my career, I have seen it happen twice, and my bro just told me about one that happened at his hospital.

Maybe it happens more often in the unit.

I fear this sort of complication but I have never seen it in my relatively short career. I use phenylephrine infusions all the time thru peripherals.