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Does mass in the adrenal gland indicate VHL?

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Posted on Fri, 29 May 2015
Question: Ever since 2003, I have had major issues. My primary doc at the time said I was mental (bipolar,schizo, major depressive, etc was the diagnosis) and sent me to a mental doctor. I took all their cocktail of meds and everything made me worse. They wanted to do ECT, and I said no. That was 2003-2007, I was done. I stopped everything. I have lived in a hole, so to speak. I was placed on disability since 2003 for mental issues. In 2011, my family thought I had a seizure, I was sent to a neurologist. The neurologist ordered a MRI of brain and neck. There was "incidentally noted within the nasopharynx from the fossa of Rosenmuller, there is a large mass measuring 3.1cm (transverse) by 1.6 (AP). This is homogeneously hyperintense on T2, isointense on T1 and demonstrates no enhancement but is only partially imaged on this examination." I found a different primary care doctor and he ordered a CT of abdomen and pelvis. The scan showed a mass in each adrenal gland. The CT showed mass on the left adrenal gland measuring 2.2cm "this is of moderately low density." Question Adrenal Adenoma. On the right adrenal gland, there is a smaller mass measuring 1.1 cm, and this is of low density, question adenoma". My blood pressure readings are at and below normal, however, my pulse is 93 - 143 BPM, constant elevation with peaks and symptoms.(headaches, major sweating, and fits of aggression). My dad passed away with renal cell carcinoma which spread to lungs, lymph nodes and finally neck. My dad was one of few back then to test at Emory University, interleaukin and interferon. The gave him 5 years, my dad went 10. What should I do? The doctors I have been to seem to either down play the issue or are just clueless.

Could it be what my new PC suggested? A Pheo?
doctor
Answered by Dr. Shehzad Topiwala (34 minutes later)
Brief Answer:
VHL

Detailed Answer:
Sorry to learn about your medical condition.

There is a rare medical condition called VHL (von Hippel-Lindau) disease in which the two problems that you have alluded to here are present, sometimes long with other diseases.
The first is 'pheochromocytoma'. You have been tested for that in the 24 hour urine sample. It appears to be negative.
I encourage you to see an endocrinologist in person and consider getting retested preferably on a day when you have a 'spell'

RCC (Renal Cell Carcinoma) can be a part of the VHL syndrome.

I have noted that your CMP (liver/kidney blood tests and electrolytes/glucose are normal)

When I see someone like you in my practice I order the following tests in addition to a thorough physical examination:

TSH
Free T4
Midnight Salivary cortisol

The cortisol test is to screen for a condition called Cushings syndrome, in which here we want to make sure that the adrenal adenomas are not making excessive cortisol. The saliva test I have mentioned is recommended only if you have a conventional sleep-wake cycle ie you sleep at regular bedtime at night and wake in the morning hours.
If you have shift work or an unusual sleep-wake pattern this test may not be appropriate for you. In such instances, alternative forms of testing are available to screen for this condition. Examples are 24 hour urine cortisol (with creatinine)

Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Shehzad Topiwala (1 hour later)
I had a follow up with an endocrinologist, after the nephrologist pretty much said I was mental. I was pissed! I stated everything about my dad and my symptoms including the right flank pain. he didn't know where the pain was from. I will not wait like my dad did, he started peeing blood and was stage 4.
doctor
Answered by Dr. Shehzad Topiwala (1 hour later)
Brief Answer:
Follow up

Detailed Answer:
Despite your compelling episodes of headaches, sweating and blood pressure swings, it is notable that your 24 hour urine test for pheochromocytoma has not come back positive.

I suggest a fresh endocrine evaluation with consideration for repeat testing for

24 hour urine Fractionated Catecholamines and metanephrines

This would be particularly useful on the day you experience such a spell of symptoms.

Furthermore it requires a detailed overall assessment including medication history to ensure the test results are reliable.

Checking for Cushings syndrome is also important.

An academic center such as a university endocrinology department will help you rule out the possibility of VHL

Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
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Answered by
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Dr. Shehzad Topiwala

Endocrinologist

Practicing since :2001

Answered : 1663 Questions

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Does mass in the adrenal gland indicate VHL?

Brief Answer: VHL Detailed Answer: Sorry to learn about your medical condition. There is a rare medical condition called VHL (von Hippel-Lindau) disease in which the two problems that you have alluded to here are present, sometimes long with other diseases. The first is 'pheochromocytoma'. You have been tested for that in the 24 hour urine sample. It appears to be negative. I encourage you to see an endocrinologist in person and consider getting retested preferably on a day when you have a 'spell' RCC (Renal Cell Carcinoma) can be a part of the VHL syndrome. I have noted that your CMP (liver/kidney blood tests and electrolytes/glucose are normal) When I see someone like you in my practice I order the following tests in addition to a thorough physical examination: TSH Free T4 Midnight Salivary cortisol The cortisol test is to screen for a condition called Cushings syndrome, in which here we want to make sure that the adrenal adenomas are not making excessive cortisol. The saliva test I have mentioned is recommended only if you have a conventional sleep-wake cycle ie you sleep at regular bedtime at night and wake in the morning hours. If you have shift work or an unusual sleep-wake pattern this test may not be appropriate for you. In such instances, alternative forms of testing are available to screen for this condition. Examples are 24 hour urine cortisol (with creatinine)