Carcinoid or Mastocytosis

[Nanlyn]

My story begins in Jan of 2005 with unexplained flushing.  I am 45 yr old female.  Hormone tests came back ok - flushing is not hot flashes related to menopause.  
Doctor tested for carcinoid with the 24hr urine and came back normal.  Blood work showed high histamine so they started testing for Mastocytosis.  All bloodwork was normal.  Did a lung, abdominal & pelvic CT scan.  Only one thing showed as a fibroid on ovary but was ok.  I noticed brown spotting on my arm and neck which dermatologist thought was just sun damage but did a skin biopsy anyway.  Turned out positive for Mastocytosis (TMEP).  Did bone marrow biopsy to check for systemic involvement - all Normal.  So hematologist decides to send me to Sloan in NY for second opinion just to be sure.  Went to Sloan to a hematologist there who is familiar with Mastocytosis.  He is not completely convinced about the Mastocytosis diagnosis since the type, TMEP is often confused with Carcinoid.  So now, he is suggesting a Octreotide Scan to finally once and for all rule out Carcinoid and then know we will just live with the Mastocytosis.  Please tell me what you think and what about this scan?  I am terribly claustrophobic and can't sit still for too long (which is what you need to do with this test).  Would Xanax help me with that?

[Kevin Pho, MD]

The octreotide scan has a complementary role with other imaging studies (such as CT, MRI or ultrasound).  By itself, it may not be accurate anough to make the diagnosis - however if the CT scans have been non-revealing thus far, it can be used for further testing.  Thus, I think it is a reasonable test to consider if carcinoid is still in question.

Patient information on the scan can be found here:
http://www.healthsystem.virginia.edu/internet/radiology/nuclearradiology/octreoscan.cfm#To

If anxiety is a concern, you can discuss with your personal physician whether a short-acting sedative like Xanax may be helpful.

Followup with your personal physician is essential.

This answer is not intended as and does not substitute for medical advice - the information presented is for patient education only. Please see your personal physician for further evaluation of your individual case.

Kevin, M.D.
http://www.straightfromthedoc.com

[Sarah Connors]

• Besides flushing have you been having any other symptoms e.g. Cardiovascular, Gastrointestinal, Musculoskeletal etc. since you have just mentioned flushing & skin tan in your post. Could you be a bit more elaborate?
• Did the urinary sample also show high levels of histamines?
• Surprises me that only on a cuatneous biopsy a diagnosis of not just Mastocytosis but TMEP was made, that also on your insistence.
• Don’t want to sound rude but the histopath. doc must really be highly competent to rule out the other forms of Mastocytosis.
• Did the bone marrow biopsy reconfirm the diagnosis?
• The scan report seems ok.
• Taking Xanax for the test would do no harm.

All of the above are my personal thoughts, views, opinions.

Take care & Best of luck.

[Nanlyn]

Yes.  My urine test did show high histamine level.  The bone marrow biopsy showed no evidence of systemic involvement of the Mastocytosis. The only positive was the skin biopsy.  Went to second opinion hematologist at Sloan.  He is currently re-reading all of the slides and in the meantime wants that scan done to completely rule out carcinoid (which can be confused with Mastocytosis when that has been diagnosed as TMEP).  Would love to hear from you further on this if you have experience with either of these diseases.  I do not have any other symptoms except for the flushing.  Feel free to email me (easier to converse that way) ***@****.  Thanks.

[Sarah Connors]

• Did the urine sample show raised levels of 5-HIAA (Serotonin & its break down products)?
• Would you describe the dermatological signs as raised, itchy, and discolored, because sun tan signs are different e.g. polymorphic light eruption?
• If not, than I feel that you do have the localized form of Mastocytosis, at the moment & would probably rule out Carciniod.
• As regards to which subtype of Mastocytosis you have, would have to look at the slide to give my personal opinion.
• My personal advice to you would be that after the octreoide test & ruling out of Carciniod altogether, keep a watch that the localized Mastocytosis doesn’t progress to the systemic / malignant form.

         Take care & best of luck.

[Nanlyn]

Hi Sarah.  I am wondering if you are a doctor or nurse or some medical professional.  That would be great if you are and are familiar with both of these diseases.  Is that the case?

Anyway, a little history as far as the testing goes:
The 5-HIAA test levels (done 3 different times) 3.8, 2.8 & 5.3
My very first original urine had histamine of 102.3.  The blood histamine was actually normal.

As far as the skin biopsy, the report says the following:
Number of mast cells is aprroximately 12-15/HPF most likely representing a subtle manifestation of Mastocytosis such as TMEP. Multiple sections were examined.

The appearance of my skin is just smooth brown patches on my forearm - they are a bunch of little spots in one big patch from my wrist to half way up to my elbow.  They are not red and bumpy.  

I would love to hear more from you.  And can you tell me a little more about that scan since I am so anxious about it?
Thank You.

[Sarah Connors]

• Please correct me if I am wrong. Am assuming that the values posted are in mg/dl & not mmol/dl?
• Blood histamine levels have never been raised?
• Could you clarify “Number of mast cells is approximately 12-15/HPF?”  Assuming the Number of ABNORMAL mast cells 12-15/HPF, as normal mast cells are present in the skin too.
• What abnormalities histopathologically could the doc see in the abnormal mast cells e.g. a high N/C ratio, any other worth mentioning?
• On the basis of 12-15/HPF in various sections he diagnosed it as pauci i.e. meaning a few & gave a diagnosis of TMEP subtype. What numbers/HPF approx in his/her books would be needed to give a diagnosis of the other subtypes?
• Do the skin lesions itch at times?
• Is the bunch of little spots on the brown patch raised?
• How frequently do you get the episodes of flushing & how long do they last? Do they terminate spontaneously?
• Regarding the scan it is a Nuclear Med, Radiological test. Pretty accurate to diagnose the slightest & the smallest mass of neuroendocrine tumor IF present & has been missed by the CT/ MRI. It shall help in ruling out Carciniod all together. Relax take it easy, its no big deal.
• Any other ailment besides this that you have or any medications that you take?

Don’t mind my asking so many questions because the more details I have the more helpful I could be, that’s what I think & is my philosophy.

At times my replies could be late due to the nature of my job.

Take care & good luck once again.