VOTRIENT

Dear Jessica,

This is the first time I've been on the American Cancer support site.  I've had a rare cancer for the last five years called Pediatric-Like Gist or Wildtype Gist.  There are others on our support site that have tried all the "Gleevec" line of medications.  You might sign up and post there or the paraganglioma website that has great information from Erin MacBean and others dealing with tumors for many many years.  My father died of paraganglioma tumors when they were called Chemodectomas in 1974 after a 15 year battle with various tumors returning . . . I've been researching "my" cancer that I inherited from him for the last 5 years.  There is a DNA mutation that because is present means my body will produce four different types of tumors:  paragangliomas (and pheos), GIST tumors (which is what I've had so far with new mets in Liver, Stomach and Renal), Renal Tumors and Pulmonary Chrondomas (these are fairly benign).  

I just returned from the National Cancer Institute in Bethesda Maryland where I was flown out for a week of clinical tests.  Dr. Pacak and his team headed by Karen Adams is doing research on paragangliomas.  If you were interested in pursuing a second opinion then you might consider contacting Dr. Pacak at NIH.  He runs a testing clinic (I had two MRIs and two PET scans while I was there for a week).  If the paragangliomas can be surgically removed they will do it at NIH if you want.  The cost for tests and surgery when at NIH is free.  You might look into it.  There were some other patients while I was on campus for a week who were going through some experimental radiation treatments for rare cancers and target bone marrow transplants with the patients own bone marrow.

My cancer is called "Carney-Stratakis Dyad" because I have had GIST tumors to date but have the potential to grow paragangliomas as well. My father's only sister also died of paragangliomas.  If you haven't had a DNA test you might look for the SDHx mutation.  Being tested was important to me so I could do my best to be pro-active which is why I took a week to be a "guinea pig" at NIH as well as confirm where I do have active tumors.

Paratroopers:   https://www.facebook.com/groups/pheoparasupportgroup

Pediatric Gist Support:  http://www.gistsupport.org/pediatric-gist-carney-triad.php

I hope this information can help.  I could send you the names of patients on Votrient as well if you'd like so you could personally ask their experience.

Cathy Sherman Freeman

GIST tumor stomach age 50, 2/3rds stomach removed, Liver mets age 53, 2/3rds of liver removed (it grew back within four months), age 55 new mets to liver, stomach near renal.  SDHB germline mutation, Wildtype GIST or Pediatric-like GIST, Carney-Stratakis Dyad.