An Update from Barbara

I cede the platform to my better half, as I just didn't have the energy to put a rational post together after a pretty long day at Beth Israel.  So hereafter are Barbie's words:

Thank you all for your kind thoughts and help through this incredible past two weeks.

Many of you know that we looked forward to a meeting today with two surgeons (upper GI cancer folks) who are currently running with the ball on this issue.  We hoped to come away from the meeting with a clear picture of what we face, and the best path forward.  But as with every test and procedure involved in trying to solve the mystery of exactly what we are dealing with,  what actually happens is not what anyone predicted; it is like a seemingly small shrub, which, when investigated further, has a huge and complex root system. Every root has many branches going from it, any one of which may represent the actual situation. We are still determining which root system to follow.

What is known: Greg has an ampullary adenoma, a tumor located on a flap at the end of the common bile duct (called the Ampulla of Vatar), which drains the liver, the gall bladder, and the pancreas.  While the biopsies taken so far do not indicate a malignancy, there is a very high false negative with these tumors, where 25-30% of “benign” tumors are actually already cancerous (in which case, it is classified as ampullary carcinoma).  Adenomas in this area always become malignant and are aggressive, though not as fast growing as those associated with pancreatic tissue. Greg’s tumor is too large for laparoscopic removal, but it does seem to be relatively superficial, and probably will be a good candidate for resection (surgical removal), as it appears not to be highly vascular.  That being said, the recommended procedure is a 6-hour operation that removes and reconstructs parts of several organs in that area.  But a small unexplained spot appeared on the liver, visible on MRI but not large enough to see clearly on a CAT scan or ultrasound.  Were it not for this unexplained lesion, they would be scheduling surgery directly at this point.

If this liver spot is cancerous, then the cancer has metastasized, and we are dealing with a radically different situation.  If so, they will not subject Greg to a large and complicated surgery, but rather would move directly to chemo therapy.  The immediate task is to define the nature of that spot.  Since it is in such a difficult location and is very small, they will first try another MRI, scheduled for next Wednesday, to see if the spot has shrunk, or stayed the same. There is a chance that the spot was caused by the infection resulting from the blocked bile duct. In that case, it should recede and shrink — that is our greatest hope at the moment.  If it has not gotten smaller, they may try a biopsy either as a separate operation, or as the opening act to the big operation they propose, called a Whipple.  Apparently they can have a pathologist right there in the operating room who could read the biopsy on the spot — and either they would go ahead if the cells are normal, or they would just stop the procedure at that time.

If the liver issue is resolved, then there is the huge and complex Whipple procedure itself.  Google it.  It is no fun.  But, if successful, it can result in a “cure”, unlike Multiple Myeloma, where there is no cure, but where the disease is “managed” with a quiver full of new drugs. 

Another wrinkle.  Greg’s mother’s family has a lot of cancer.  There is a small correlation between Multiple Myeloma and ampullary cancers, caused by a genetic mutation.  The offending genes have been identified, much as the presence of a BRACA gene is a strong predator of breast cancer.  Currently they are running genetic tests on Greg’s blood and other material he has left behind in various labs.  If he has that mutation, then our kids and their kids will need to be screened so they can be watched appropriately throughout their lives.  If he does not have that gene, then it is just a case of bad luck/coincidence.

This was going to be a quick update, just to let you all know where we stand.  But it is difficult to keep things short, as the situation is intrinsically complex.  We do not want to be learning about GI cancers, but we must.  Perhaps Wednesday’s MRI, if it shows a shrunken liver spot, can remove that unknown and the surgery will be scheduled.  If that MRI is inconclusive, we will have to get the cellular information in another way.

Greg’s situation is incredibly rare.  Cancer of the ampulla accounts for just 0.2% of all cancers — perhaps 7,000 in the USA annually (vs. 150,000 colon cancers, 250,000 breast, etc.)   By luck, thanks to daughter-in-law Bridget’s being a doctor at Beth Israel in Boston, we happen to be in the hospital that performs many of these operations — it stands either #2 or #3 in number of pancreatic surgeries performed annually in the country.  The lead surgeon does about 200 ampullary surgeries a year — a very high number, so that is good.  Interestingly, the doctor said Boston is unique — Harvard Medical School doctors are spread throughout the city’s many fine medical institutions, so that certain hospitals become associated with different specialties — lung with one hospital, blood cancers with another, etc.  We landed in the right place for upper GI cancers, and are very impressed with the doctors.

Once again, thank you for all your good wishes.  I wish I could convey good news, a definite schedule, a clear diagnosis and good prognosis.  None of those are possible at the moment. 

If you do not receive Greg’s blog, you may sign up for it, and he will provide a pretty good background on his encounter with some of life’s nastier diseases.  On the positive side, the doctors say he is very fortunate that this tumor caused a blockage, and therefore was caught quite early.  Most cancers in this area are asymptomatic and therefore are discovered too late, or by chance (when someone has an accident and the state of their upper GI is revealed for another purpose.)  Also, Greg has always been very active and is in very good shape. But, the strong drugs he takes for Multiple Myeloma must be processed by the liver, and they have had an effect…..

We’ll update you as things progress and we discover more.

Barbara