Saturday, September 17, 2011

Report from ISP 2011 Part II

After chatting with the organizers, I realized that the primary reason to have the meeting in Disney Paris was financial—similar hotels in downtown Paris would cost twice as much. In addition, there is a grand chateau nearby that is undeniably French.

On 9/16, the lectures were mostly on progress in basic and clinical research. Regarding basic research, it seems rather clear that there are two “clusters” in pheo pathogenesis. The clusters concept is not entirely new (I wrote on the clusters before) but now the molecular details are worked out to a large extent. The scheme of pheo pathogenesis is a labyrinth of interconnected molecules. As I also do basic research myself, I am sometimes sarcastic about this kind of scheme. If everything affects everything else, how do we break the pathways? We need to figure out what the “driver” molecule is. But is there such a driver?

Some researchers reported the results of experimental targeted therapy in cell models, animal models, and in patients. The animal models are particularly intriguing and are real breakthroughs.

This meeting is very heavy on characterizing patients with SDH mutations and their individual mutations. The European countries are leading this effort. I have always wondered if SDH mutations are more common in Europe. In this meeting, I saw again some small countries reporting hundreds of patients with SDH mutations. I myself have not seen many patients in the US with clear familial paraganglioma syndromes. An alternative explanation is the difference in health care system. I cannot tell you how many patients I no longer follow due to their change of insurance plan. The national insurance system in Europe is a blessing to patients with rare and complicated diseases like pheo.

During the meeting, especially around 3 am when I was suddenly awake and trying unsuccessfully to go back to sleep, I reflected on the big picture of pheo patient care and where we should direct our research resource. I will elaborate my thoughts in later posts.

Dr. Pheo

Friday, September 16, 2011

Report from ISP 2011 Part I

The 3rd International Symposium on Pheochromocytoma (called “ISP 2011” by the organizers) is being held in a Disneyland Hotel outside Paris. I am here attending this meeting. It is a 4-day meeting but most academic activities are concentrated on Sept 15 and 16.

The specific Disneyland hotel is kind of an odd choice of venue for an American attendee like me. I would pick a downtown Paris hotel on the left bank. Apparently my European colleagues are tired of Paris and prefer a place on a theme park. The hotel is full of little cute girls and boys dressed in Disney costumes, or pestering their parents to buy them a set or 2 of. The hotel is so serious about the Disney theme that you see Monsieur Mickey everywhere, including on shower curtain. At one moment today, I felt I was at home until someone said Bonjour to me. And a bottle of coke costs 3.5 Euros.

The meeting itself is simply fantastic so far. Today’s lectures and posters are actually very educational to me even though I believe I knew a few things about pheo before the meeting (I indeed presented a poster on pheo clinical care). Really great is the discussion with colleagues. All the big shots on pheo are here, including most doctors I recommended as pheo experts in a post 2 years ago. I am pleased to say that we share similar experience and opinions on many controversial issues. Patient support groups also have a prominent presentation.

The following is an incomplete list of new things I learned today:
1. Practical half-life of plasma metanephrines is longer than most people thought. Plasma metanephrines can linger for one month after complete pheo resection.
2. Most attendees agree on the importance of preoperative preparation even when the patient does not have hypertension at the time of diagnosis.
3. First-hand accounts of the discovery processes of the novel pheo genes TMEM127, SDHAF2, and MAX, by the discoverers themselves.
4. The 3 novel genes, however fascinating for studying pheo pathogenesis, probably have limited value in clinical care for most patients.
5. Apart from family history and clinical predictors, pheo markers and pheo tissue staining can also predict specific mutations.
6. No real progress in pathological diagnosis of malignant pheo. Basically even an expert pathologist cannot accurately tell if a primary pheo is benign or malignant.
7. Surgical indications of head and neck paragangliomas.
8. Still there are no commercial labs that can do plasma methoxytyramine in the US.
9. More and more people realize that pheo causes heart damage. I have observed that for many years and it is nice to see people now agree.
10. Some new vagaries of pheo. For example, cardiomyopathy can have a waxing-and-waning course even after phenoxybenzamine.

To be continued…

Dr. Pheo