Problem
Issues and Questions Requiring Resolution:
Biochemical Diagnosis
and Localization
What are the preferred
biochemical tests or testing algorithms for confirming or excluding pheochromocytoma
and what cost-benefit factors should be considered?
What precautions
should be considered to minimize or avoid false-positive results or drug-interferences
during biochemical testing and imaging procedures?
What evidence for the
presence of a tumor justifies imaging studies?
What imaging strategies
are appropriate for localization of pheochromocytoma and in what order should
they be applied?
Genetics
Should all patients with pheochromocytomas undergo genetic testing for possible
disease-causing mutations, or should this be confined to patients where there
is a suspicion of an existing syndrome or only to young patients?
What
are cost-benefit factors to be taken into account when genetic testing is considered?
How
should paragangliomas and pheochromocytomas best be defined?
Management
and Treatment
What are the most appropriate
strategies for the medical management of patients with pheochromocytoma before
and during surgery? Which drugs and at what doses should these drugs be used?
Which clinical parameters should be followed? Which surgical approaches or alternatives
to surgery for different adrenal and extra-adrenal tumors should be considered?
What
medical management should be considered for pregnant patients with pheochromocytoma
or those who are asymptomatic and normotensive?
What is the appropriate
follow-up of patients who have undergone surgical resection of a pheochromocytoma?
What
should be done for patients with non-resectable tumors or malignancies? Is chemotherpay
useful? Does MIBG radionuclide therapy provide adequate treatment? Can such existing
therapies be improved upon or tailored according to tumor characteristics? Is
there a need for new targets for treatment of malignant pheochromocytoma and if
so, what are the most promising leads and approaches to identify such targets
and develop new drugs for these targets? Is there a need for experimental models
of pheochromocytoma, and if so, for what specific purpose?
Pathology
Can a reproducible, statistically validated pathology scoring system be developed
to identify high-risk/poor prognosis tumors within groups with known or unknown
mutations?
Can pathology be helpful in identifying tumors with particular
mutations?
What ancillary immunohistochemical studies should be performed
for risk assessment, phenotype characterization, and identification of targets
for therapy
