Recommendations
from ISP2005: Resolution of many of the problem issues in the form of
generally agreed upon recommendations was not always possible due to a lack of
suitably sized evidence-based studies. It also became clear during the course
of discussions that strict guidelines are rendered inappropriate by variability
in the presentation of tumors and by international differences in medical approaches
or availability of tests and therapies. Where provided, outlined recommendations
are therefore intended to complement sound clinical judgment, and are not provided
as rigid guidelines. Biochemical Diagnosis and Localization
Initial biochemical testing for pheochromocytoma should include measurements of
plasma free metanephrines or urinary fractionated metanephrines [ details]. - Reference
intervals for initial tests of plasma or urinary fractionated metanephrines should
be established primarily to ensure optimum diagnostic sensitivity, with specificity
a secondary consideration [details].
- Testing
algorithms should not simply rely on a binary approach for test interpretation
(i.e., whether a test result is negative or positive), but should instead take
advantage of the continuous nature of biochemical test results [details].
- Interpretation
of positive test results in the "grey area" requires consideration -
and where possible elimination - of causes of false-positive results before further
confirmatory testing is initiated [details].
- Imaging
studies to search for a pheochromocytoma should usually only be initiated once
biochemical or other evidence of the tumor is reasonably compelling [details].
-
Anatomic imaging studies - CT or MRI - provide the most appropriate
tools for initial localization of a pheochromocytoma. Although additional functional
imaging studies may not always be called for, such studies can be useful to prove
that a localized mass is indeed a pheochromocytoma and to correctly detect any
extension of disease, not identified by anatomic imaging [details].
There are now reasonable arguments for more widespread genetic
testing than previously practiced; however, it is currently neither appropriate
nor cost-effective to test every disease-causing gene in every patient with a
pheochromocytoma. Rather, the decision to test, and which genes to test, requires
judicious consideration of numerous factors [ details]. - Pheochromocytomas
are neuroendocrine tumors derived from catecholamine-producing chromaffin cells
of the adrenal medulla, whereas extraadrenal paragangliomas arise from chromaffin
cells of the extraadrenal paraganglia. However, the same genetic testing often
applies to both [details].
All patients
with a biochemically positive pheochromocytoma or paraganglioma should receive
appropriate preoperative medical management to block the effects of released catecholamines
[ details].
All patients should receive appropriate follow-up after surgical resection of
a pheochromocytoma or paraganglioma [ details]. Concerted
efforts are required to identify new targets for treatment of metastatic pheochromocytoma
and set up multicenter clinical trials to examine and compare existing and new
therapies [ details].
No consensus was reached on adoption of a formal scoring system;
however, it was recommended that pathology reports should conform to templates
or checklists for minimal standard reporting endorsed by several pathology associations.
The templates list the major elements of the proposed scoring systems and permit
or encourage reporting of additional optional elements [ details].
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