Pheochromocytoma and Paraganglioma
Pheochromocytoma and Paraganglioma
The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL).
The Task Force included a chair selected by the Endocrine Society Clinical Guidelines Subcommittee (CGS), seven experts in the field, and a methodologist. The authors received no corporate funding or remuneration.
This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. The Task Force reviewed primary evidence and commissioned two additional systematic reviews.
One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of the Endocrine Society, European Society of Endocrinology, and Americal Association for Clinical Chemistry reviewed drafts of the guidelines.
The Task Force recommends that initial biochemical testing for PPGLs should include measurements of plasma free or urinary fractionated metanephrines. Consideration should be given to preanalytical factors leading to false-positive or false-negative results. All positive results require follow-up. Computed tomography is suggested for initial imaging, but magnetic resonance is a better option in patients with metastatic disease or when radiation exposure must be limited. 123I-metaiodobenzylguanidine scintigraphy is a useful imaging modality for metastatic PPGLs. We recommend consideration of genetic testing in all patients, with testing by accredited laboratories. Patients with paraganglioma should be tested for SDHx mutations, and those with metastatic disease for SDHB mutations. All patients with functional PPGLs should undergo preoperative blockade to prevent perioperative complications. Preparation should include a high-sodium diet and fluid intake to prevent postoperative hypotension. We recommend minimally invasive adrenalectomy for most pheochromocytomas with open resection for most paragangliomas. Partial adrenalectomy is an option for selected patients. Lifelong follow-up is suggested to detect recurrent or metastatic disease. We suggest personalized management with evaluation and treatment by multidisciplinary teams with appropriate expertise to ensure favorable outcomes.
This continuing medical education activity should be of substantial interest to endocrinologists and other health care professionals that treat patients with Pheochromocytoma and Paraganglioma.
Upon completion of this educational activity, learners will be able to:
- Identify the best biochemical test strategy for PPGL
- Utilize the preferred functional imaging strategy to locate PPGL
- Determine the priorities in genetic testing in patients with a proven PPGL
- Manage a patient with PPGL before surgical removal
STATEMENT OF INDEPENDENCE
As a provider of continuing medical education (CME) accredited by the Accreditation Council for Continuing Medical Education, the Endocrine Society has a policy of ensuring that the content and quality of this educational activity are balanced, independent, objective, and scientifically rigorous. The scientific content of this activity was developed under the supervision of The Pheochromocytoma and Paraganglioma Guidelines Task Force.
The faculty, committee members, and staff who are in position to control the content of this activity are required to disclose to the Endocrine Society and to learners any relevant financial relationship(s) of the individual or spouse/partner that have occurred within the last 12 months with any commercial interest(s) whose products or services are related to the CME content. Financial relationships are defined by remuneration in any amount from the commercial interest(s) in the form of grants; research support; consulting fees; salary; ownership interest (e.g., stocks, stock options, or ownership interest excluding diversified mutual funds); honoraria or other payments for participation in speakers' bureaus, advisory boards, or boards of directors; or other financial benefits. The intent of this disclosure is not to prevent CME planners with relevant financial relationships from planning or delivery of content, but rather to provide learners with information that allows them to make their own judgments of whether these financial relationships may have influenced the educational activity with regard to exposition or conclusion.
The Endocrine Society has reviewed all disclosures and resolved or managed all identified conflicts of interest, as applicable.
The following committee members who planned and/or reviewed content for this activity reported no relevant financial relationships: Jacques Lenders, MD, PhD, FRCP (chair); Quan-Yang Duh, MD; Graeme Eisenhofer, PhD; Anne-Paule Gimenez-Roqueplo, MD; Stefan K. G. Grebe, MD, PhD; M. Hassan Murad, MD; Mitsuhide Naruse, MD, PhD; Karel Pacak, MD, PhD, DSc and William Young, MD, MSc
Endocrine Society staff associated with the development of content for this activity reported no relevant financial relationships.
Use of professional judgment:
The educational content in this activity relates to basic principles of diagnosis and therapy and does not substitute for individual patient assessment based on the health care provider’s examination of the patient and consideration of laboratory data and other factors unique to the patient. Standards in medicine change as new data become available.
Drugs and dosages:
When prescribing medications, the physician is advised to check the product information sheet accompanying each drug to verify conditions of use and to identify any changes in drug dosage schedule or contraindications.
POLICY ON UNLABELED/OFF-LABEL USE
The Endocrine Society has determined that disclosure of unlabeled/off-label or investigational use of commercial product(s) is informative for audiences and therefore requires this information to be disclosed to the learners at the beginning of the presentation. Uses of specific therapeutic agents, devices, and other products discussed in this educational activity may not be the same as those indicated in product labeling approved by the Food and Drug Administration (FDA). The Endocrine Society requires that any discussions of such “off-label” use be based on scientific research that conforms to generally accepted standards of experimental design, data collection, and data analysis. Before recommending or prescribing any therapeutic agent or device, learners should review the complete prescribing information, including indications, contraindications, warnings, precautions, and adverse events.
PRIVACY AND CONFIDENTIALITY STATEMENT
The Endocrine Society will record learner's personal information as provided on CME evaluations to allow for issuance and tracking of CME certificates. The Endocrine Society may also track aggregate responses to questions in activities and evaluations and use these data to inform the ongoing evaluation and improvement of its CME program. No individual performance data or any other personal information collected from evaluations will be shared with third parties.
ACKNOWLEDGEMENT OF COMMERCIAL SUPPORT
This activity is not supported by educational grant(s) from commercial supporters.
- 2.00 AMA PRA Category 1 Credits™
- 2.00 CME Certificate of Participation