Full Guideline: Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline
JCEM June 2014
Jacques W. M. Lenders, Quan-Yang Duh, Graeme Eisenhofer, Anne-Paule Gimenez-Roqueplo, Stefan K. G. Grebe, Mohammad Hassan Murad, Mitsuhide Naruse, Karel Pacak, William F. Young, Jr.
Objective:
The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL).
Conclusions:
- 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.
- I-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.
Read the Full Guideline
