Diagnosis and Treatment of Pheochromocytoma and Paraganglioma

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.

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