What are the guidelines on the diagnosis and treatment of pheochromocytoma and paraganglioma (PPGL)?

Updated: Dec 23, 2018
  • Author: Evan S Ong, MD, MS; more...
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In 2014, The Endocrine Society (TES), the American Association for Clinical Chemistry (AACC), and the European Society of Endocrinology (ESE) released joint clinical practice guidelines for the management of pheochromocytoma and paraganglioma (referred to together as PPGL). The guidelines include recommendations (based on strong evidence) and suggestions (based on weaker evidence). [21]

In patients suspected of having PPGL, biochemical testing via measurement of plasma free metanephrines or urinary fractionated metanephrines is recommended. The use of liquid chromatography with mass spectrometry or electrochemical-detection methods is suggested over other laboratory methods. Patients with a known germline mutation that predisposes to PPGL should undergo periodic biochemical testing. [21]

The 2010 guidelines from the North American Neuroendocrine Tumor Society (NANETS) recommend biochemical testing for pheochromocytoma that includes measurements of fractionated metanephrines in plasma, urine, or both, as available, in the following cases [22] :

  • Symptomatic patients

  • Patients with an adrenal incidentaloma

  • Patients who have a hereditary risk for developing a pheochromocytoma or paraganglioma (extra-adrenal pheochromocytoma)

For imaging studies, the joint TES/AACC/ESE guidelines recommend computed tomography (CT) as first line, rather than magnetic resonance imaging (MRI). However, MRI is an option in certain patients, such as those with metastatic PPGL; those allergic to CT contrast media; and those for whom radiation exposure should be limited, such as pregnant women. [21]

For preoperative management, TES/AACC/ESE recommendations include the following:

  • Blockade of hormonally functional PPGL to prevent cardiovascular complications

  • Medical treatment to normalize blood pressure and heart rate

  • A high-sodium diet with supplemental fluid intake to prevent severe hypotension after removal of the tumor

The TES/AACC/ESE guidelines recommend minimally invasive (eg, laparoscopic) adrenalectomy for most adrenal pheochromocytomas, with open resection reserved for very large or invasive pheochromocytomas. Open resection is suggested for paragangliomas, although laparoscopic resection is an option for smaller tumors. Partial adrenalectomy is also a possibility for certain types of patients.

In the immediate postoperative period, the TES/AACC/ESE guidelines recommend monitoring of blood pressure, heart rate, and glucose levels. Postoperative measurement of plasma or urine metanephrine levels and lifelong annual biochemical testing are suggested.

For genetic testing, TES/AACC/ESE recommendations are as follows:

  • Patients with PPGLs should be engaged in shared decision-making for genetic testing

  • Patients with paraganglioma should undergo testing of succinate dehydrogenase (SDH) mutations

  • Patients with metastatic disease should undergo testing for SDHB mutations

  • Genetic testing should include pre- and posttest genetic counseling

The 2010 NANETS recommendations for treatment of advanced disease include the following [22] :

  • Surgical debulking to release tumor pressure on surrounding tissues or to decrease tumor mass

  • In select patients, radiofrequency ablation or cryoablation are options

  • Chemotherapy is preferred in patients with negative metaiodobenzylguanidine (MIBG) scintigraphy and in those with rapidly progressing tumors

  • External-beam irradiation of bone metastases or radiofrequency and cryoablation in selected cases only

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