NEWS & PERSPECTIVE DRUGS & DISEASES CME & EDUCATION ACADEMY CONSULT VIDEO DECISION POINT

Drugs & Diseases > Endocrinology

Pheochromocytoma Recommended
Guidelines DISEASES & CONDITIONS
Neuroendocrine Tumors
Updated: Aug 20, 2021 | Author: Michael A Blake, MBBCh, MRCPI, FRCR;
Guidelines
Chief Editor: George T Griffing, MD more...

27 Feedback Treatment Selection

for
Gastroenteropancreatic
Neuroendocrine Tumors: Is It
Time to Combine?
SECTIONS 0. 5 C M E C R E D I TS

Guidelines Summary
In 2014, the Endocrine Society (ES), 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). [68]

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. [68]

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 [37] :

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 ES/AACC/ESE guidelines

recommend CT scanning as first line, rather than 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.
[68]

The National Comprehensive Cancer

Network guidelines recommend the following tests for
the evaluation of suspected PPGL [69] :

Plasma free or 24-hour urine fractionated

metanephrines
Contrast-enhanced chest/abdominal/pelvic
multiphasic CT scanning or MRI
Genetic counseling

If metastatic disease is suspected, the guidelines

recommend the following additional tests [69] :

MIBG scan
Somatostatin receptor scintigraphy
Fluorodeoxyglucose PET (FDG-PET)/CT scan
If bone symptoms, bone scan

For genetic testing, ES/AACC/ESE recommendations

are as follows [68] :

Patients with PPGLs should be engaged in shared

decision-making for genetic testing
Patients with paraganglioma should undergo
testing of succinate dehydrogenase mutations
Patients with metastatic disease should undergo
testing for SDHB mutations
Genetic testing should include pretest and
posttest genetic counseling

For preoperative management, ES/AACC/ESE

recommendations include the following [68] :

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 ES/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. [68]

In the immediate postoperative period, the

ES/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. [68]

The 2010 NANETS recommendations for treatment of

advanced disease include the following [37] :

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 MIBG scintigraphy and in those with
rapidly progressing tumors
External-beam irradiation of bone metastases or
radiofrequency and cryoablation in selected
cases only

Medication

SECTIONS
Pheochromocytoma

Overview

Presentation

DDx

Workup

Treatment

Guidelines

Medication

Medication Summary

Alpha Blockers, Antihypertensives

BPH, Alpha Blocker

Vasodilators

Beta Blockers, Nonselective

Beta Blockers, Beta1 Selective

Antihypertensives, Other

Radiopharmaceuticals

Show All

Questions & Answers

Media Gallery

References

What to Read Next on Medscape

NEWS & PERSPECTIVE

Related Conditions
and Diseases SLIDESHOW

Neuroendocrine Tumors
Guidelines

Pancreatic
Neuroendocrine (Islet Tissue-Sparing
Cell) Tumor Imaging Resection, Ablation Pancreatic Cancer:
Option for NET Liver Difficult Diagnosis,
Carcinoid Tumor Mets Ominous Outlook
Thymic Tumors
2023 ASCO GI
Systemic Treatment of Symposium: New,
Metastatic Measured Optimism
Gastroenteropancreatic
Neuroendocrine Tumors
Longer Survival Not Top
5-Hydroxyindoleacetic Priority for Some Patients
Acid (5-HIAA) With Cancer

TOOLS

Drug Interaction
Checker

Pill Identifier

Calculators

Formulary

FIND US ON ABOUT MEMBERSHIP APP

About Medscape About You Medscape

Privacy Policy Professional Information

Editorial Policy Newsletters & Alerts

Cookies Market Research

Terms of Use

Advertising Policy

Help Center

WEBMD NETWORK EDITIONS

Medscape Live Events English

WebMD Deutsch

MedicineNet Español

eMedicineHealth Français

RxList Português

WebMD Corporate UK

Medscape UK

All material on this website is protected by copyright, Copyright © 1994-2023 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.