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New guideline recommends 10-year follow-up for pheochromocytoma, paraganglioma
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Patients who have undergone surgery to remove a pheochromocytoma or paraganglioma should be followed for at least 10 years to assess for local or metastatic tumor recurrence, according to a new clinical practice guideline released by the European Society of Endocrinology.
The incidence of tumor recurrence after surgery for pheochromocytoma or paraganglioma (PPGL) is rare, about one per 100 person-years, according to researchers; however, more than 40% of new events are malignant recurrences, and new events may occur after a 5-year period.
Pierre-François Plouin
“The general follow-up procedure should include annual measurement of metanephrines, which are markers for PPGL, in both the blood and urine,” Pierre-François Plouin, MD, PhD, professor of vascular medicine at Universite Paris Descartes and Hôpital Européen Georges Pompidou in Paris, said in a press release. “This would be a standard for all patients. Measurement of chromogranin A, a protein elevated in some PPGL patients, is only recommended in a subgroup. Additional guidance is then given for specific conditions, such as pregnancy, the elderly and frail patients.”
In a systematic review, Plouin and colleagues analyzed 42 studies that included 38 patient cohorts with PPGL tumors, along with records from 1,153 patients from the European Network for the Study of Adrenal Tumors. A total of 2,396 patients had undergone curative surgery.
Pooled incidence rate of postoperative new events, based on data from 34 cohorts, was 0.98 (95% CI, 0.71-1.25) per 100 person-years.
“The 5-year risk of new events is 27% when the size of the tumor exceeds 150 mm, but there is no ‘safe’ tumor size below which there is no risk for a new event,” the researchers wrote. “Likewise, the 5-year risk of a new event is 27% in young patients (< 20 years old) and around 20% to 25% in those with paraganglioma, but older patients and those with pheochromocytoma still have a 5-year risk of new events around 10%.”
Researchers recommended that patients who undergo surgery for PPGL be provided with a personalized estimate of the risk for new events based on age, genetic test results and tumor site and size. In addition, high-risk patients, including young patients and those with a predisposition for the disease, should receive lifetime follow-up.
The new guideline, according to researchers, complements the clinical practice guideline recently published by the Endocrine Society, which focused on the diagnosis and treatment of PPGL.
The new clinical practice guideline was published online in the European Journal of Endocrinology. – by Regina Schaffer
Disclosure: The researchers report no relevant financial disclosures.
Perspective
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Stephanie L. Lee, MD, PhD, ECNU
This guideline is a complement to the Endocrine Society clinical practice guideline published in 2014, but there clearly are some differences in their recommendations. I believe the differences are supported with relatively poor quality data, with a lot of the differences driven by expert opinion.
I was surprised with the European recommendations because they really emphasize screening with plasma free metanephrine or 24-hour urinary fractionated metanephrines, but some tumors do not make metanephrine. Some tumors make dopamine instead. The Endocrine Society guidelines are a little more specific, noting that how a clinician should follow the patient depends on the tumor. This guideline also makes a relatively big issue about screening patients with plasma methoxytyramine, something elevated only with metastatic disease in pheochromocytoma or paraganglioma, but, according to my reading of the literature, there are a limited number of papers using this.
This guideline is a very brief document and includes a lot of generalities. It may create more questions during follow-up as clinicians are looking at patients who are sporadic vs. genetic. It really is a lot of expert opinion vs. objective data.
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