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Multicenter Study
. 2023 Jan 17;108(2):397-404.
doi: 10.1210/clinem/dgac563.

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma

Minghao Li  1 , Tamara Prodanov  2 , Leah Meuter  2 , Michiel N Kerstens  3 , Nicole Bechmann  4 , Aleksander Prejbisz  5 , Hanna Remde  6 , Henri J L M Timmers  7 , Svenja Nölting  8   9 , Sara Talvacchio  2 , Annika M A Berends  3 , Stephanie Fliedner  10 , Mercedes Robledo  11   12 , Jacques W M Lenders  1   7 , Karel Pacak  2 , Graeme Eisenhofer  1   4 , Christina Pamporaki  1
Affiliations
Multicenter Study

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma

Minghao Li et al. J Clin Endocrinol Metab. .

Abstract

Context: Long-term follow-up has been recommended for patients with pheochromocytoma or paraganglioma (PPGL) due to potential for recurrent disease. However, the need to follow patients with sporadic PPGL has recently become controversial.

Objective: To investigate the prevalence of recurrence among patients with sporadic compared with hereditary PPGL and to identify predictors of recurrence for sporadic disease.

Methods: This multicenter study included retrospective data from 1127 patients with PPGL. In addition to sex and age at primary tumor diagnosis, clinical information included location, size, and catecholamine phenotype of primary tumors, genetic test results, and subsequent development of recurrent and/or metastatic disease. Patients with sporadic PPGL were defined as those with negative genetic test results.

Results: Prevalence of recurrence among patients with sporadic PPGL (14.7%) was lower (P < 0.001) than for patients with pathogenic variants that activate pseudohypoxia pathways (47.5%), but similar to those with variants that activate kinase pathways (14.9%). Among patients with sporadic recurrent PPGL, 29.1% and 17.7% were respectively diagnosed at least 10 and 15 years after first diagnosis. Multivariable regression analysis showed that a noradrenergic/dopaminergic phenotype (HR 2.73; 95% CI, 1.553-4.802; P < 0.001), larger size (HR 1.82; 95% CI, 1.113-2.962; P = 0.017) and extra-adrenal location (HR 1.79; 95% CI, 1.002-3.187; P = 0.049) of primary tumors were independent predictors of recurrence in sporadic PPGL.

Conclusion: Patients with sporadic PPGL require long-term follow-up, as supported by the 14.7% prevalence of recurrent disease, including recurrences at more than 10 years after first diagnosis. The nature of follow-up could be individualized according to tumor size, location, and biochemical phenotype.

Keywords: disease free period; metastases; paraganglioma; pheochromocytoma; recurrence; sporadic.

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Figures

Figure 1.
Figure 1.
Flow chart of patients enrolled in the study.
Figure 2.
Figure 2.
Cumulative hazard of recurrence (A) and only metastatic disease (B) among patients with sporadic vs hereditary PPGL. Patients with cluster 1 PPGL presented with a 2.45-fold higher risk of recurrence compared with those with sporadic tumors, whereas risk of recurrence was similar between patients with cluster 2 and sporadic PPGL (A). Similarly, patients with cluster 1 PPGL presented with a 2.48-fold higher risk of metastasis compared with those with sporadic tumors, whereas risk of recurrence was similar between patients with cluster 2 and sporadic PPGL (B).
Figure 3.
Figure 3.
Recurrence- (A) and metastasis- (B) free period of patients with sporadic PPGL and recurrent disease. Percentage of patients that developed recurrence (A) or only metastatic disease (B) within the specific time periods (5, 10, 15 years) corresponds to the black part of each bar.
Figure 4.
Figure 4.
Multivariable Cox regression analysis for predictors of recurrent disease for patients with sporadic PPGL.
See this image and copyright information in PMC

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