Postoperative Recurrences in Patients Operated for Pheochromocytomas and Paragangliomas: New Data Supporting Lifelong Surveillance

SIMPLE SUMMARY: At least 10% of pheochromocytomas (PHEOs) and paragangliomas (PPGLs) may recur after the initial surgery. The optimal follow-up time for these tumors remains unknown. We present a cohort of recurrent PPGLs in a clinical care setting of a quaternary center. In this paper, we describe recurrence patterns based on tumor location (head and neck paragangliomas, thoracoabdominal paragangliomas, and pheochromocytomas). We report that the overall mean delay of recurrence was 9.7 years and that one-third of the cohort had a recurrence more than 10 years after the initial surgery. Additionally, 17.6% of recurrent PHEOs were smaller than the predicted cutoff for recurrence (5 cm). Finally, more than 50% of recurrent PPGLs harbored a germline mutation in a susceptibility gene. In sum, this paper supports that overall, the safest option remains a lifelong follow-up. ABSTRACT: At least 10% of pheochromocytomas (PHEOs) and paragangliomas (PGLs) (PPGLs) may recur after the initial surgery. Guidelines recommend annual screening for recurrence in non-metastatic tumors for at least 10 years after the initial surgical resection and lifelong screening in high-risk patients. However, recent data suggest that a shorter follow-up might be appropriate. We performed a retrospective analysis on patients with PPGLs who had local and/or metastatic recurrences between 1995 and 2020 in our center. Data were available for 39 cases of recurrence (69.2% female) including 20 PHEOs (51.3%) and 19 PGLs (48.7%) (13 head and neck (HNPGL) and 6 thoracoabdominal (TAPGL)). The overall average delay of recurrence was 116.6 months (14–584 months) or 9.7 years and the median was 71 months or 5.9 years. One-third of the cohort had a recurrence more than 10 years after the initial surgery (10–48.7 years). The average tumor size at initial diagnosis was 8.2 cm for PHEOs, 2.7 cm for HNPGLs, and 9.6 cm for TAPGLs. Interestingly, 17.6% of PHEOs were under 5 cm at the initial diagnosis. Metastatic recurrence was identified in 75% of PHEOs, 15.4% of HNPGLs, and 66.7% of TAPGLs. Finally, 12/23 (52.2%) patients with recurrence who underwent genetic testing carried a germline mutation. Overall, the safest option remains a lifelong follow-up.