Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes

Abstract

ObjectiveTo study the characteristics, risk factors, and outcomes of local-regional recurrence of pheochromocytoma and paraganglioma (PPGL).MethodsClinical data of 96 PPGL patients with local-regional recurrence and 112 patients without recurrence were retrospectively analyzed.ResultsRecurrent patients exhibited a median recurrence time of 6.0 (4.0, 9.0) years after resection of the primary tumor. SDHB mutation [HR 4.1 (1.7, 9.5), p=0.001), primary tumor size ≥5cm [HR 2.3 (1.1, 4.7), p=0.028], and average Ki-67 count ≥3% in the primary tumor [HR 2.6 (1.4, 4.9), p=0.003] were independent predictors for recurrence of PPGL. Primary tumor sizes ≥5cm [HR 5.1 (1.7, 15.3), p=0.003] and average Ki-67 counts ≥3% of the primary tumor [HR 2.4 (1.1, 5.2), p=0.035] were independent predictors for recurrence of pheochromocytoma, while SDHB mutation [HR 4.6 (1.5, 13.9), p=0.007] was a predictor for paraganglioma recurrence. Among recurrent patients, 47% (45/96) had multiple nodules in recurrent sites, and 58% (56/96) had metastases, with 20% (19/96) being implantation metastases. The risk of metastases (42% vs. 25%, p=0.030) and death (15% vs. 8%, p=0.003) was significantly increased in untreated patients after recurrence compared with treated patients.ConclusionLong-term follow-up is necessary for all PPGL patients. Risk factors for recurrence of pheochromocytoma and paraganglioma differ, with primary tumor size and average Ki-67 count representing independent predictors for pheochromocytoma patients and SDHB mutations predicting paraganglioma recurrence. Although the treatment of recurrence can be difficult, patients should be treated once recurrence is detected as it decreases the risk of metastases and death.

Reduced Risk of Corporal Tumors in Patients With Head and Neck Paragangliomas With p.Pro81Leu Mutations

“…Our study was not able to delineate which patients underwent treatment and which were observed. This is much higher than the previously reported 6% to 8% 17,18 over a 4‐year follow‐up period, though more similar to the rates of all paragangliomas reported by Cui et al 19 Those researchers followed 66 patients, 29 with known recurrent pheochromocytoma and paraganglioma, and found 21% experienced recurrence within 10 to 20 years. As paragangliomas in the head and neck are known to be slow‐growing, 20 timing may play a role in the detection of recurrence or progression.…”

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma

“…The latter might involve genes that impact telomere maintenance, such as NOP10, ATRX, and TERT, or MAML3 gene translocations (14,37). Larger tumors might also harbor a higher rate of genomic alterations and have a higher probability of being locally advanced, with capsular, vascular, or surrounding tissue invasion, conditions related to high rates of recurrence (4,7,38). The above possibilities might also explain recurrences in more than 50% of sporadic PPGL that involved metastatic disease.…”

Pediatric malignant pheochromocytoma with atypical presentation as vision changes, lung metastasis, and recurrence: a case report

“…and Parasiliti-Caprino et al . suggested that lager primary tumor sizes (≥ 8 cm in diameter), Ki-67 (counting 2–10%), younger age (10 years old), and higher PASS value (score at least 8) were associated with recurrence of pheochromocytoma for this patient [ 7 – 9 ]. Additionally, Amar et al .…”

“…In addition, Cui et al . found that metastatic patients with pheochromocytoma (PHEOs)/ganglioneuroblastoma (PGLs) with SDHB mutations had a shorter postoperative progression-free survival [ 7 ]. Therefore, an unplanned surgery was recommended in our case.…”