Recurrence Rate of Sporadic Pheochromocytomas After Curative Adrenalectomy: A Systematic Review and Meta-analysis

Abstract

Background Evidence on follow-up duration for patients with sporadic pheochromocytomas is absent and current guidelines of the European Society of Endocrinology (ESE), American Association of Clinical Endocrinologists and Endocrine Surgeons (AACE/AAES) and Endocrine Society (ES) are ambiguous about the appropriate duration of follow-up. The aim of this systematic review and meta-analysis is to evaluate the recurrence rate of sporadic pheochromocytomas after curative adrenalectomy. Materials and Methods A literature search in PubMed, Embase and the Cochrane Library was performed. A study was eligible if it included a clear report on the number of sporadic patients, recurrence rate and follow-up duration. Studies with an inclusion period before 1990, <2 years of follow-up, <10 patients and unclear data on sporadic nature of pheochromocytomas were excluded. A meta-analysis on recurrence was performed provided that the heterogeneity was low (I2 < 25%) or intermediate (I2 26-75%). Hozo’s method was used to calculate weighted mean follow-up duration and weighted time to recurrence with combined standard deviations. Results A total of 13 studies, including 430 patients, was included in the synthesis. The meta-analysis results describe a pooled recurrence rate after curative surgery of 3% (95%CI 2-6%, I2 = 0%) with a weighted mean time to recurrence of 49.4 months (SD = 30.7) and a weighted mean follow-up period of 77.3 months (SD = 32.2). Conclusions This meta-analysis shows a very low recurrence rate of 3%. Prospective studies, including economical and health effects of limited follow-up strategies for patients with truly sporadic pheochromocytomas should be considered.

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

“…A recent systematic review showed that the overall rate of recurrent PPGL was 0.98 events/100 person-years, which suggested the risk of recurrence after complete resection was previously underestimated ( 9 ). In a meta-analysis of 13 studies, the mean time to recurrence was approximately 4 years (0.5-12) ( 6 ). In our study, recurrent patients exhibited a median recurrence time of 6.0 (4.0, 9.0) years after resection of the primary tumor, which is longer than previous reports ( 2 , 6 ).…”

“…In a meta-analysis of 13 studies, the mean time to recurrence was approximately 4 years (0.5-12) ( 6 ). In our study, recurrent patients exhibited a median recurrence time of 6.0 (4.0, 9.0) years after resection of the primary tumor, which is longer than previous reports ( 2 , 6 ). Our data suggest that the follow-up of all patients after tumor resection should be at least 10-20 years, as recurrences may occur after a prolonged time after initial treatment.…”

突然の心不全を契機に発見された基礎疾患のない若年者の褐色細胞腫の1例(A case of pheochromocytoma in young patient without cardiovascular risk factors that found out due to sudden heart failure)

“…先行論文では，既往がない若年者で褐色細胞腫と診断する難しさと重要性を指摘する 9)。実際，早期発見されず心臓移植やペースメーカー留置など不必要かつ侵襲性の高い処置が施された症例もあった 9)。本症例でも，受診直後の循環器内科医による「たこつぼ型心筋症」との診断は，褐色細胞腫と迅速に診断する難しさを如実に表している。各臓器に特化した診療科では症状からその臓器に焦点を当てて診療するため見落としてしまう疾患も多々ある。しかし総合的に診療する救急医の役目はこの見落としを補うことである。ありふれた症状から褐色細胞腫という稀な疾患を想起することは，すべての医療者にとって困難である。一方で，一般的に褐色細胞腫は診断されれば手術により再発はほぼない 10), 11)。ただし，報告によっては再発リスクが高い，との警告もある 12)。長い将来のある若年者での再発は看過できない。適切な診断・治療およびフォローアップのため，すべての医療者は，改めて既往のない若年者での褐色細胞腫に注意するよう認識すべきだろう。…”

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

“…Recurrences were more frequent in patients with PGLs and familial disease [ 17 ]. A recent meta-analysis by Holscher et al showed a 3% recurrence rate in sporadic PHEOs after curative adrenalectomy with a median recurrence time of 49.4 months and a follow-up time of 77.3 months [ 18 ]. These two recent meta-analyses show lower recurrence rates than what was initially believed, calling into question the long-term follow-up for PPGLs.…”

“…In sum, the optimal timing, duration, and modality of follow-up vary from study to study due to a lack of systematic studies and the rarity of the disease [ 6 , 9 , 18 ]. Our primary objective for this study was to retrospectively evaluate the rate and delay of recurrence in our cohort of patients with PPGLs in a real-life clinical setting.…”