Long-Term Outcomes After Surgery for Pheochromocytoma and Sympathetic Paraganglioma

Overview

Journal Cancers (Basel)

Publisher MDPI

Specialty Oncology

Date 2023 Jun 10

PMID 37296853

Authors

Francesca Torresan

Arianna Beber

Donatella Schiavone

Stefania Zovato

Francesca Galuppini

Filippo Crimi

Filippo Ceccato

Maurizio Iacobone

Affiliations

Soon will be listed here.

Abstract

Background: The prognosis of pheochromocytoma and sympathetic paraganglioma (PHEO/sPGL) is difficult to predict at the time of diagnosis and long-term follow-up data are scarce, especially for apparently benign and sporadic variants. The aim of the study was to analyze the long-term outcomes in PHEO/sPGL patients.

Methods: A monocentric series of 170 patients who underwent surgery for PHEO/sPGL was analyzed.

Results: The study cohort included 91 female and 79 males with a median age of 48 years (range 6-83). The majority of PHEO/sPGL cases were considered apparently benign at the time of diagnosis; evident malignant behavior was found in 5% of cases. The overall 10-year risk of recurrence was 13%, but it rose up to 33% at 30 years. The risk of new tumor recurrence was higher in patients with hereditary tumors, but the risk was still significant in patients with apparently sporadic variants (20-year risk: 38% vs. 6.5%, respectively; < 0.0001). The risk of metastatic recurrence was higher in patients with locally aggressive tumors at diagnosis, but the risk was present also in apparently benign variants (5-year risk: 100% vs. 1%, respectively; < 0.0001).

Conclusions: Lifelong follow-up is required not only for hereditary PHEO/sPGL but also for apparently benign and sporadic tumors at diagnosis because of the risk of long-term recurrent disease.

Citing Articles

Progress in surgical approaches and outcomes of patients with pheochromocytoma and paraganglioma.

Friedman L, Ramamoorthy B, Nilubol N Best Pract Res Clin Endocrinol Metab. 2024; 39(1):101954.

PMID: 39366823 PMC: 11788041. DOI: 10.1016/j.beem.2024.101954.

Metastatic disease and major adverse cardiovascular events preceding diagnosis are the main determinants of disease-specific survival of pheochromocytoma/paraganglioma: long-term follow-up of 303 patients.

Raber W, Schendl R, Arikan M, Scheuba A, Mazal P, Stadlmann V Front Endocrinol (Lausanne). 2024; 15:1419028.

PMID: 39234504 PMC: 11371702. DOI: 10.3389/fendo.2024.1419028.

Genetic and Molecular Biomarkers in Aggressive Pheochromocytomas and Paragangliomas.

Torresan F, Iacobone C, Giorgino F, Iacobone M Int J Mol Sci. 2024; 25(13).

PMID: 39000254 PMC: 11241596. DOI: 10.3390/ijms25137142.

Bilateral pheochromocytomas: clinical presentation and morbidity rate related to surgery technique and genetic status.

Lider Burciulescu S, Livia Gheorghiu M, Muresan A, Gherlan I, Patocs A, Badiu C Endocr Connect. 2024; 13(4).

PMID: 38318817 PMC: 10959043. DOI: 10.1530/EC-23-0466.

Surgery for advanced adrenal malignant disease: recommendations based on European Society of Endocrine Surgeons consensus meeting.

Mihai R, De Crea C, Guerin C, Torresan F, Agcaoglu O, Simescu R Br J Surg. 2024; 111(1).

PMID: 38265812 PMC: 10805373. DOI: 10.1093/bjs/znad266.

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