Outcomes of Surgical Management of Retroperitoneal Paragangliomas: A Single Centre Experience

Retroperitoneal paragangliomas (RP-PGLs) are rare extra-adrenal neuroendocrine tumors representing ~ 1-3% of retroperitoneal masses. They may secrete catecholamines, causing hypertension and life-threatening complications if untreated. Surgical resection is the mainstay of therapy, yet data-particularly from the Indian subcontinent-are scarce. To evaluate clinical presentation, diagnostic workup, perioperative management and long-term outcomes of RP-PGL resections at a tertiary oncology center. A retrospective analysis of 30 patients who underwent RP-PGL resection from 2005 to 2023 was done. Preoperative evaluation was based on standard institutional protocols after a multidisciplinary discussion. Data collected included operative time, blood loss, histopathology (tumor size, stage, margins, SDH/MEN mutations) and Clavien-Dindo morbidity. Overall survival (OS) and disease-free survival (DFS) were estimated via Kaplan-Meier analysis. Median age was 44 years (range 22-66); 40% were male. Presentations: pain (56.7%), incidental mass (23.3%), classical symptoms (6.7%). Half had secretory tumors; 40% of these exhibited uncontrolled hypertensions. R0 resection was achieved in 73.3%, including 13.3% multivisceral/vascular resections. SDH and MEN mutations were found 16.7%, and 3.3% respectively. Postoperative morbidity ≥Clavien-Dindo IIIa occurred in 16.7%, with no 90-day mortality. At a median follow-up of the 36.9 months (95% C.I: 10.9 to 62.8 months), median OS and DFS were not reached and the 3-year Overall survival (OS) and 3-year disease free survival (DFS) was 95.5% (91.1 to 99.9%) and 89.1% (81.6 to 96.6%) respectively. Three recurrences occurred; two responded to peptide receptor radionuclide therapy. Surgical management of RP-PGLs is safe and achieves excellent long-term survival. A standardized diagnostic algorithm, multidisciplinary perioperative optimization and readiness for complex resections are key to favorable outcomes.