Solid Pseudopapillary Neoplasms (SPNs) of the Pancreas: Appraisal of Contemporary Clinicopathologic Features - Clinical Implications and Surgical Outcomes of Patients Treated and Followed at a Single Institution.
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BACKGROUND: Surgeons rarely encounter solid pseudopapillary neoplasms (SPNs) of the pancreas. Contemporary clinicopathologic features, operative interventions, and oncologic outcomes remain under-reported. METHODS: We reviewed clinical data and pathology from a single institution's experience of patients who underwent surgical resection with primary and recurrent SPNs treated between January 2004 and December 2023. Patient demographics, imaging results, tumor characteristics, treatment methods, genomic testing outcomes, recurrence-free survival, and survival were recorded and analyzed. RESULTS: Fifty patients comprised the study group, forty-eight were females, with a median age of 33 years (IQR:15.95, 16-53 years). Thirty patients were symptomatic, most commonly presenting with abdominal pain (n = 24). SPNs frequently demonstrated solid and cystic components and a 4.1 cm median tumor size. At a median follow-up of 53 months, three patients (6%) had recurrence of disease. Ten patient tumors underwent genomic sequencing: all containing a CTNNB1 mutation. Co-occurring PTEN and TP53 mutations were identified in two patient tumors that recurred. Treatments entailed cytoreductive surgical procedures, regional, and systemic therapies. One patient succumbed to disease progression. CONCLUSION: Patients with solid pseudopapillary neoplasms had a favorable oncologic outcome. Recurrence/metachronous metastasis occurred in older patients with larger tumors, each containing a pathogenic co-mutation, and one patient experiencing traumatic tumor rupture.