Abstract

BackgroundWe describe the presentation, diagnostic pathway, and management of 28 patients with benign retroperitoneal schwannomas. MethodsA prospectively kept soft-tissue tumor database was reviewed to identify patients who presented to the Royal Marsden Hospital with retroperitoneal schwannomas. ResultsFrom 2001 to 2009, 28 patients presented with retroperitoneal schwannomas. In 13 patients, tumors were identified incidentally, 8 patients presented with pelvic or abdominal symptoms and 7 patients presented with a palpable mass. Seventeen occurred in the pelvis and 11 occurred in the abdominal retroperitoneal space. The median age was 47 years and 21 patients were women; the mean follow-up period was 39 months. Twenty patients underwent resection and 8 patients were managed conservatively with radiologic surveillance. An initial histologic diagnosis was accomplished in 19 of 28 patients. Complete resection was achieved in 17 of 20 patients and 3 patients with pelvic schwannomas underwent a subtotal resection. Resected tumor size ranged from 5 to 23 cm (median, 9 cm), and weighed between 64 and 2,300 g (median, 500 g). There was no surgical mortality. In the 3 subtotal resected tumors, no progression of residual disease or malignant transformation has been noted on follow-up imaging. All 8 patients in the surveillance group had a histologic diagnosis and typical radiologic findings. ConclusionsAn accurate preoperative diagnosis is important because the risks of complicated surgery in the retroperitoneum and pelvis for what is a benign lesion should be considered carefully. Cross-sectional imaging combined with a needle biopsy should in all cases provide the correct diagnosis. Management options include radiologic surveillance in asymptomatic patients or surgical resection in symptomatic patients.

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