Background: We describe the presentation, diagnostic pathway, and management of 28 patients with benign retroperitoneal schwannomas.
Methods: A prospectively kept soft-tissue tumor database was reviewed to identify patients who presented to the Royal Marsden Hospital with retroperitoneal schwannomas.
Results: From 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.
Conclusions: An 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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