Retroperitoneal sarcomas in children: outcomes from an institution.

Background: Retroperitoneal sarcomas are uncommon in children and tend to present as large tumors with advanced locoregional involvement of abdominopelvic structures. Surgical control of these tumors remains to be a management challenge. We reviewed our institutional experience with retroperitoneal sarcomas in children.

Materials And Methods: In a retrospective review of charts dating between 1975 and 2005, we identified patients younger than 18 years who were diagnosed with a histologically confirmed retroperitoneal sarcoma. Patients were followed prospectively through clinic visits and mail correspondence. Standard statistical methods were used for comparative, risk, and survival analyses.

Results: Twenty-one children with a mean age at presentation of 9 +/- 1 years were identified. There were more boys than girls (male/female ratio = 1.6). The most common presentations were abdominal mass/distention (76%) and pain (62%). The mean tumor size was 14.2 +/- 1.4 cm, with locoregional involvement in 62% of the patients. The common histologic types were rhabdomyosarcoma (33%) and fibrosarcoma (33%). Seventy-six percent of the patients underwent primary or secondary resection, 71% received neoadjuvant and/or adjuvant chemotherapy therapy, and 38% received radiation therapy. Complete resection was achieved in 48% of the patients, including 3 who required inferior vena cava resection and reconstruction. The 5-year disease-specific survival rates for patients who underwent complete resection and those who underwent incomplete resection were 90% and 36% (P = .018), respectively. For all patients, the mean survival time was 103 +/- 16 months and the 5-year disease-specific survival rate was 62%. Survival was significantly better for patients with low-grade sarcomas than for those with high-grade sarcomas (90% vs 36%, P = .008). Among those who underwent an initial complete resection, 50% had a recurrence at a mean time of 88 +/- 52 months (range = 3-261 months). The 30-day postoperative mortality and morbidity rates were 0% and 24%, respectively; in addition, 14% of the patients experienced long-term complications.

Conclusions: Resection of retroperitoneal sarcomas can be performed safely with minimal morbidity and mortality. Complete resection and low histologic grade are associated with significantly better disease-specific survival.