Retroperitoneal sarcomas in children: outcomes from an institution

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.

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.

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.

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.