K. Marulanda1, A. Marzinsky2, U. N. Maduekwe1, J. Cooper2, A. Hayes-Jordan1,2 1University of North Carolina,Department Of Surgery,Chapel Hill, NORTH CAROLINA, USA 2University of North Carolina,Division Of Pediatric Surgery,Chapel Hill, NORTH CAROLINA, USA
Introduction: Peritoneal metastatic disease is rare and often fatal in children. It most commonly occurs in tumors originating within the abdominopelvic cavity. Novel local control treatment strategies have been applied but prognosis remains poor with relapse rates of 70%. We have previously demonstrated the use of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) to treat select pediatric patients. Here, we present results from our pediatric CRS-HIPEC program and examine outcomes in patients with disseminated peritoneal disease treated with CRS-HIPEC.
Methods: We performed a retrospective study of children undergoing CRS-HIPEC for peritoneal disease at a tertiary referral center from July 2018 to June 2020. Primary outcomes were relapse free survival and overall survival (OS). Median OS was compared to OS in adult sarcoma patients with peritoneal metastases identified within the National Cancer Database (NCDB).
Results: A total of 21 patients (1 to 21 years) diagnosed with various sarcomas, as well as gastrointestinal and gynecological malignancies originating from the abdomen, retroperitoneum or pelvis were included. The most common malignancies were desmoplastic small round cell tumors (DSRCT) (43%), rhabdomyosarcomas (14%) and ovarian granulosa cell tumors (10%). Complete cytoreduction of gross disease was achieved in 81% of patients. Systemic chemotherapy was administered to 91% of patients, and 48% of patients received radiation before and/or after surgery. Complications were most commonly wound infections (25%), including one patient that required a wound vac, and urinary tract infections (19%) with one patient requiring ureteral stent placement for hydroureteronephrosis. The median follow-up period was 3.2 (2.0 – 7.1) months, during which 29% of patients had recurrence of disease. Of these, 33% had DSRCT. Median time to relapse was 3.3 (2.0 – 5.9) months after surgery. Recurrence was usually isolated to an extra-abdominal location (50%), although 33% of cases had an isolated intra-abdominal location and 17% had a combined intra and extra-abdominal component. There were no fatalities during the follow up period. In contrast, NCDB results showed that 16% (n=101) of adults with peritoneal metastases from abdominopelvic sarcomas died within 90 days of definitive surgery. None of these patients were treated with CRS-HIPEC and only 41% received any form of chemotherapy.
Conclusion: In this case series, we demonstrate that CRS-HIPEC is associated with improved overall survival in pediatric patients with peritoneal metastatic disease. Further analysis with a larger patient population is necessary to determine which disease characteristics are most responsive to this aggressive treatment approach.