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Long-Term Complications of Patients with Neuroblastoma

Saturday, October 20, 2012: 2:54 PM
Versailles Ballroom (Hilton Riverside)
Beth Rymeski, DO1, Linda Levin, PhD2, Karen Burns, MD3, Rajaram Nagarajan, MD3, Debra Kent, CNP3, Judy Correll, CNP3 and Roshni Dasgupta, MD, MPH4, (1)Department of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, (2)Department of Environmental Health, University of Cincinnati College of Medicine, Cincinnati, OH, (3)Department of Hematology and Oncology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, (4)Pediatric Surgery, Cincinnati Childrens Hospital, Cincinnati, OH

Purpose To describe long-term complications experienced by children who underwent surgery for neuroblastoma

Methods Retrospective review of medical records of patients followed in our long-term survivor clinic (>5 years post-treatment). Fisher’s exact tests were performed to test associations between repeat laparotomy, radiation treatments, and primary surgical procedure.

Results 82 patients were identified in our database as >5 yr survivors of neuroblastoma. The mean age at diagnosis was 18 months (SD +/- 20 months) with a mean follow-up of 16.5 yrs (SD +/- 7.5 yrs, range 5-37 yrs). Initial surgical therapy was thoractomy in 19 (23%), laparotomy with complete resection in 36 (44%), laparotomy with incomplete resection in 13 (16%), and other in 14 (17%). Stage at diagnosis was 1 in 4 patients, 2/2B in 18, 3 in 18, 4 in 30, and 4S in 12.  77% of the patients received at least one chemotherapeutic drug, 39% had at least one course of radiation, and 27% underwent bone marrow transplantation. 11 patients (13.4%) had at least one repeat laparotomy after initial surgery. Repeat laparotomy was performed for bowel obstruction in 6 patients, resection of recurrent tumor in 4 patients, and resection of radiation induced colitis in 1 patient. Repeat laparotomy was performed from 3 months to 30 years after initial surgery with a median of 9 years. The relative risk (RR) of a repeat laparotomy for patients who had at least 1 radiation treatment vs none was 4.2 (p=0.02, 95% CI 1.2-14.5). The type of initial laparotomy did not have an association with need for repeat laparotomy. The median years off treatment was significantly longer for patients who developed a second malignancy (24.9 vs 16 years, p=0.03). Secondary malignancy was diagnosed in 6 patients. 5 of 32 (15.6%) patients treated with radiation developed a second malignancy versus 1 of 50 (2%) patients receiving no radiation (p=0.03, RR 7.8, 95% CI 0.95-64). There was no significant increase in risk of malignancy observed related to total radiation dose received. Horner’s syndrome was seen in 4 of 19 (21%) thoracotomy patients and 1 neck dissection patient. Scoliosis was seen in 9 patients (11%) but was not related to type of surgery (thoracotomy vs laparotomy) or radiation. Other complications observed in the patient cohort included:  hypertension (4), osteochondromas (7), and chronic pain (7)

Conclusion This is the first study to examine long-term effects of survivors who underwent surgical resection for neuroblastoma. Risk of repeat laparotomy is significantly higher than what is described in the surgical literature (4.5% vs 9%). Radiation treatment further increases both risk of repeat laparotomy and development of secondary malignancy. Incidence of Horner’s syndrome was higher than expected. These findings stress the importance of long-term follow-up of neuroblastoma patients.