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.