Reasonable surgery for thoracic neuroblastoma in infants and children.

ABSTRACT

Neuroblastoma is the most commonly encountered soft tissue malignant tumor of childhood. Over the past 30 years we have treated 180 patients with neuroblastoma. Sixty-five percent had primary abdominal tumors and 20 percent (41 patients) had primary chest tumors. For the 22 patients under the age of 2 years, the 2 year survival rate was 87 percent. There were 19 patients who were 2 years of age or older, and of these only seven patients have survived 2 years after the diagnosis was made. The vast majority of these patients were treated with surgery (debulking type procedure) and postoperative radiation and chemotherapy. Patients with the most differentiated tumors had a remarkably good survival rate, with no deaths. However, the tumors with lesser differentiation did not stratify enough focus to draw conclusions as to survival. Staging correlated the least with survival when compared to age or grading. The 2 year survival rates for patients with Stage I, II, III, IV, and IV-S disease were 75, 82, 100, 17, and 80 percent, respectively. In conclusion, 41 cases of documented primary thoracic neuroblastoma are reviewed, with follow-up from 2 to 27 years (average 9.3 years). We have concluded from this experience that age is the main determining factor influencing survival. Heroic and/or radical surgery is contraindicated in this disease.