Improved prognosis in children with intra-abdominal non-Hodgkin's lymphoma following LSA2L2 protocol chemotherapy.

Thirty-five consecutive, previously untreated patients with intra-abdominal non-Hodgkin's lymphoma (IANHL), seen between January 1971 and June 1978, were entered on the LSA2-L2 protocol. Six patients had Stage II disease, 23 Stage III and six Stage IV. The prevalent histologic types were diffuse lymphocytic poorly differentiated (13 patients) and diffuse undifferentiated (13 patients) followed by diffuse histiocytic (5 patients) and diffuse lymphoblastic (2 patients). All patients received LSA2-L2 protocol chemotherapy. Three of 4 patients with gross residual disease following initial surgery, who were seen prior to 1974, received radiation therapy during induction chemotherapy; there were no survivors among these 3 patients. Our treatment plan was revised in 1974 to include a "second-look" laparotomy during the third week of induction chemotherapy for all patients with gross residual intra-abdominal disease following their initial surgery. The disease-free actuarial survival for the total group of 35 patients is 72%. Fifteen of the 26 surviving patients are off therapy and have shown no evidence of recurrence or metastases (median observation time 26 + months). Fifteen of 26 patients seen after 1973 underwent the second laparotomy and only two were found to have residual disease. The LSA2-L2 protocol has significantly improved the disease-free survival rate of children with IANHL, even with widespread intra-abdominal and extra-abdominal disease at diagnosis. Further, the "second-look" laparotomy in patients with large unresectable disease at presentation has proved a useful method of unequivocal evaluation of response, thus eliminating unnecessary extensive abdominal irradiation in many of these children.