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A randomized trial of combined modality therapy of childhood non-Hodgkin's lymphoma
708
ABSTRACTS
maintenance phases has resulted in 57 of 75 patients alive and in continuous remission for 2 yr or longer. Twenty-seven patients are now off treatment and doing well. Nine died from therapy complications and 13 patients r e l a p s e d . Randall W. Powell A Randomized Trial of Combined Modality Therapy of Childhood Non-Hodgkin's Lymphoma. S. Murphy and H. O.
Hustu. Cancer 45:630-637, (February), 1980. Between March 1975 and November 1978, 53 males and 16 females between the ages of 2 and 19 yr with nonHodgkin's lymphoma were treated with attempts to cure. Children in stages I-II received three-drug chemotherapy and radiotherapy. Children in stages Ill-IV received the same three-drugs and Adriamycin with or without simultaneous radiotherapy. Eighty-eight percent of children in stages Ill-IV were successfully induced; the 12% who were not had advanced intra-abdominal or CNS disease. This study evaluated the role of staging, the contribution of irradiation in combination with an effective multiple-drug regimen, and the need for better CNS prophylaxis. Whereas staging provides th.e best assessment of the extent of the disease, it does not take into account the different prognoses for B- and T-cell lymphomas. No favorable effect was exerted by radiation on children with stages Ill-IV disease. The combined toxicity of radiotherapy and multiple drug chemotherapy needs to be evaluated. Also, no uniform improvements were demonstrated by giving those patients with CNS disease a combination of cranial irradiation and intrathecal methotrexate. Nevertheless, it is estimated that 65% of the patients in this study will be alive at 2 yr after receiving treatment under these clinical trials, and this represents an improvement.--Jane F. Goldthorn Malignant Taratomas in Infancy and Childhood. M. M .
Woolley. World J Surg 4:39-47 (January), 1980. A review of 137 teratomas from 1941 to 1976 revealed 28 with a malignant component with 16 originating in the sacrococcygeal area, 6 in the ovary, one in the testis, two in the mediastinum and three intracranially. Of these patients 84% have died. Treatment varied as to site with surgical removal of the tumor being the most likely means of disease control. Postoperative therapy would include chemotherapy and radiation therapy in sacrococcygeal lesions. Ovarian tumors usually can be resected and chemotherapy utilized. Peritoneal implants of glial tissue require microscopic evaluation because benign glial tissue usually imports a benign course while malignant glial implants require more aggressive therapy. Testicular lesions are treated by radical orchiectomy in the infant and younger child while lymph node dissection, radiation, and chemotherapy are added in the older patients. One of the commentaries following the paper recommends lymph node dissection in all ages. In the commentary attention is directed towards the repair of the levator musculature after removal of a sacrococcygeal teratoma in order to prevent fecal incontinence problems.Randall W. Powell Neuroblastoma: An Analysis of 160 cases. J. L.
Grosfeld
and R. L. Baehner. World J Surg 4:29-38 (January), 1980.
One hundred sixty patients treated for neuroblastoma from 1948 to 1978 form the basis for this report. Ninetyseven were male and 63 female. Seventy-four patients presented at less than 2 yr of age, 28 between 2 and 3 yr of age and 58 over 3 yr of age. A breakdown by disease stage revealed 5 in stage I, 31 in II, 26 in III, 82 in IV and 16 in stage IV-S. Three tumors originated in the neck, 16 in the mediastinum, 136 in the abdomen and 3 in the pelvis. VMA levels were elevated in 85% of the patients when expressed as /~g VMA/mg creatinine and late in the series 25% of those examined had positive CEA levels. Treatment for stage I lesions was by surgical removal alone while stage II patients underwent resection followed by radiation therapy. Stage III patients underwent resection with subsequent radiation and chemotherapy. Stage IV patients received intensive radiation and chemotherapy with either delayed primary surgical therapy or "second-look" operations to remove tumor. Survival rates by stages were as follows: Stage 1 5 of 5 (100%); I1, 23 of 31 (74%); III, 9 of 26 (34.6%); IV, 8 of 82 (10%); IV-S, 12 of 16 (75%). Patients less than one year of age had 29 of 39 surviving (74.3%) while 15 of 35 (42.8%) survived in the 1-2 age group, 5 of 28 (17.8%) in the 2-3 age group and 8 of 58 (13.7%) in the over 3 age group. Seventy percent of patients responded to chemotherapy and the mean survival time was extended from 3-20 mo but the survival rate was not improved by chemotherapy. The two critical determinants of survival remain the age and stage of the patient at the time of diagnosis.--Randall W. Powell The Management of Rhabdomyosarcoma in Children and
Young Adults. D. M. Hays. World J Surg 4:15-28 (January), 1980. Representing 5%-15% of all malignant solid tumors and 4%-8% of all malignant diseases in children under 15 yr of age in the U.S., rhabdomyosarcoma has undergone improvement in survival rates from 20% to 70% in the past 15 yr. Combined modes of therapy, especially multiple-agent chemotherapy, have increased survival due to control of micrometastases, and residual regional tumor and elimination of established metastatic disease. From 1972-1978, more than 750 patients registered under the Intergroup Rhabdomyosarcoma Study (IRS) have undergone treatment with multiple therapeutic modes including surgery, radiation, and chemotherapy. Findings include the lack of improvement in survival and relapse with irradiation in Group 1 patients (tumor completely resected) and insignificant difference in chemotherapy regimens (2 drug versus 3 drug) in Group II patients. The addition of Adriamycin to VAC did not improve response in Group III (gross tumor left after resection) and Group IV (metastatic disease) patients. As far as site is concerned, orbital and pelvic rhabdomyosarcomas have shown excellent response in all stages while extremity lesions have responded well in Groups I and II. Studies in progress are evaluating the use of chemotherapy and limited resection for the control of pelvic tumors with encouraging results. Evaluation of methods to prevent CNS spread of head and neck lesions, a lethal event usually, could result in further improvement. Orbital lesions respond quite well to only irradiation and chemotherapy with greater than 80% survival rates. Paratesticular lesions with combined
ABSTRACTS
maintenance phases has resulted in 57 of 75 patients alive and in continuous remission for 2 yr or longer. Twenty-seven patients are now off treatment and doing well. Nine died from therapy complications and 13 patients r e l a p s e d . Randall W. Powell A Randomized Trial of Combined Modality Therapy of Childhood Non-Hodgkin's Lymphoma. S. Murphy and H. O.
Hustu. Cancer 45:630-637, (February), 1980. Between March 1975 and November 1978, 53 males and 16 females between the ages of 2 and 19 yr with nonHodgkin's lymphoma were treated with attempts to cure. Children in stages I-II received three-drug chemotherapy and radiotherapy. Children in stages Ill-IV received the same three-drugs and Adriamycin with or without simultaneous radiotherapy. Eighty-eight percent of children in stages Ill-IV were successfully induced; the 12% who were not had advanced intra-abdominal or CNS disease. This study evaluated the role of staging, the contribution of irradiation in combination with an effective multiple-drug regimen, and the need for better CNS prophylaxis. Whereas staging provides th.e best assessment of the extent of the disease, it does not take into account the different prognoses for B- and T-cell lymphomas. No favorable effect was exerted by radiation on children with stages Ill-IV disease. The combined toxicity of radiotherapy and multiple drug chemotherapy needs to be evaluated. Also, no uniform improvements were demonstrated by giving those patients with CNS disease a combination of cranial irradiation and intrathecal methotrexate. Nevertheless, it is estimated that 65% of the patients in this study will be alive at 2 yr after receiving treatment under these clinical trials, and this represents an improvement.--Jane F. Goldthorn Malignant Taratomas in Infancy and Childhood. M. M .
Woolley. World J Surg 4:39-47 (January), 1980. A review of 137 teratomas from 1941 to 1976 revealed 28 with a malignant component with 16 originating in the sacrococcygeal area, 6 in the ovary, one in the testis, two in the mediastinum and three intracranially. Of these patients 84% have died. Treatment varied as to site with surgical removal of the tumor being the most likely means of disease control. Postoperative therapy would include chemotherapy and radiation therapy in sacrococcygeal lesions. Ovarian tumors usually can be resected and chemotherapy utilized. Peritoneal implants of glial tissue require microscopic evaluation because benign glial tissue usually imports a benign course while malignant glial implants require more aggressive therapy. Testicular lesions are treated by radical orchiectomy in the infant and younger child while lymph node dissection, radiation, and chemotherapy are added in the older patients. One of the commentaries following the paper recommends lymph node dissection in all ages. In the commentary attention is directed towards the repair of the levator musculature after removal of a sacrococcygeal teratoma in order to prevent fecal incontinence problems.Randall W. Powell Neuroblastoma: An Analysis of 160 cases. J. L.
Grosfeld
and R. L. Baehner. World J Surg 4:29-38 (January), 1980.
One hundred sixty patients treated for neuroblastoma from 1948 to 1978 form the basis for this report. Ninetyseven were male and 63 female. Seventy-four patients presented at less than 2 yr of age, 28 between 2 and 3 yr of age and 58 over 3 yr of age. A breakdown by disease stage revealed 5 in stage I, 31 in II, 26 in III, 82 in IV and 16 in stage IV-S. Three tumors originated in the neck, 16 in the mediastinum, 136 in the abdomen and 3 in the pelvis. VMA levels were elevated in 85% of the patients when expressed as /~g VMA/mg creatinine and late in the series 25% of those examined had positive CEA levels. Treatment for stage I lesions was by surgical removal alone while stage II patients underwent resection followed by radiation therapy. Stage III patients underwent resection with subsequent radiation and chemotherapy. Stage IV patients received intensive radiation and chemotherapy with either delayed primary surgical therapy or "second-look" operations to remove tumor. Survival rates by stages were as follows: Stage 1 5 of 5 (100%); I1, 23 of 31 (74%); III, 9 of 26 (34.6%); IV, 8 of 82 (10%); IV-S, 12 of 16 (75%). Patients less than one year of age had 29 of 39 surviving (74.3%) while 15 of 35 (42.8%) survived in the 1-2 age group, 5 of 28 (17.8%) in the 2-3 age group and 8 of 58 (13.7%) in the over 3 age group. Seventy percent of patients responded to chemotherapy and the mean survival time was extended from 3-20 mo but the survival rate was not improved by chemotherapy. The two critical determinants of survival remain the age and stage of the patient at the time of diagnosis.--Randall W. Powell The Management of Rhabdomyosarcoma in Children and
Young Adults. D. M. Hays. World J Surg 4:15-28 (January), 1980. Representing 5%-15% of all malignant solid tumors and 4%-8% of all malignant diseases in children under 15 yr of age in the U.S., rhabdomyosarcoma has undergone improvement in survival rates from 20% to 70% in the past 15 yr. Combined modes of therapy, especially multiple-agent chemotherapy, have increased survival due to control of micrometastases, and residual regional tumor and elimination of established metastatic disease. From 1972-1978, more than 750 patients registered under the Intergroup Rhabdomyosarcoma Study (IRS) have undergone treatment with multiple therapeutic modes including surgery, radiation, and chemotherapy. Findings include the lack of improvement in survival and relapse with irradiation in Group 1 patients (tumor completely resected) and insignificant difference in chemotherapy regimens (2 drug versus 3 drug) in Group II patients. The addition of Adriamycin to VAC did not improve response in Group III (gross tumor left after resection) and Group IV (metastatic disease) patients. As far as site is concerned, orbital and pelvic rhabdomyosarcomas have shown excellent response in all stages while extremity lesions have responded well in Groups I and II. Studies in progress are evaluating the use of chemotherapy and limited resection for the control of pelvic tumors with encouraging results. Evaluation of methods to prevent CNS spread of head and neck lesions, a lethal event usually, could result in further improvement. Orbital lesions respond quite well to only irradiation and chemotherapy with greater than 80% survival rates. Paratesticular lesions with combined