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Postoperative radiation therapy for residual Wilms' Tumor. Review of group III patients in the national Wilms' Tumor study
270
ABSTRACTS NEOPLASMS
The Changing Management of Nephroblastema. A. W. Wilkinson. J. Roy. Coll. Surg. Edinb. 21:120-129 (March) 1976. This is described as an educational paper and is not a report of new work or new figures on the condition. The history of the treatment of the t u m o r is traced from 1815 up to and including the description of the current trial, which is being organised by the Medical Research C o u n cil Working Party on E m b r y o n a l T u m o r s in Childhood. The clinical features, staging, and management of the tumor, including operation and chemotherapy are described. Three illustrative cases are detailed and recently published survival figures in N o r t h America and The Hospital for Sick Children, London, are quoted. A. J. Dougall
Wilms' Tumor: Natural History and Prognostic Factors. A Retrospective Study of 248 Cases Treated at the Institut-Gustave-Raussy 19521967. J. Lemerle, F. Tournade, R. Gerard-Marchant, R. Flamant, D. Sarrazin, F. Flamant, M. Lemerle, S. Jundt, J. Zucker, and O. Schweisguth. Cancer 37:2557-66, (May) 1976. This is a statistical analysis of a series of 248 children with nephroblastoma, treated at the major pediatric cancer center in France. Therapeutic modalities employed are largely outdated, but a n u m b e r of additional features are of special interest. These patients were treated by nephrectomy, radiotherapy (frequently preoperative), and actinomycin D, usually a single course. The relapse rate in all cases was 54~. The three major prognostic factors which were identified were patient age, stage, and histologic pattern. Age appeared to be a factor only as it was related to stage and histologic type. A high relapse rate and poor survival was found in patients who had what was termed "radiotherapy induced changes," found in 15~ of patients who received preoperative radiotherapy. In these patients, the t u m o r histology was so distorted by radiotherapy that diagnosis was barely possible. The survival rate in that segment of the total group which could be analyzed for prognosis was 55~. N o patient developed a recurrence (local or distant) more than 54 m o n t h s following nephrectomy, and 96~o of recurrences occurred within 24 m o after initial surgery. N o patient died who survived 57 m o following nephrectomy. Daniel M. Hays
Preoperative Versus Postoperative Radiotherapy, Single Versus Multiple Courses of Actinamycin D, in the Treatment of Wilms' Tumor-Preliminary Results of a Controlled Clinical Trial Conducted the International Society of Pediatric Oncalogy (S.I.O.P.). J. Lemerle, P. A. Voute, M. F. Tournade, J. F. M. Delemarre, B. Jereb, L. Ahlstrom, R. Flamant, and R. Gerard-Marchant. Cancer 38: 647-54, (August) 1976.
The S.I.O.P. is an organization of 43 pediatric institutions in Europe, the Near East, and Africa that conduct cancer trials with c o m m o n protocols. This study, conducted between September 1971 and October 1974, registered 398 patients, 195 of which were eligible for radomization. Although the significance of this study is diminished by the results of subsequent trials in the U.S. (National Wilms' T u m o r Study), some features remain of considerable interest. In the initial radiotherapy study, patients were randomized between those who received both preoperative a n d postoperative radiotherapy vs. those who received only postoperative radiotherapy of the same general volume. N o significant difference in survival or recurrence free survival was noted, but the rate of gross rupture at surgery was significantly different, i.e., 3 patients in the group receiving preoperative radiotherapy vs. 20 patients in the group receiving only postoperative radiotherapy. This was said to produce increased morbidity. The rate of misdiagnosis in the preoperative irradiation group was 4~o. The second trial concerned the influence of single vs. multiple courses of actinomycin D, which was similar to a study carried out by Childrens Cancer Study G r o u p in the U.S. between 1969 and 1972. It is of interest that in this trial, no significant difference was found in the recurrence-free survival rates 5 4 ~ and 5 8 ~ (or the survival rate 8 2 ~ and 8 6 ~ in these two groups). T h e comparable U.S. study showed a highly significant difference between these two groups, and there is no explanation of this disparity in results. Daniel M. Hays
Postoperative Radiation Therapy for Residual Wilms' Tumor. Review of Group III Patients in the National Wilms' Tumor Study. M. Tefft, G. J. D'Angio, and W. Grant. Cancer 37:2768-72 (June) 1976.
This is one of a series of articles describing the results of National Wilms' T u m o r Study I. It concerns patients in the N W T S in G r o u p III, those in which there was residual t u m o r con-
ABSTRACTS
fined to the abdomen following nephrectomy (or laparotomy). Included were patients who (a) had been biopsied before nephrectomy or whose tumor ruptured before or during surgery; (b) had had implants on peritoneal surfaces; (c) were seen to have lymph node involvement, beyond the abdominal periaortic chain, and finally, (d) were in a group in which complete resection was not possible because of local infiltration. Following surgery, Group IIl patients received radiotherapy and were randomized into three groups receiving either (a) actinomycin D, (b) vincristine, or (c) actinomycin D and vincristine. Fifty-eight patients were included in Group Ill, including 40 in which it was felt that the residual tumor was confined to the renal fossa. The latter group of patients were originally scheduled to receive whole abdominal irradiation, but as the long-range effects of this type of therapy became realized during the course of the study, the area irradiated w~s reduced to a flank field, including the complete vertebral bodies in many. Thus, of the 40 patients in whom the disease was felt to be localized to the renal fossa, 25 received radiation to the renal fossa only, 9 received total abdominal irradiation, and 6 total abdominal radiation with a "boost" to the renal fossa. Five of these patients (5/40) developed recurrence within the abdominal cavity. In two of these, however, the recurrence was in the contralateral kidney. The one "true" abdominal occurrence developed in a patier~t who received total abdominal radiotherapy plus a " b o o s t " to the renal fossa. In contrast, 18 patients were felt to have disease probably involving peritoneal cavity, and received total abdominal radiation. Three of these patients developed recurrence within the abdomen. It is the conclusion o f the National Wilms' Tumor Study that local radiation of the renal fossa would appear to be sufficient treatment for the disease believed to be limited to that region, including cases of local "spill." Whole abdominal radiation is recommended for gross diffuse peritoneal contamination with tumor cells.--Daniel M. Hays Skeletal Alterations Following Irradiation for Wilms' Tumor, with Particular Reference to Scoliosis and Kyphosis, E. J. Riseborough, S. L. Grabias, R. h Burton and N. Jaffe. J. Bone Joint Surg. 58A:526-536 (June) 1976. Eighty-one surviving patients with nephrectomy for Wilms' tumor with subsequent irradia-
271 tion therapy were studied. Twenty-eight percent of the patients developed vertebral growth arrest lines as early as 6 mo postirradiation. Sixtyseven of the patients developed irregularity of their end-plates with altered vertebral height and trabecular patterns. Sixteen patients developed narrowing and breaking of the vertebral bodies. All patients showed some failure of development of the vertebral body. Fifty-seven patients developed structural scoliosis with a tendency to early rigidity and an average mean curve of 21 ~ by the Cobb measurement. Twentyone patients developed structural kyphosis associated with the scoliosis. Other skeletal abnormalities were seen. The younger the patient and the greater the radiation dose, the more the x-ray changes in bony development were seen. Treatment measures were discussed. Anthony H. Alter Congenital Neuroblastoma with Paraplegia. A. Moschos and D. Anagnostakis. HeN. paed. Acta. 30:521-523 (April) 1976. A case of congenital neuroblastoma presenting with paraplegia in a newborn infant is described. The tumor was situated in the lumbar spine and was removed by laminectomy on the 6th day of life. Treatment was completed with vincristine. There was no recurrence within 2 yr, but flaccid paralysis and anesthesia of both legs are still present. A review of the literature shows that the prognosis is good in infants as far as the tumor is concerned, but that the paraplegia is irreversible.--C. Jaussi-Bovet Benign and Malignant Teratoma in Children: Analysis of 85 Patients. J. L. Grosfeld, T. V. N. Ballantine, D. Lowe, and R. L. Baehner. Surgery 80:297-305 (September) 1976. The 85 infants and children with teratomas seen at the James Whitcomb Riley Hospital at Indianapolis from 1960-1975 are the basis of this report. Statistically there were 58 (68~) o/ girls and 27 (32/o) boys. The location of the tumor was sacrococcygeal in 55 (64.8~), mediastinal in 10 (11.7~o), gonadal in 10 (11.7~), presacral in 4 (4.8}/o), retroperitoneal in 3 (3.5~), and cervical in 3 (3.5~). Eighteen or 21.2~ were malignant. The peculiarities of presentation and management and prognosis of teratomas occurring in each location are discussed. The sacrococcygeal teratomas were the largest group, 41 of 55 being seen in the first month of life; only 1 (2.4~o) of these was malignant. Ten (70~) of
ABSTRACTS NEOPLASMS
The Changing Management of Nephroblastema. A. W. Wilkinson. J. Roy. Coll. Surg. Edinb. 21:120-129 (March) 1976. This is described as an educational paper and is not a report of new work or new figures on the condition. The history of the treatment of the t u m o r is traced from 1815 up to and including the description of the current trial, which is being organised by the Medical Research C o u n cil Working Party on E m b r y o n a l T u m o r s in Childhood. The clinical features, staging, and management of the tumor, including operation and chemotherapy are described. Three illustrative cases are detailed and recently published survival figures in N o r t h America and The Hospital for Sick Children, London, are quoted. A. J. Dougall
Wilms' Tumor: Natural History and Prognostic Factors. A Retrospective Study of 248 Cases Treated at the Institut-Gustave-Raussy 19521967. J. Lemerle, F. Tournade, R. Gerard-Marchant, R. Flamant, D. Sarrazin, F. Flamant, M. Lemerle, S. Jundt, J. Zucker, and O. Schweisguth. Cancer 37:2557-66, (May) 1976. This is a statistical analysis of a series of 248 children with nephroblastoma, treated at the major pediatric cancer center in France. Therapeutic modalities employed are largely outdated, but a n u m b e r of additional features are of special interest. These patients were treated by nephrectomy, radiotherapy (frequently preoperative), and actinomycin D, usually a single course. The relapse rate in all cases was 54~. The three major prognostic factors which were identified were patient age, stage, and histologic pattern. Age appeared to be a factor only as it was related to stage and histologic type. A high relapse rate and poor survival was found in patients who had what was termed "radiotherapy induced changes," found in 15~ of patients who received preoperative radiotherapy. In these patients, the t u m o r histology was so distorted by radiotherapy that diagnosis was barely possible. The survival rate in that segment of the total group which could be analyzed for prognosis was 55~. N o patient developed a recurrence (local or distant) more than 54 m o n t h s following nephrectomy, and 96~o of recurrences occurred within 24 m o after initial surgery. N o patient died who survived 57 m o following nephrectomy. Daniel M. Hays
Preoperative Versus Postoperative Radiotherapy, Single Versus Multiple Courses of Actinamycin D, in the Treatment of Wilms' Tumor-Preliminary Results of a Controlled Clinical Trial Conducted the International Society of Pediatric Oncalogy (S.I.O.P.). J. Lemerle, P. A. Voute, M. F. Tournade, J. F. M. Delemarre, B. Jereb, L. Ahlstrom, R. Flamant, and R. Gerard-Marchant. Cancer 38: 647-54, (August) 1976.
The S.I.O.P. is an organization of 43 pediatric institutions in Europe, the Near East, and Africa that conduct cancer trials with c o m m o n protocols. This study, conducted between September 1971 and October 1974, registered 398 patients, 195 of which were eligible for radomization. Although the significance of this study is diminished by the results of subsequent trials in the U.S. (National Wilms' T u m o r Study), some features remain of considerable interest. In the initial radiotherapy study, patients were randomized between those who received both preoperative a n d postoperative radiotherapy vs. those who received only postoperative radiotherapy of the same general volume. N o significant difference in survival or recurrence free survival was noted, but the rate of gross rupture at surgery was significantly different, i.e., 3 patients in the group receiving preoperative radiotherapy vs. 20 patients in the group receiving only postoperative radiotherapy. This was said to produce increased morbidity. The rate of misdiagnosis in the preoperative irradiation group was 4~o. The second trial concerned the influence of single vs. multiple courses of actinomycin D, which was similar to a study carried out by Childrens Cancer Study G r o u p in the U.S. between 1969 and 1972. It is of interest that in this trial, no significant difference was found in the recurrence-free survival rates 5 4 ~ and 5 8 ~ (or the survival rate 8 2 ~ and 8 6 ~ in these two groups). T h e comparable U.S. study showed a highly significant difference between these two groups, and there is no explanation of this disparity in results. Daniel M. Hays
Postoperative Radiation Therapy for Residual Wilms' Tumor. Review of Group III Patients in the National Wilms' Tumor Study. M. Tefft, G. J. D'Angio, and W. Grant. Cancer 37:2768-72 (June) 1976.
This is one of a series of articles describing the results of National Wilms' T u m o r Study I. It concerns patients in the N W T S in G r o u p III, those in which there was residual t u m o r con-
ABSTRACTS
fined to the abdomen following nephrectomy (or laparotomy). Included were patients who (a) had been biopsied before nephrectomy or whose tumor ruptured before or during surgery; (b) had had implants on peritoneal surfaces; (c) were seen to have lymph node involvement, beyond the abdominal periaortic chain, and finally, (d) were in a group in which complete resection was not possible because of local infiltration. Following surgery, Group IIl patients received radiotherapy and were randomized into three groups receiving either (a) actinomycin D, (b) vincristine, or (c) actinomycin D and vincristine. Fifty-eight patients were included in Group Ill, including 40 in which it was felt that the residual tumor was confined to the renal fossa. The latter group of patients were originally scheduled to receive whole abdominal irradiation, but as the long-range effects of this type of therapy became realized during the course of the study, the area irradiated w~s reduced to a flank field, including the complete vertebral bodies in many. Thus, of the 40 patients in whom the disease was felt to be localized to the renal fossa, 25 received radiation to the renal fossa only, 9 received total abdominal irradiation, and 6 total abdominal radiation with a "boost" to the renal fossa. Five of these patients (5/40) developed recurrence within the abdominal cavity. In two of these, however, the recurrence was in the contralateral kidney. The one "true" abdominal occurrence developed in a patier~t who received total abdominal radiotherapy plus a " b o o s t " to the renal fossa. In contrast, 18 patients were felt to have disease probably involving peritoneal cavity, and received total abdominal radiation. Three of these patients developed recurrence within the abdomen. It is the conclusion o f the National Wilms' Tumor Study that local radiation of the renal fossa would appear to be sufficient treatment for the disease believed to be limited to that region, including cases of local "spill." Whole abdominal radiation is recommended for gross diffuse peritoneal contamination with tumor cells.--Daniel M. Hays Skeletal Alterations Following Irradiation for Wilms' Tumor, with Particular Reference to Scoliosis and Kyphosis, E. J. Riseborough, S. L. Grabias, R. h Burton and N. Jaffe. J. Bone Joint Surg. 58A:526-536 (June) 1976. Eighty-one surviving patients with nephrectomy for Wilms' tumor with subsequent irradia-
271 tion therapy were studied. Twenty-eight percent of the patients developed vertebral growth arrest lines as early as 6 mo postirradiation. Sixtyseven of the patients developed irregularity of their end-plates with altered vertebral height and trabecular patterns. Sixteen patients developed narrowing and breaking of the vertebral bodies. All patients showed some failure of development of the vertebral body. Fifty-seven patients developed structural scoliosis with a tendency to early rigidity and an average mean curve of 21 ~ by the Cobb measurement. Twentyone patients developed structural kyphosis associated with the scoliosis. Other skeletal abnormalities were seen. The younger the patient and the greater the radiation dose, the more the x-ray changes in bony development were seen. Treatment measures were discussed. Anthony H. Alter Congenital Neuroblastoma with Paraplegia. A. Moschos and D. Anagnostakis. HeN. paed. Acta. 30:521-523 (April) 1976. A case of congenital neuroblastoma presenting with paraplegia in a newborn infant is described. The tumor was situated in the lumbar spine and was removed by laminectomy on the 6th day of life. Treatment was completed with vincristine. There was no recurrence within 2 yr, but flaccid paralysis and anesthesia of both legs are still present. A review of the literature shows that the prognosis is good in infants as far as the tumor is concerned, but that the paraplegia is irreversible.--C. Jaussi-Bovet Benign and Malignant Teratoma in Children: Analysis of 85 Patients. J. L. Grosfeld, T. V. N. Ballantine, D. Lowe, and R. L. Baehner. Surgery 80:297-305 (September) 1976. The 85 infants and children with teratomas seen at the James Whitcomb Riley Hospital at Indianapolis from 1960-1975 are the basis of this report. Statistically there were 58 (68~) o/ girls and 27 (32/o) boys. The location of the tumor was sacrococcygeal in 55 (64.8~), mediastinal in 10 (11.7~o), gonadal in 10 (11.7~), presacral in 4 (4.8}/o), retroperitoneal in 3 (3.5~), and cervical in 3 (3.5~). Eighteen or 21.2~ were malignant. The peculiarities of presentation and management and prognosis of teratomas occurring in each location are discussed. The sacrococcygeal teratomas were the largest group, 41 of 55 being seen in the first month of life; only 1 (2.4~o) of these was malignant. Ten (70~) of