- Email: [email protected]
Treatment of neuroblastoma with vitamin B.12
ABSTRACTS
tained most of the injuries. A comparison of statistical data for children's injuries show football in Seattle to have less injuries than children get in day-to-day living.--Anthony H. Alter.
NERVOUS SYSTEM GAMMA-CISTERNOGRAPHY IN INFANTS AND
CHILDREN. R. Oberson. Schweiz. Med. Wschr. 100:867-874, 1970. Cisternography with 100 #Ci iodated serum-albumin was performed in 15 normal children and 34 pathological cases, in the age group of 2 months to 15 years. In the normal, movement and absorption of CSF are very rapid. Data obtained from pathological cases are manifold: (1) In the case of diffusely reduced leptomeningeal pericerebral permeability or local circulatory impairment at important crossroads such as incisural notch or optochiasmatic cistern, either constant or transitory ventricular contamination may be observed. This apparently reversed flow of CSF could be accounted for by an increased or newly developed absorptive function of the ependyme, and this phenomenon constitutes the main indication for surgical treatment of hydrocephalus. (2) If leptomeningeal permeability is only slightly and/or diffusely impaired, the bulk flow of CSF, i.e., absorption of some of its constituents, appears to be slowed. In this situation the patient should be followed at monthly intervals, in order not to miss the opportunity of surgical correction. (3) Absence of any apparent bulk flow would indicate an obstruction of the fourth ventricle foramina. (4) Gammacisternography is also indicated in cases where CSF leakage is suspected. Here the examination precisely locates the fistula (direct proof of which is given by the picture on the scan) or shows contamination of the adjacent tissues and fluids (indirect proof of fistula) .--M. Bettex.
693
of a Pudenz-Heyer valve in a case of congenital hydrocephalus, a boy aged 3 9/12 years developed a severe nephrotic syndrome complicated by macrohematuria, arterial hypertension and renal failure. Staphylococcus albus was present in the shunt valve. Renal biopsy revealed subacute glomerulonephritis. With immunohistochemical procedures precipitates of IgM, IgG and complement were demonstrated in the glomerula. After removal of the shunt all pathological renal findings disappeared except for slight residual proteinuria.--M. Bettex.
NEOPLASMS CHEMOTHERAPY, RADIOTHERAPY, AND HEPATIC LOBECTOMY FOR HEPATOBLASTOMA IN AN INFANT; REPORT OF A SURVIVAL. R. E.
Hermann and D. Lousdate. Surgery 68: 383-388 (August), 1970.
A hepatoblastoma in the right lobe of the liver of a fifteen month old boy at surgical exploration was deemed nonresectable. Vincristine, Cytoxin, and Cobalt 60 teletherapy resulted in a decrease in size of the tumor mass, the appearance of calcification, and a thriving child. Improvement was so dramatic that 8 months later (at 23 months of age) a right hepatic lobectomy was uneventfully carried out. Three years later the child remains well without evidence of any recurrent or residual tumor. This case is reported because of the unusual beneficial response of hepatoblastoma to radiation and chemotherapy.--William K. Sieber. TREATMENT
OF
NEUROBLASTOMA WITH
VITAMIN B.12. M. J. S. Langman. Arch. Dis. Child. 45:385-387 (June), 1970.
This is a report of a Medical Research Council working party investigating the value of vitamin B.12 in the treatment of neuroblastoma. While most people had abandoned B. 12 as a useful treatment for neuroblastoma the report on the clinical course of 134 paDIFFUSE GLOMERULONEPHRITIS DUE TO tients is of some importance. Twenty-six of INFECTED VENTRICULO-ATRIAL SHUNT these patients were unsuitable for analysis. ("SHUNT-NEPHRITIS"). U. G. Stauffer, A . Sixty-five had received treatment with vitaCsomor, H. J. Pliiss and W. H. Ititzig. min B.12 at some time during their illness Schweiz. Med. Wschr. 100:1288-1291, and 43 had no such treatment. Four of the 1970. treated group, however, had received the vitamin only by mouth, a treatment which Three years after successful implantation
694 was claimed to be ineffective and these were therefore transferred to the control group. To avoid bias, those patients who received vitamin B.12 as a late secondary treatment were excluded and the main comparison was restricted to 35 whose treatment had been started less than 1 month after the diagnosis had been made and 47 who received either no vitamin B or only oral treatment. Only nine ( 8 % ) of the whole group were still alive at the time of the last enquiry; these included four ( 9 % ) of the 47 who had not received vitamin B.12 and two ( 6 % ) of the 35 who had received early treatment with vitamin B. 12. In addition three ( 18 % ) were still alive of the 17 who had been given vitamin B.12 at uncertain dates, but even if all this group had properly been included in the early vitamin treated group the survival rate in that group would have been raised to 10 per cent, which would still have been immaterially different from the group who did not receive any vitamin B.12.--James Lister. CONGENITAL WILMS' TUMOR: CASE REPORT. E. S. Wright. Brit. J. Urol. 4 2 : 2 7 0 272 (June), 1970. A case of congenital nephroblastoma is reported. Treatment was by nephrectomy alone and at 30 months after operation the child is well. Histologically, the tumour was composed of dense mesenchymal tissue but mature tubular structures and foci of cartilage were also present. Because of the high survival rate with these cases, treatment should be by nephrectomy without irradiation or chemotherapy. The good prognosis may be a reflection of the high incidence of well differentiated tumours.---J. H. Johnston. STAGING IN RELATION TO TREATMENT OF NEPHROBLASTOMA WITH ACTINOMYCIN D. Clare Hilton and Jean W. Keeling. Brit. J. Urol. 42:265-269 (June), 1970. Seventy-four cases of nephroblastoma seen at G r e a t Ormond Street Hospital are reviewed. Four neonatal cases were excluded since the histology was atypical. Three groups were recognized according to the stage of the disease. Stage 1. The tumour was limited to the kidney and was removed in its entirety. All 25 cases in this group had nephrectomy and
ABSTRACTS all but one had postoperative irradiation to the kidney bed. Sixteen patients ( G r o u p A ) had Actinomycin D and 9 ( G r o u p B) did not. Thirteen G r o u p A children are well 8 months to 6 years after treatment. Seven G r o u p B patients are well 20 months to 9 years later. Stage 2. Local extension beyond the kidney, for example to the para-aortic glands, to the renal vein or to the perirenal tissues had occurred. Of the 23 children in this stage, 20 had nephrectomy and three had biopsy only. All had postoperative irradiation. Fifteen ( G r o u p A ) had Actinomycin D; eight of these have died. Eight (Group B) had no chemotherapy; six have died. Stage 3. Distant metastases were present. Treatment of the 12 children in this stage was very varied. Three are alive from 2 8 9 years to 5 years. Histology. Of the tumours in Stage 1 cases, 13 were differentiated and 12 were undifferentiated. In Stage 2 the corresponding proportions were 6 : 17 and in stage 3, 2 : 9. The authors reaffirm that the extent of the disease at the time of diagnosis is of prime prognostic importance. They consider that treatment with Actinomycin D should be based on accurate clinical staging. There is no indication that the drug should be used in Stage 1 cases as part of the initial treatment. Actinomycin D is indicated postoperaively in Stage 2 cases. Repeated courses are more effective than a single course. A dosage of 60 #g/kg. is given after operation and before radiotherapy. Subsequently, 75 /tg/ kg. is given at three monthly intervals up to I5 m o n t h s . - - / . H. Johnston. [Another important factor is the age of the patient at the time of discovery and treatment.--Ed.]
TOTAL PROSTHETIC TRANSPLANTATION OF THE iNFERIOR VENA CAVA, WITH VENOUS DRAINAGE RESTORATION OF THE ONE REMAINING KIDNEY ON THE GRAFT, SUCCESSFULLY PERFORMED ON A CHILD WITH WILMS' TUMOR. L. Sarti. Surgery 67:851855 ( M a y ) , 1970. Resection of a right pulmonary metastasis and total replacement of the infrahepatic veda cava and distal left renal vein by a Y-shaped corrugated tubular Teflon graft after en bloc right nephrectomy for a huge Wilms' tumor involving the veda cava in a
tained most of the injuries. A comparison of statistical data for children's injuries show football in Seattle to have less injuries than children get in day-to-day living.--Anthony H. Alter.
NERVOUS SYSTEM GAMMA-CISTERNOGRAPHY IN INFANTS AND
CHILDREN. R. Oberson. Schweiz. Med. Wschr. 100:867-874, 1970. Cisternography with 100 #Ci iodated serum-albumin was performed in 15 normal children and 34 pathological cases, in the age group of 2 months to 15 years. In the normal, movement and absorption of CSF are very rapid. Data obtained from pathological cases are manifold: (1) In the case of diffusely reduced leptomeningeal pericerebral permeability or local circulatory impairment at important crossroads such as incisural notch or optochiasmatic cistern, either constant or transitory ventricular contamination may be observed. This apparently reversed flow of CSF could be accounted for by an increased or newly developed absorptive function of the ependyme, and this phenomenon constitutes the main indication for surgical treatment of hydrocephalus. (2) If leptomeningeal permeability is only slightly and/or diffusely impaired, the bulk flow of CSF, i.e., absorption of some of its constituents, appears to be slowed. In this situation the patient should be followed at monthly intervals, in order not to miss the opportunity of surgical correction. (3) Absence of any apparent bulk flow would indicate an obstruction of the fourth ventricle foramina. (4) Gammacisternography is also indicated in cases where CSF leakage is suspected. Here the examination precisely locates the fistula (direct proof of which is given by the picture on the scan) or shows contamination of the adjacent tissues and fluids (indirect proof of fistula) .--M. Bettex.
693
of a Pudenz-Heyer valve in a case of congenital hydrocephalus, a boy aged 3 9/12 years developed a severe nephrotic syndrome complicated by macrohematuria, arterial hypertension and renal failure. Staphylococcus albus was present in the shunt valve. Renal biopsy revealed subacute glomerulonephritis. With immunohistochemical procedures precipitates of IgM, IgG and complement were demonstrated in the glomerula. After removal of the shunt all pathological renal findings disappeared except for slight residual proteinuria.--M. Bettex.
NEOPLASMS CHEMOTHERAPY, RADIOTHERAPY, AND HEPATIC LOBECTOMY FOR HEPATOBLASTOMA IN AN INFANT; REPORT OF A SURVIVAL. R. E.
Hermann and D. Lousdate. Surgery 68: 383-388 (August), 1970.
A hepatoblastoma in the right lobe of the liver of a fifteen month old boy at surgical exploration was deemed nonresectable. Vincristine, Cytoxin, and Cobalt 60 teletherapy resulted in a decrease in size of the tumor mass, the appearance of calcification, and a thriving child. Improvement was so dramatic that 8 months later (at 23 months of age) a right hepatic lobectomy was uneventfully carried out. Three years later the child remains well without evidence of any recurrent or residual tumor. This case is reported because of the unusual beneficial response of hepatoblastoma to radiation and chemotherapy.--William K. Sieber. TREATMENT
OF
NEUROBLASTOMA WITH
VITAMIN B.12. M. J. S. Langman. Arch. Dis. Child. 45:385-387 (June), 1970.
This is a report of a Medical Research Council working party investigating the value of vitamin B.12 in the treatment of neuroblastoma. While most people had abandoned B. 12 as a useful treatment for neuroblastoma the report on the clinical course of 134 paDIFFUSE GLOMERULONEPHRITIS DUE TO tients is of some importance. Twenty-six of INFECTED VENTRICULO-ATRIAL SHUNT these patients were unsuitable for analysis. ("SHUNT-NEPHRITIS"). U. G. Stauffer, A . Sixty-five had received treatment with vitaCsomor, H. J. Pliiss and W. H. Ititzig. min B.12 at some time during their illness Schweiz. Med. Wschr. 100:1288-1291, and 43 had no such treatment. Four of the 1970. treated group, however, had received the vitamin only by mouth, a treatment which Three years after successful implantation
694 was claimed to be ineffective and these were therefore transferred to the control group. To avoid bias, those patients who received vitamin B.12 as a late secondary treatment were excluded and the main comparison was restricted to 35 whose treatment had been started less than 1 month after the diagnosis had been made and 47 who received either no vitamin B or only oral treatment. Only nine ( 8 % ) of the whole group were still alive at the time of the last enquiry; these included four ( 9 % ) of the 47 who had not received vitamin B.12 and two ( 6 % ) of the 35 who had received early treatment with vitamin B. 12. In addition three ( 18 % ) were still alive of the 17 who had been given vitamin B.12 at uncertain dates, but even if all this group had properly been included in the early vitamin treated group the survival rate in that group would have been raised to 10 per cent, which would still have been immaterially different from the group who did not receive any vitamin B.12.--James Lister. CONGENITAL WILMS' TUMOR: CASE REPORT. E. S. Wright. Brit. J. Urol. 4 2 : 2 7 0 272 (June), 1970. A case of congenital nephroblastoma is reported. Treatment was by nephrectomy alone and at 30 months after operation the child is well. Histologically, the tumour was composed of dense mesenchymal tissue but mature tubular structures and foci of cartilage were also present. Because of the high survival rate with these cases, treatment should be by nephrectomy without irradiation or chemotherapy. The good prognosis may be a reflection of the high incidence of well differentiated tumours.---J. H. Johnston. STAGING IN RELATION TO TREATMENT OF NEPHROBLASTOMA WITH ACTINOMYCIN D. Clare Hilton and Jean W. Keeling. Brit. J. Urol. 42:265-269 (June), 1970. Seventy-four cases of nephroblastoma seen at G r e a t Ormond Street Hospital are reviewed. Four neonatal cases were excluded since the histology was atypical. Three groups were recognized according to the stage of the disease. Stage 1. The tumour was limited to the kidney and was removed in its entirety. All 25 cases in this group had nephrectomy and
ABSTRACTS all but one had postoperative irradiation to the kidney bed. Sixteen patients ( G r o u p A ) had Actinomycin D and 9 ( G r o u p B) did not. Thirteen G r o u p A children are well 8 months to 6 years after treatment. Seven G r o u p B patients are well 20 months to 9 years later. Stage 2. Local extension beyond the kidney, for example to the para-aortic glands, to the renal vein or to the perirenal tissues had occurred. Of the 23 children in this stage, 20 had nephrectomy and three had biopsy only. All had postoperative irradiation. Fifteen ( G r o u p A ) had Actinomycin D; eight of these have died. Eight (Group B) had no chemotherapy; six have died. Stage 3. Distant metastases were present. Treatment of the 12 children in this stage was very varied. Three are alive from 2 8 9 years to 5 years. Histology. Of the tumours in Stage 1 cases, 13 were differentiated and 12 were undifferentiated. In Stage 2 the corresponding proportions were 6 : 17 and in stage 3, 2 : 9. The authors reaffirm that the extent of the disease at the time of diagnosis is of prime prognostic importance. They consider that treatment with Actinomycin D should be based on accurate clinical staging. There is no indication that the drug should be used in Stage 1 cases as part of the initial treatment. Actinomycin D is indicated postoperaively in Stage 2 cases. Repeated courses are more effective than a single course. A dosage of 60 #g/kg. is given after operation and before radiotherapy. Subsequently, 75 /tg/ kg. is given at three monthly intervals up to I5 m o n t h s . - - / . H. Johnston. [Another important factor is the age of the patient at the time of discovery and treatment.--Ed.]
TOTAL PROSTHETIC TRANSPLANTATION OF THE iNFERIOR VENA CAVA, WITH VENOUS DRAINAGE RESTORATION OF THE ONE REMAINING KIDNEY ON THE GRAFT, SUCCESSFULLY PERFORMED ON A CHILD WITH WILMS' TUMOR. L. Sarti. Surgery 67:851855 ( M a y ) , 1970. Resection of a right pulmonary metastasis and total replacement of the infrahepatic veda cava and distal left renal vein by a Y-shaped corrugated tubular Teflon graft after en bloc right nephrectomy for a huge Wilms' tumor involving the veda cava in a