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J Tröger

Publications and source records attributed to J Tröger.

99 records · Page 6Linked to original sources
PubMedDate not supplied

Neuroblastoma preoperatively treated as nephroblastoma: does inadequate therapy worsen the prognosis?

BACKGROUND: The current standard in the treatment of nephroblastoma is preoperative chemotherapy based on radiological appearance. After subsequent surgical removal few tumours proved histologically to be neuroblastoma. We asked whether initial chemotherapy according to nephroblastoma trials would change the prognosis for those neuroblastoma patients. RESULTS: Out of 1603 patients registered in the German neuroblastoma trials, 29 patients (1.8 %) have preoperatively been treated according nephroblastoma protocols. Advanced stages (11 stage 3, 12 stage 4) were dominant. Diagnostic work up of those patients revealed elevation of catecholamine metabolites in only 39 % (compared to 80 % of the control patients) and mIBG uptake in only 71 % (compared to 89 % of the control patients). Elevation of NSE was observed in 92 % of patients (control group 72 %). Patients with preoperative nephroblastoma treatment were older than the patients of the control group. Risk factors like MYCN amplification or elevation of LDH were more often detected. The outcome of the patients with preoperative chemotherapy according nephroblastoma trials was worse than that of the control group, but risk group adapted survival analysis revealed no disadvantage. CONCLUSION: The prognosis of children with neuroblastoma tumours, which have been radiologically classified as nephroblastoma, is inferior compared to the prognosis of patients without preoperative nephroblastoma therapy. The difference appears to be associated rather with more unfavourable biology than with the element "preoperative chemotherapy".

Adolescent↗
PubMedDate not supplied

Results of the SIOP 93-01/GPOH trial and study for the treatment of patients with unilateral nonmetastatic Wilms Tumor.

BACKGROUND: The treatment of Wilms Tumor is integrated into clinical trials since the 1970's. In contrast to the National Wilms Tumor Study Group (NWTSG) the SIOP trials and studies largely focus on the issue of preoperative therapy to facilitate surgery of a shrunken tumor and to treat metastasis as early as possible. PATIENTS AND METHODS: In the SIOP 93-01/GPOH trial and study 1 020 patients with a newly diagnosed renal tumor were registered. 847 of them had a histological proven Wilms Tumor, of whom 637 were unilateral localized, and 173 tumors had an other histology [40 congenital mesoblastic nephroma (CMN), 51 clear cell sarcoma (CCSK), 24 rhabdoid tumor (RTK) and 58 other tumors]. Preoperative chemotherapy in benign tumors was given to 1.3 % of the patients. The main objective of the trial was the randomized question, if the postoperative two drug chemotherapy for stage I in intermediate risk or anaplasia can be reduced from conventional 3 courses to an experimental 1 course without loss of efficacy. RESULTS: 519 patients with unilateral nonmetastatic Wilms did receive preoperative chemotherapy. The histology in this group of patients was of intermediate risk in 469 (90 %) patients, 14 (3 %) tumors were low risk and 36 (7 %) high risk. The stage distribution of the tumors was stage I in 315 (61 %), stage II N- in 126 (24 %), stage II N+ in 25 (5 %) and stage III in 36 (7 %) patients. In 17 (3 %) patients the tumor stage remained unclear. Tumor volume was measured in 487 patients before and in 402 after preoperative chemotherapy. The median tumor volume did shrink from 353 to 126 ml. The amount of volume reduction depends on the histological subtype. The event free survival (EFS) after 5 years was 91 % for all patients with unilateral Wilms tumor without distant metastasis. Randomisation was done in 43.7 % for stage I patients and there was no difference in EFS for both treatment arms (90 versus 91 %). The EFS is identical for patients with stage I and II N- (0.92), as well as for stage II N+ and III (0.82). The tumor volume after chemotherapy is a prognostic factor for intermediate risk tumors with the exception of epithelial and stromal predominant tumors. These two subtypes often present as large tumors, they do not shrink during preoperative chemotherapy but they still have an excellent prognosis. On the other hand the prognosis of patients with blastemal predominant subtype after preoperative chemotherapy is worse than in any other patient group of intermediate risk tumors. There are less blastemal predominant tumors compared to primary surgery, but they are chemotherapeutic resistant selected by the preoperative chemotherapy. CONCLUSION: Patients with unilateral Wilms tumor without metastasis have an excellent prognosis. The post-operative chemotherapy in stage I can be reduced to 4 weeks without worsening treatment outcome. The reduction of the tumor volume could be identified as a helpful marker for stratification of post-operative treatment. Post-chemotherapy blastemal predominant subtype of Wilms tumor has to be classified as high risk tumor. Focal anaplasia has a better prognosis than diffuse anaplasia and will be classified as intermediate risk tumor.

Adolescent↗
PubMedDate not supplied

[Diagnostic value of imaging procedures in nephroblastoma before preoperative chemotherapy: initial results].

According to the international SIOP 9 study preoperative chemotherapy for patients between 6 months and 16 years old is a major component of the therapeutic management of Wilms' tumour. Prior to treatment, the diagnosis is established by diagnostic imaging alone, without biopsy. This study was therefore undertaken to verify the accuracy of diagnostic imaging in the German GPO study group. Between July 1988 and November 1990, 156 patients with known histology were registered in the study centre. Of these patients, 67% (105/156) received preoperative chemotherapy. The diagnosis was initially established by US, TVP and CT, and in some cases MRT without prior biopsy. Of these preoperatively treated patients, 92.4% (97/105) had Wilms' tumour or one of its variants. Five patients had a different malignant tumour. Three cases, i.e. 2.8% of the preoperatively treated patients or 1.9% of all registered patients with known histology, had benign tumours of the kidney. Morphologically, the Wilms' tumour was revealed by a characteristic inhomogeneity in sonography and the CT scan. The inhomogeneity in the CT scan increased following injection of contrast medium. Intratumoral bleeding and cystic areas were observed frequently in the native scan. Calcifications were seen in 5% of the cases. The predictive value of pretherapeutic diagnostic imaging was good enough to justify instituting chemotherapy without diagnostic biopsy. It is still difficult in diagnostic imaging to differentiate the very rare benign cystic nephroma from malignant nephroblastoma. Intravenous pyelography proved to be the best method for distinguishing extrarenal from renal tumours.

Child↗
PubMedDate not supplied

[Prenatal sonographic diagnosis of a case of Desbuquois familial osseous dysplasia].

A rare form of osteodysplasia could be diagnosed by prenatal sonographic screening. Characteristic for this rare type of dysplasia, described only in 8 cases by Desbuqois and Piussan so far, is a severe dwarfism, generalized muscle hypotonia, mental retardation, asynchronized ossification, osteoporosis and considerable desaxiations of tubular bones and articulations. Radiologically, polydactylia and especially an increase in size of the trochanter minor can be observed. In the case described, this autosomal recessive hereditary disease had already been diagnosed for the first child of this consanguineous couple, which had died at 7 weeks after birth. During the second pregnancy of this patient the malformation could be diagnosed by ultrasound in the 24th week of gestation and the pregnancy was terminated. Autopsy and fetogram showed the malformations typical for this rare dysplasia.

Bone and Bones↗
PubMedDate not supplied

[Incidence of typical nephrocalcinosis ultrasound findings in premature infants during enteral calcium and phosphate administration].

Supplementation of calcium and phosphate is recommended in nutrition of low birth weight infants to ensure a physiological development of postnatal bone mineralisation. To investigate whether high dose calcium supplementation increases the risk of renal calcification in preterm infants, serial ultrasound examinations were performed in 30 preterm infants (gestational age 29.5 (26-35) weeks; birth weight 1382 (610-2010) g) before, during and after oral calcium and phosphate supplementation. Total calcium input was on average 3.1 mmol/kg/d, total phosphate input on average 2.1 mmol/kg/d). All children showed normal kidney ultrasound before entering the study. During mineral supplementation three children developed hyperechoic renal medullary pyramids, the typical ultrasound pattern of nephrocalcinosis. Supplementation was stopped immediately and pathologic ultrasound patterns disappeared in all 3 children.

Calcium, Dietary↗