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Biomedical subjects

U Göbel

Publications and source records attributed to U Göbel.

At least 19 recordsLinked to original sources
PubMed1995

[Germ cell tumors in children and adolescents].

3.7% of the diagnoses in the Pediatric Tumour Registry (Zentrales Tumor-Register) of the German Society for Pediatric Oncology and Haematology (Gesellschaft für Pädiatrische Onkologie und Hämatologie) concern germ cell tumors. The annual incidence has increased from 0.22 in 1980 to 0.60 per 100,000 children in 1992. The most common localizations are the coccygeal region (25%), followed by the testes (20%), the ovaries (20%) or the brain (20%). Some malignant germ cell tumors tend to secret the tumour markers Alpha-1-Feto-protein and HCG, and may then be diagnosed clinically due to the increased serum or spinal fluid levels. In the cases of extensive tumors growth and secreting intracranial germ cell tumors, a preoperative chemotherapy is favourable. The latest chemotherapy regime containing Cis-Platinum allows us to achieve long term remission in 80% of cases. Risk factors for the regime include histology, localization stage, and incomplete resection of the primary tumors.

Adolescent↗
PubMed1995

Wegener's granulomatosis masquerading as breast cancer.

We describe a patient with Wegener's granulomatosis whose disease presented as pseudotumor of the orbit and a breast mass. Both findings were misinterpreted and errors in diagnosis resulted, despite the availability of rapid and accurate diagnostic tests for this disease. We report this case to emphasize the more unusual presentations of Wegener's granulomatosis.

Breast Neoplasms↗
PubMed1995

Osteosarcoma of the trunk treated by multimodal therapy: experience of the Cooperative Osteosarcoma study group (COSS).

The case histories of all patients with osteosarcoma of the trunk entered into the consecutive studies COSS 80 through COSS 86 of the Cooperative German/Austrian Osteosarcoma Study Group (COSS) were analyzed in order to evaluate their clinical characteristics and the impact of modern neoadjuvant therapy on prognosis. They were compared to those of all patients with extremity osteosarcoma treated according to the same protocols. While tumors of the trunk comprised only 32 (4.8%) of 665 primary classical osteosarcomas, secondary osteosarcomas were much more likely to be located in bones of the axial skeleton (6 of 18, 33%). Patients with primary osteosarcoma of the axial skeleton were older (mean: 20.8 vs. 15.2 years, P < 0.01) and were more likely to present with metastases at diagnosis (34% vs 12%, P < 0.001) than those with primary extremity osteosarcoma. In contrast to extremity tumors, local surgical treatment failure was very common in osteosarcomas of the trunk. Complete tumor removal was achieved in less than half of all evaluable cases. The prognosis of eight patients with localized primary axial osteosarcoma and effective surgical local control was not inferior to that of 483 equally evaluable patients with extremity tumors. In conclusion, while secondary systemic spread of axial osteosarcoma may be avoided in patients treated with multiagent chemotherapy, successful treatment is often barred by primary metastatic disease and inability to control the local tumor site.

Adolescent↗
PubMed1995

Successful liver treatment of a juvenile granulosa cell tumor in a 4-year-old child by regional deep hyperthermia, systemic chemotherapy, and irradiation.

Juvenile granulosa cell tumors (JGCT) of the ovary are rare in children and adolescents. About 90% are diagnosed in early-stage FIGO I with favorable prognosis. More advanced stages (FIGO II-IV) have a poor clinical outcome and chemotherapy alone cannot avoid tumor progression. Regional deep hyperthermia (RHT) induced by microwave technique has been established as an additional modality for treatment of different tumors. However, in cases with liver involvement there are technical problems which have not yet been solved. We report on a 4-year-old child who suffered from diffuse liver metastases 10 months after JGCT of the left ovary. After chemotherapy including ifosfamide, etoposide, and carboplatin in combination with RHT and consolidation radiotherapy, the patient has been in complete remission for 1 year of follow-up. This Case Report indicates the feasibility of combining surgery, chemotherapy, hyperthermia, and radiation therapy (which can also be an effective treatment modality for advanced granulosa cell tumor) for the treatment of liver metastases of JGCT, but does not allow comparisons of these treatments.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1995

12q13, a new recurrent breakpoint in acute non-lymphoblastic leukemia.

The karyotypes of 312 successfully analyzed samples of children with acute non-lymphoblastic leukemia (ANLL), which were sent to us by 72 German hospitals, were examined in order to find new recurrent chromosome abnormalities of possible clinical relevance. Whereas most of the patients had one of the specific aberrations of ANLL or a normal karyotype, random numerical or structural changes were found in 61 children (20%). Four of them showed an abnormality involving band 12q13: t(12;17)(q13;q21), t(12;21)(q13;q21), t(2;12)(p13;q13), and t(5;12)(p11;q13). Despite the fact that FAB subtypes were different (M0, M1 M6, AHL), the blasts of all patients were characterized by immaturity and were difficult to classify. The breakpoint 12q13 might be of clinical importance in ANLL, because the four patients in our study, as well as the 21 patients with this aberration found in the literature, had a very poor prognosis.

Child↗
PubMed1995

Tumefactive megalocytic interstitial nephritis in a patient with Escherichia coli bacteremia.

Megalocytic interstitial nephritis is rare and primarily affects the cortex in an otherwise normal kidney. We recently encountered a patient with Escherichia coli bacteremia and oliguric acute renal failure who died of gram-negative septicemia. At autopsy, this patient's kidneys displayed typical features of megalocytic interstitial nephritis. We were able to perform special stains suggesting that the histiocytic interstitial cells originated from infiltrating macrophages. Our patient illustrates that macrophage proliferation can result in interstitial inflammation sufficiently severe to cause anuric acute renal failure.

Acute Kidney Injury↗
PubMed1995

The cytodiagnosis of meningeal involvement in familial haemophagocytic lymphohistiocytosis.

The cytological appearances of the cerebrospinal fluid (CSF) in two cases of familial haemophagocytic lymphohistiocytosis (FHL) are described. The presence of numerous lymphocytes and immature macrophages in the CSF, some of which showed lymphohistiocytosis, was indicative of meningeal involvement. The appearance of large numbers of immature macrophages indicated rapid deterioration and death within a few weeks. Furthermore, some CSF samples taken at times when the patients were asymptomatic contained suspicious cells, indicating asymptomatic persistence of the meningeal lesions. Thus, cytological examination of CSF can assist with the management of patients with this rare disease.

Cerebrospinal Fluid↗
PubMed1995

Osteosarcoma in a 16-year-old boy with Baller-Gerold syndrome.

We report a 16-year-old growth deficient boy with craniosynostosis, radial hypoplasia and hypoplastic thumbs. These findings are consistent with the autosomal recessively inherited Baller-Gerold syndrome (BGS) which furthermore shows a great variability of concomitant occasional anomalies. At the age of 16 years our patient suffered from an osteosarcoma of the left distal femur. The occurrence of malignancies has not yet been reported in the hitherto described 22 patients with BGS.

Abnormalities, Multiple↗
PubMed1995

Isolated extramedullary relapse in children with acute lymphoblastic leukemia: a comparison between treatment results of chemotherapy and bone marrow transplantation. BFM Relapse Study Group.

The purpose of this study was to determine the therapeutic efficacy of different treatment strategies, namely chemotherapy, allogeneic and autologous bone marrow transplantation (BMT), for extramedullary relapse of acute lymphoblastic leukemia (ALL) in children in second or subsequent remission. Between 1983 and 1993, 165 patients up to 19 years of age with extramedullary relapse of ALL were registered in the multicenter ALL-REZ BFM trials. One hundred and thirty four children received chemotherapy only; 17 children were grafted from HLA-identical sibling donors 152 days (46-392 days) after diagnosis of relapse, and 14 children underwent autologous BMT after a median time of 137 (range 23-300) days. Event-free survival (EFS) at 5 years was 0.47 +/- 0.05 for patients receiving chemotherapy: 0.76 +/- 0.07 for late, 0.33 +/- 0.08 for early and 0.33 +/- 0.07 for very early relapsed patients. Sixty five patients are in complete remission (CR), 61 patients relapsed, 5 died from therapy related complications, 2 patients in CR were lost to follow-up and one patient developed a second malignancy. For patients who had undergone BMT, EFS at 5 years was 0.36 +/- 0.10 without significant difference between autologous BMT (8 of 14 in CR, 6 relapsed) and allogeneic BMT (6 of 17 in CR, 4 died of acute toxicity, and 7 relapsed).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Treatment of malignant testicular tumors in childhood: results of the German National Study 1982-1992.

The German Cooperative Protocol for treatment of testicular germ cell tumors in childhood registered 106 patients from January 1982 through February 1992. Sixty-one patients suffered from yolk sac tumors (YST); 25 patients from differentiated teratomas (TD); 19 patients from malignant teratomas of either intermediate (MTI), undifferentiated (MTU), or trophoblastic type (MTT), and 1 patient from a seminoma. A stratified chemotherapy based on stage and histology was administered in addition to unilateral orchiectomy: Standard chemotherapy consisted of four treatments with vinblastine, bleomycin, and cisplatinum. If viable tumor was suspected after two treatments with standard chemotherapy, a delayed explorative laparotomy was done. There were two options based on the histological findings: In case of complete tumor regression, the standard chemotherapy was continued. In case of incomplete tumor response, patients received a salvage chemotherapy consisting of three treatments with VP 16 (etoposide), ifosfamide, and cisplatinum. In addition three injections with VP 16 were given as a maintenance therapy. The following results were obtained: YST: 59 patients with stage I. Forty-nine patients were followed according to "wait and see" policy. Eight of these needed a delayed standard chemotherapy. The relapse free survival of all 61 patients in 100%. Median observation time is 49 months. TD: Twenty-five patients had stage I. No chemotherapy was given. The relapse free survival is 100%. Median observation time is 48 months. Malignant teratomas (MTI, MTU, MTT): 8 patients had stage I. Three of these received adjuvant chemotherapy and 5 lymphadenectomy without chemotherapy. All patients survived without relapse. Nine patients had stage II and received standard chemotherapy. Four of these patients had a delayed explorative laparotomy leading to a salvage therapy in two patients. All patients survived relapse free. Two patients had stage III. Of these 1 received standard chemotherapy and is well. One patient suffering from MTU stage IIIA died due to candida septicemia during salvage therapy. Median observation time of the entire group is 60 months.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1994

Expression of the CEA gene family members NCA-50/90 and NCA-160 (CD66) in childhood acute lymphoblastic leukemias (ALLs) and in cell lines of B-cell origin.

The carcinoembryonic antigen (CEA) and the classical non-specific cross-reacting antigens (NCAs) belong to the CEA gene family which is part of the immunoglobulin superfamily. In normal hematopoiesis, CEA gene family members (CGMs) have only been reported on cells of myeloid and monocytic origin. In the present study, we analyzed 62 childhood acute lymphoblastic leukemias (ALLs) and seven surface immunoglobulin positive (sig+) B-cell lines for the expression of the CEA family members CEA, NCA-50/90, NCA-95, NCA-160, CGM1 and CGM7. We demonstrated that members of the CEA family were present in 76% of childhood ALLs of B- and T-cell origin. In ALLs of B-cell origin, 82% of the samples expressed at least one CEA subgroup member: 38% NCA-50/90 (CD66c), 31% NCA-160 (CD66a), and 13% both. Six of seven B-cell lines solely expressed NCA-160. In seven ALL of T-cell origin, sole NCA-160 expression was present in 29% of the cases. CEA and CGM1 were not expressed in childhood ALLs or in the sIg+ B-cell lines. In 15 ALLs and seven B-cell lines which could be analyzed for CGM7 expression, the antigen was not detected. NCA-95 was not expressed in 91% of the B-lineage ALLs, in T-lineage ALLs and in the B-cell lines. However, five B-lineage ALLs showed conflicting data on the binding patterns of two, on leukocytes specifically NCA-95 recognizing antibodies suggesting either expression of unknown forms of NCA-95 or NCA-50/90 or of a yet unknown member of the CEA family in these ALL cells. The expression of CEA subgroup members in childhood ALL cells might have prognostic impacts, as an inverse correlation exists between NCA expression on leukemic blasts and the risk factor white blood count at diagnosis.

Adolescent↗
PubMed1993

Simultaneous genotypic and immunophenotypic analysis of interphase cells for the detection of contaminating maternal cells in cord blood and their respective CFU-GM and BFU-E.

Contamination of cord blood (CB) specimens by maternal blood provides a source of cells that may be capable of graft-versus-host reactivity. To confirm the genetic purity of collected CB samples (six samples, volume 110 +/- 21 ml, total nucleated cells 1.22 +/- 0.36 x 10(9)) the HLA-DR beta exon 2 for the noninherited material allele was examined by polymerase chain reaction amplification. No maternal cell contamination was detected in samples of 1 x 10(5) cells. In the case where the mother was homozygous for DR and DQ, the purity of the sample could not be tested by PCR and therefore in situ hybridization on interphase cells with a fluorescein labeled Y- and X-probe in male CB specimens was performed. This sensitive method also revealed no contamination of the CB by maternal white cells. In addition, picked CB-CFU-GM and BFU-E colonies (cultured in hu-SLF, GM-CSF, and Epo) were analyzed by simultaneous genotypic (for Y and X) and immunophenotypic analysis (monoclonal antibodies [MAbs] CD13, CD14, CD2, CD8, CD4, and glycophorin A). This approach permits simultaneous visualization of both the immunophenotype (MAbs, APAAP, red fluorescence) and the genotype (chromosomes, fluorescein isothiocyanate, green fluorescence) within the same cell. In contrast to PCR and restriction fragment length polymorphism, this method has the advantage that the donor-recipient origin of each lymphohematopoietic lineage (i.e., BFU-E, CFU-GM, T cells, B cells) can be determined in sex-mismatched transplantations. Thus confocal scanning laser microscopy is most suitable not only for the detection of contaminating maternal cells, but also for the detection of mixed hematopoietic chimerism after transplantation.

Adult↗
PubMed1992

Des-gamma-carboxyprothrombin (PIVKA II) and plasma vitamin K1 in newborns and their mothers.

Assessments of the vitamin K status in newborns and their mothers by means of des-gamma-carboxy-prothrombin (PIVKA II) measurement have given equivocal results. Part of the variability could be attributed to differences in sensitivity (i.e. the ability to detect small concentrations) and validity (i.e. ability to detect vitamin K deficiency) of the methods applied. None of these methods have yet been validated with respect to plasma vitamin K1. In 22 healthy mother/infant pairs PIVKA II was determined using three different assays including ratio Xa/ecarin (Xa/ec), crossed immunoelectrophoresis (CIE), and an ELISA with a monoclonal antibody (MAB). The results were compared with conventional clotting tests and plasma vitamin K1. The following results were obtained: Cord blood: Clotting tests within age-related normal ranges; PIVKA II detection rates: 0/22 (Xa/ec), 1/22 (CIE), 4/22 (MAB); plasma vitamin K1: undetectable in 20/22. Mothers: Clotting tests all within normal range; PIVKA II detection rates: 1/22 (Xa/ec), 0/22 (CIE), 5/22 (MAB); plasma vitamin K1 (pg/ml) for all mothers (median; range): 186; 55-833; for PIVKA II positive mothers: 213; 59-699. PIVKA II detectability in newborns and mothers was not correlated. The results show an increase in sensitivity for PIVKA II detection in the order of MAB >> CIE > Xa/ec. Due to the very low plasma vitamin K1 at birth, no correlation was possible between cord PIVKA II detectability and plasma vitamin K1. However, in mothers at term PIVKA II MAB appears to be unrelated to the vitamin K status.

Adult↗