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Biomedical subjects

C E Urban

Publications and source records attributed to C E Urban.

18 recordsLinked to original sources
PubMed1997

Neuroblastoma screening in infants postponed after the sixth month of age: a trial to reduce "overdiagnosis" and to detect cases with "unfavorable" biologic features.

BACKGROUND: Encouraged by Japanese reports of the benefits of screening 6 month-old infants for neuroblastoma, a neuroblastoma screening program was introduced in Austria in 1991. However, because of concerns related to "overdiagnosis" by screening at this age, the screening test was performed at a later age. METHODS: From March 1991 to February 1995 neuroblastoma screening was performed on filter paper urine specimens in 100,043 Austrian infants (median age 8.5 months). Primary analysis of urine catecholamines (vanillylmandelic acid and homovanillic acid was performed by use of an E1A method. Questionable or positive results were confirmed by high performance liquid chromatography (HPLC). A double retest was requested following a positive HPLC result. RESULTS: Twenty-one infants were admitted to a hospital following repeatedly elevated values of vanillymandelic acid (VMA) and/or homovanillic acid (HVA). Eleven infants were found to have neuroblastoma (three stage 1, four stage 2B, four stage 3). Treatment consisted of surgery alone with total or subtotal resection in eight cases, surgery and chemotherapy in two cases, and chemotherapy alone in one case. Biologic features were assessed in all tumors excluding ploidy in one case. The majority of the tumors analyzed were near-triploid (9/10), however, two tumors revealed N-myc amplification. CONCLUSIONS: Our results demonstrate that stage distribution and biologic features of neuroblastomas diagnosed by screening at 8.5 months are different from the results of screening at 6 months. Furthermore, the detection of one neuroblastoma among 9,100 screened infants is significantly lower than the incidence of the Japanese screening program. Our results suggest that screening at an age of 7 to 10 months reduces overdiagnosis and may be of more benefit than earlier screening.

Age Factors↗
PubMed1996

Connatal localized neuroblastoma. The case to delay treatment.

BACKGROUND: Spontaneous regression is well documented for a subset of widespread neuroblastomas (Stage 4S) and for localized residual tumors after incomplete resection. Possible spontaneous regression of untreated localized neuroblastoma in infants is frequently discussed, but has very rarely been demonstrated clinically. METHODS: We report four patients with localized neuroblastomas detected early; all were tumors of the adrenal gland. One patient was detected antenatally by ultrasound, the other three tumors were detected incidentally by sonography at age of birth, 1 week, and 7 weeks, respectively. In three patients treatment was delayed in order to await a possible spontaneous regression, and in one patient treatment was delayed due to an uncertain diagnosis. RESULTS: Local tumor growth was observed in three patients, and the tumors were removed 7, 12, and 16 weeks, respectively, after the initial diagnosis of the neuroblastoma. All three patients are free of disease. The fourth patient developed liver metastases 4 weeks after the first suspicion of neuroblastoma. Progressive disease ended in death at the age of 17 months. CONCLUSIONS: None of the four patients showed spontaneous tumor regression. Noninvasive examinations and invasive investigations (in two patients) were unable to predict the tumor's behavior. Based on present knowledge, a general "wait and see" strategy cannot be recommended for early and incidentally detected neuroblastoma patients.

Adrenal Gland Neoplasms↗
PubMed1996

Screening for neuroblastoma in late infancy by use of EIA (enzyme-linked immunoassay) method: 115000 screened infants in Austria.

The aim of this study was to investigate the feasibility of a neuroblastoma screening programme for children in late infancy, based on collaboration of general paediatricians and practitioners in Austria, using the technique of enzyme-linked immunoassay (EIA) for biochemical analyses. Analysis of catecholamine metabolites in spot urine samples by EIA with high performance liquid chromatography as a backup was undertaken. Austrian infants (median age 8.7 months) were screened. Overall compliance was 30%. The EIA method had a high rate (6.7%) of false-positive results. 28 infants were admitted to hospital. In 15 cases, neuroblastoma was found (four stage 1, five stage 2B, six stage 3). The EIA method can be used for neuroblastoma screening, but requires a backup analytical technique in order to avoid unnecessary hospital admissions. The stage distribution and biological features of neuroblastomas diagnosed by screening at a later age are different from those detected by earlier screening. Screening in late infancy might be of more benefit than early screening.

Austria↗
PubMed1996

Multimodal strategies as prerequisite for organ-salvaging tumor surgery.

Curative therapy for solid tumors often requires combined-modality treatment. In the following paper the importance of different strategies as prerequisite for organ salvaging tumor surgery and for successful treatment will be shown. Unfavorable surgical conditions (e.g. site, extent of the tumor) often result in nonresectability or mutilating surgery. Preoperative therapies must try to achieve a maximum tumor shrinkage aiming at attaining resectability and preservation of organ function and life. The exact evaluation of the response to preoperative therapy is important since the degree of the response correlates directly with outcome. Unfavorable response to preoperative therapy means unfavorable prognosis and requires further, more aggressive therapy. A very important parameter in this evaluation is the "histologic response", measured by the degree of tumor devitalization at the time of definitive surgery. However, even in cases with complete clinical and histological remission, complete resection of the tumor after preoperative treatment is essential. Despite good response to preoperative treatment followed by complete resection, therapy must be sufficiently continued after surgery. Preoperative therapy should achieve maximum tumor control, that means total necrosis of all tumor cells to make a complete resection of the devitalized tumor easier and to get the possibility to preserve organ function.

Actuarial Analysis↗
PubMed1996

[5 years neuroblastoma screening in Austria: rate of participation, results and a comparison with other screening areas].

In late 1990 a screening program for the early detection of neuroblastoma in infants was introduced in Austria. The program is performed on a voluntary basis in collaboration with general pediatricians and practitioners. Filter strips for urine collection are distributed to parents of infants aged seven to nine months on the occasion of a routine check up. The samples are sent to the laboratory by parents and analysed for vanillylmandelic acid (VMA) and homovanillic acid (HVA). Between January 1991 and December 1995 125,201 infants were screened. The compliance rate was 26.8% for Austria, but great differences were seen for different regions (65% in Carinthia, 10% in Vorarlberg). 30 children were admitted to hospital for investigation of repeatedly elevated urine catecholamines. A neuroblastoma was identified in 16 cases. In 12 of these cases at least one unfavorable prognostic factor was present (stage > or = 3, elevated LDH, unfavorable histology, N-myc amplification, di- or tetraploidy). Neuroblastoma screening of infants aged more than six months seems to detect predominantly those tumors which are unlikely to regress spontaneously. The observation of one false negative case, however, demonstrates that neuroblastomas which become clinically manifest at a later date may remain undetected by early screening. Possible advantages of shifting screening to a later age and repeated screening are discussed.

Austria↗
PubMed1994

Familial hemophagocytic lymphohistiocytosis associated with disseminated T-cell lymphoma: a report of two siblings.

Two siblings with evidence of disseminated T-cell lymphoma at the time of diagnosis of familial hemophagocytic lymphohistiocytosis (FHL) are reported, an association which has not been described previously. The first child with typical clinical and laboratory features of FHL died shortly after admission, before diagnosis could be established. Retrospective analysis of autoptic tissue revealed marked hemophagocytosis as well as morphological and immunohistochemical features suggestive of disseminated T-cell lymphoma. In the second child, FHL was diagnosed in time. Subsequent histologic investigation of bone marrow biopsies displayed a focal infiltration by T-cell lymphoma. DNA hybridization studies provided evidence of a monoclonal T-cell receptor beta chain gene rearrangement. Following conventional chemotherapeutic induction for FHL, the patient received an allogeneic bone marrow transplant (BMT) from a related healthy donor. Currently, 17 months after BMT, the boy is in unmaintained remission from FHL and T-cell lymphoma. The current pathogenetic concepts for FHL and a possible relationship between T-cell lymphoma and FHL are discussed.

Blotting, Southern↗
PubMed1993

Undifferentiated (embryonal) sarcoma of the liver in childhood. Successful combined-modality therapy in four patients.

BACKGROUND: Undifferentiated (embryonal) sarcoma of the liver is a rare, highly malignant, mesenchymal tumor presenting predominantly in late childhood. Four girls, ages 6-13 years, who were treated with combined-modality regimens are reported. METHODS: In the first and second patients, hemihepatectomy resulted in complete removal of the tumor, and multiple-agent chemotherapy was administered postoperatively. In the third patient, only partial resection could be accomplished initially. By synchronous radiation therapy and chemotherapy, the tumor decreased to an extent that it could be resected completely and was totally devitalized on histologic examination. Postoperative chemotherapy was discontinued after 8 weeks. In the fourth patient, the tumor was not resectable at diagnostic biopsy. Polychemotherapy led to a significant reduction of the tumor size, and resection with clear margins could be performed subsequently. Because histologic necrosis amounted to about 95%, postoperative chemotherapy was also discontinued after 6 weeks. RESULTS: All four patients remain well without evidence of tumor recurrence after 79, 41, 36, and 22 months from diagnosis. CONCLUSIONS: The authors suggest that a multimodal therapeutic regimen should be used in patients with undifferentiated hepatic sarcoma.

Adolescent↗
PubMed1978

[Dimethyl-triazeno-imidazole-carboxamide (DTIC) in combination chemotherapy for childhood neuroblastoma (author's transl)].

Eight children with state III or IV neuroblastoma were treated with courses of chemotherapy consisting of nitrogen mustard (6 mg/m2), vincristine (2 X 1.5 mg/m2), doxorubicin (40 mg/m2) and dimethyl-triazeno-imidazolecarboxamide (850 mg/m2) every three weeks. Three children died after a median survival of 8 (+/-5) months. Five children (62%) have now survived for a medium duration of 21 (+/-16) months without clinical evidence of disease. These results are far better than previously-achieved survival rates at our department.

Child, Preschool↗
PubMed1978

[Systemic and biochemical manifestations of hepatoblastomas (author's transl)].

Among 220 children with malignant solid tumors diagnosed at the University Children Hospital and Department of Pediatric Surgery in Graz six (1,8%) hepatoblastomas were found. All patients were boys. Systemic manifestations may precede the discovery of the abdominal mass and may be important for diagnosis and follow-up.

Carcinoma, Hepatocellular↗
PubMed1977

["Pseudoleukemia" in a newborn infant with trisomy 21].

The increased incidence of leukemia in patients with trisomy 21 is well established. A blood disorder which may be mistaken for acute leukemia may occur in infants with trisomy 21. The authors report a newborn with trisomy 21 and hematologic findings suggesting acute leukemia (hyperleukocytosis, anemia, thrombocytopenia; replacement of bone marrow by blast-like cells). Without treatment spontaneous and complete recovery occurred. Cytochemistry and therapy are discussed in this paper.

Chromosomes, Human, 21-22 and Y↗
PubMedDate not supplied

Partial nephrectomy in well-responding stage I Wilms' tumors: report of three cases.

We report on 3 of 21 stage I Wilms' tumor patients with tumor volumes of 190, 890, and 1300 mL, respectively, who responded dramatically to preoperative chemotherapy according to the Austrian/Hungarian Wilms' Tumor Study 89 by tumor volume reductions to 20%, 23%, and 13%, respectively. Radical resection of the tumors with renal preservation was achieved in all patients. Postoperative studies did not show any functional deficit in the preserved kidney. The patients have been in complete remission for 26, 33, and 60 months, respectively. Our preliminary results indicate that tumor resection should be feasible in patients with good responding stage I Wilms' tumors.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

Treatment of invasive pulmonary aspergillosis in severely neutropenic children with malignant disorders using liposomal amphotericin B (AmBisome), granulocyte colony-stimulating factor, and surgery: report of five cases.

Five children with malignancies developed invasive pulmonary aspergillosis during chemotherapy-induced neutropenia. All patients were treated with liposomal amphotericin B and human recombinant granulocyte colony-stimulating factor. Two patients did not recover from bone marrow aplasia and died from organ-infiltrating fungal invasion. Two patients who recovered from bone marrow aplasia survived after surgery of the pulmonary lesions. The fifth patient had a complete resolution of invasive pulmonary aspergillosis after neutrophil recovery without surgical intervention. We conclude that not only the antifungal treatment but also the recovery of granulocytes are important in localizing invasive forms of Aspergillus infections in patients with profound immunosuppression.

Adolescent↗
PubMedDate not supplied

Portal vein thrombosis in a 17-year-old female adolescent with essential thrombocytosis.

A 17-year-old girl known to suffer from essential thrombocytosis (ET) was admitted with severe abdominal pain that was due to sonographically verified portal and hepatic vein thrombosis. ET is a well-documented disease and portal vein thrombosis has often been reported in adults but not in children and adolescents suffering from ET. Coincidence of ET, urinary tract infection, oral contraception, and cessation of acetylsalicylic acid therapy might be responsible for the early manifestation of portal and hepatic vein thrombosis in our patient.

Adolescent↗