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  • 1
    ISSN: 1432-1076
    Keywords: Cancer ; Leukaemia ; Hepatitis ; Hepatitis-C virus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A total of 203 paediatric cancer treatment survivors were tested for serum antibodies against hepatitis-C virus (anti-HCV). Anti-HCV was detected in 41 patients (20.2%) with first generation anti-HCV ELISA. Positive results were confirmed in all samples retested with a second generation ELISA (n=35) and in all but two cases re-analysed by immunoblotting (n=23). Anti-HCV positive children had received significantly more blood product transfusions compared to seronegative patients. In 75 children (32%) chronic liver disease was found. It was defined as an elevation of serum alanine aminotransferase values to a least 2.5 times the upper limit of normal persisting for 6 months or longer. Hepatitis A was never detected, and in 58 children the chronic hepatopathy was unexplained by hepatitits B (non-A non-B chronic liver disease). Of these patients 29 (50%) were seropositive for anti-HCV. Surprisingly, non-A/non-B chronic liver disease was associated with anti-HCV in 14 of 19 solid tumour patients (78.9%), but in no more than 14 of 39 leukaemia and lymphoma patients (35.9%). This phenomenon was not explained by different rates of cytomegalovirus disease and drug toxicity related hepatopathies between the two groups. It may be related to differences of leukaemia/lymphoma compared to solid tumour therapy schedules (differential immuno-suppression and liver toxicity).
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  • 2
    ISSN: 1432-1084
    Keywords: Key words: Children ; Respiratory system ; CT ; Secondary lung neoplasms
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The purpose of this paper is to clarify the distribution of benign vs malignant pulmonary nodules which are seen on spiral CT in children with malignant extra-thoracic solid tumors. Seventy-four children with known solid, extra-thoracic tumors underwent spiral CT of the chest. According to the initial and follow-up (interval 9.2 ± 4.7 months) findings, the children were graded into four groups: I = normal; II = solitary nodule unchanged at follow-up; III = multiple nodules with one or more than one unchanged at follow-up; and IV = solitary or multiple nodules all changed at follow-up. Nodules without change at follow-up were regarded as benign. Forty-nine children did present with normal pulmonary CT exams. In 7 cases solitary pulmonary nodules were found unchanged (group II) at follow-up and in 2 cases (group III) some of the nodules were stationary. Thus, 12 % (9 of 74) presented with at least one pulmonary nodule that did not change at follow-up. Solitary nodules (in groups II and IV) with a diameter 〈 5 mm were in 70 % (7 of 10) unchanged at follow-up and regarded as benign. In children with known solid extra-thoracic tumors at initial presentation, 70 % of solitary nodules ( 〈 5 mm) may be benign. To avoid overstaging, smaller solitary nodules must not automatically be regarded as metastases.
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  • 3
    ISSN: 1432-1076
    Keywords: Ewing tumours ; EWS-FLI-1 fusion transcripts ; Circulating tumour cells ; Tumour biopsy ; Minimal metastatic disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Ewing sarcoma and the closely relate peripheral primitive neuroectodermal tumour, recently referred to as Ewing tumour (ET), are characterised by unique gene rearrangements on chromosome 22. The breakpoints have been cloned and shown to fuse the Ewing sarcoma gene to one of two closely related ETS proto-oncogens, FLI-1 or ERG, which reside on chromosomes 11 and 21, respectively. The rearrangement results in the expression of specific hybrid transcripts which can be detected with high sensitivity by the reverse transcriptase polymerase chain reaction technique (RT-PCR) in primary tumours, blood and bone marrow. We report on a 7-month-old boy with a pelvic Ewing sarcoma in whom circulating tumour cells were identified in the peripheral blood during open tumour biopsy by RT-PCR. However, before and 6 days after surgery no tumour cells could be detected in the peripheral blood. Conclusion The application of RTPCR to monito shedding of tumour cells during surgical intervention will nelp to evaluate if open biopsy potentially contributes to metastatic tumour cell spread.
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  • 4
    ISSN: 1432-1076
    Keywords: Key words Ewing tumours ; EWS-FLI-1 fusion transcripts ; Circulating tumour cells ; Tumour ; biopsy ; Minimal metastatic disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Ewing sarcoma and the closely related peripheral primitive neuroectodermal tumour, recently referred to as Ewing tumour (ET), are characterised by unique gene rearrangements on chromosome 22. The breakpoints have been cloned and shown to fuse the Ewing sarcoma gene to one of two closely related ETS proto-oncogens, FLI-1 or ERG, which reside on chromosomes 11 and 21, respectively. The rearrangement results in the expression of specific hybrid transcripts which can be detected with high sensitivity by the reverse transcriptase polymerase chain reaction technique (RT-PCR) in primary tumours, blood and bone marrow. We report on a 7-month-old boy with a pelvic Ewing sarcoma in whom circulating tumour cells were identified in the peripheral blood during open tumour biopsy by RT-PCR. However, before and 6 days after surgery no tumour cells could be detected in the peripheral blood. Conclusion The application of RT-PCR to monitor shedding of tumour cells during surgical intervention will help to evaluate if open biopsy potentially contributes to metastatic tumour cell spread.
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  • 5
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Subkutane Weichteilknoten ; Differentialdiagnose ; Alveolar soft part sarcoma ; Therapie ; Key words Subcutaneous mass ; Differential diagnosis ; Alveolar soft part sarcoma ; Treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: A slow growing indolent subcutaneous mass can be caused by a variety of benign and malignant soft tissue proliferations, however, it is often the first sign of soft tissue sarcomas. Radiological findings are unspecific and biopsy with histopathological analysis is needed. Method: Three female patients (age 9, 191/12 and 232/12 years) are presented with a slow growing subcutaneous mass, which was shown to be a malignant non-embryonal soft tissue sarcoma (alveolar soft part sarcoma). Results: Alveolar soft part sarcoma (ASPS) is a very rare malignancy. At diagnosis, 2 patients already had metastatic disease. One girl died before therapy, the other one suffers from slowly progressive pulmonary metastases 41/2 years after diagnosis, despite multi-modal treatment approaches. The third patient received polychemotherapy and local irradiation after complete tumor resection and is free of disease 8 months after diagnosis. Conclusion: ASPS is a rare slow growing malignancy with an extraordinary tendency to metastasize. The prognosis depends on wether the tumor is operable and whether there are metastases.
    Notes: Zusammenfassung Fragestellung: Langsam wachsende nicht schmerzhafte subkutane Knoten können durch unterschiedlichste gut- oder bösartige mesenchymale Proliferationen verursacht sein. Da sich hinter diesem Zeichen oft ein Malignom verbirgt und bildgebende Analysen nur selten den Weg zur Diagnose weisen, ist eine bioptische Klärung dringend erforderlich. Methode: Wir berichten über 3 Patientinnen (Alter 9, 191/12 und 231/6 Jahre) bei denen sich hinter einem langsam wachsenden subkutanen Knoten ein nicht embryonales Sarkom (Alveolar soft part sarcoma) verbarg. Ergebnisse: Das Alveolar soft part sarcoma (ASPS) ist ein ausgesprochenes seltenes Malignom. Zum Diagnosezeitpunkt lag bei 2 Patientinnen bereits eine Metastasierung vor. Eine Patientin verstarb noch vor Behandlungsbeginn. Bei der 2. sind Lungenmetastasen trotz multimodaler Therapie 41/2 Jahre nach Diagnosestellung langsam progredient. Die 3. Patientin erhielt nach Resektion eines lokalisierten ASPS am Oberarm postoperativ eine Polychemo- und lokale Strahlentherapie und ist 8 Monate nach der Diagnosestellung ohne Krankheitszeichen. Schlußfolgerung: Das ASPS ist ein langsam wachsender Tumor mit ausgeprägter Metastasierungstendenz. Die Prognose ist im wesentlichen von der Operabilität des Primärtumors und dem Vorliegen von Metastasen abhängig.
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  • 6
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Dyspnoe ; Chronischer Husten ; Kindesalter ; Pleuropulmonales Blastom ; Kombinierte Therapie ; Key words Dyspnea ; Chronic cough ; Childhood ; Pleuropulmonary blastoma ; Combined therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: Chronic cough and thoracic pain, commonly caused by upper airway infections, are frequently seen symptoms in pediatrics. These “benign” symptoms are very seldom caused by malignant disorders. Methods: In two patients (male, age 41/12 and 43/12 years) chronic cough and thoracic pain were due to a malignant pulmonary neoplasm (Pleuropulmonary blastoma). Results: Pleuropulmonary blastoma is a rare malignant primary pulmonary neoplasm in childhood with a poor prognosis. An incomplete resection was performed in both cases. Both patients received postoperative radiotherapy and combination chemotherapy. One boy died, the other one is alive and well more than 6 years after diagnosis without evidence of disease. Conclusion: Our findings indicate that even after incomplete resection subsequent multimodal therapy (adjuvant chemotherapy and radiotherapy) can provide a long term remission.
    Notes: Zusammenfassung Fragestellung: Dyspnoe, chronischer Husten und Brustschmerzen sind häufige Symptome in der pädiatrischen Praxis und werden meist durch banale Infekte der Atemwege verursacht. Nur sehr selten verbirgt sich hinter diesen „gutartigen“ Symptomen eine bösartige Erkrankung. Methode: Wir berichten über 2 Knaben (Alter 4 1/12 und 4 3/12 Jahre) bei denen chronischer Husten und Thoraxschmerzen durch einen bösartigen Lungentumor (pleuropulmonales Blastom) verursacht worden waren. Ergebnisse: Das pleuropulmonale Blastom (PPB) ist ein ausgesprochen seltener, primär intrathorakaler Tumor des Kindesalters mit schlechter Prognose. Bei keinem der beiden Patienten gelang chirurgisch eine vollständige Tumorentfernung. Beide Kinder erhielten postoperativ eine kombinierte Chemo- und Radiotherapie. Ein Knabe verstarb, der 2. Patient ist mehr als 6 Jahre nach der Diagnose ohne Krankheitszeichen. Schlußfolgerungen: Diese Erfahrung zeigt, daß auch nach unvollständiger chirurgischer Tumorentfernung bei konsequenter Anwendung eines multimodalen Therapiekonzepts eine länger anhaltende Remission möglich ist.
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  • 7
    ISSN: 1432-0584
    Keywords: Liposomal amphotericin B ; Granulocytopenia ; Cancer ; Children ; Tolerance
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Conventional amphotericin B (Amph-B) is the drug of choice for treating systemic fungal infections. Recently, a new formulation has become available, encapsulated in liposomes (Amph-lip). This new form of administration was developed in order to lower the acute side effects and to offer the possibility of administering high doses of amphotericin B. Experience with Amph-lip is limited, especially in children. We treated four children with documented systemic fungal infections with Amph-lip and administered it empirically to 12 children. Fifteen of these 16 children were severely granulocytopenic oncologic patients. One 3-month-old baby suffered from systemic candidiasis. Amph-lip was preferred to conventional Amph-B in children with organ dysfunction developing as a consequence of conventional chemotherapy or bone marrow transplantation, after failure of conventional Amph-B to improve a fungal infection, and after adverse drug reactions had occurred. The daily doses of Amph-lip ranged from 1 to 6 mg/kg (median 3 mg/kg), the cumulative doses from 13 to 311 mg/kg (median 75 mg/kg). Acute adverse reactions or organ function abnormalities attributable to Amph-lip did not occur in 402 administrations. Amph-lip has proven to be well tolerated by children in terms of acute toxicity and in the long term. Although large cumulative doses were given, organ function abnormalities attributable to Amph-lip doses were not detected in any of ten long-term survivors over a median observation time of 36 months (range 30–44 months). Amph-lip appears to be a promising alternative antifungal treatment, especially for patients with impaired organ function, when high doses of amphotericin B are necessary.
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  • 8
    ISSN: 1432-0711
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 9
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
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  • 10
    ISSN: 0165-4608
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
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