Library

X
feed icon rss

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Export
  • 1
    Electronic Resource
    Electronic Resource
    No evidence of WT1 involvement in a Burkitt's lymphoma in a patient with Denys–Drash syndrome (1998)
    Springer
    Annals of oncology 9 (1998), S. 627-631 
    Details
    ISSN: 1569-8041
    Keywords: Burkitt's lymphoma ; Denys–Drash syndrome ; WT1
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background: We previously reported the case of a patient affected withDenys–Drash syndrome (DDS), who developed disseminated EBV-relatedBurkitt's lymphoma (BL) after kidney transplantation. Here, we describe themolecular characterisation of the WT1 gene in the constitutional and tumourDNA of this patient. Patients and methods: WT1 exons 2 to 10 were sequenced in constitutionaland tumour DNAs. By Southern blotting the latter was also investigated forthe presence of gene rearrangements. Gene expression analysis in tumourcells was performed by reverse transcriptase-polymerase chain reaction(RT-PCR). Results: A germline missense mutation affecting one of the zinc fingerdomains of the gene, and previously reported in other DDS cases, wasobserved. No alterations of the constitutionally wild-type WT1 allele and noexpression of the gene were observed in BL cells. A small group of BLs fromother paediatric patients showed a variable expression of WT1. Conclusions: Our findings indicate that WT1 is unlikely to be involved inthe onset of BL in our case. However, a possible role of the gene in at leasta subset of these lymphoproliferative diseases may be suggested.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1008248905766
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 2
    Electronic Resource
    Electronic Resource
    Management of medulloblastoma and ependymoma in infants: a single-institution long-term retrospective report (2000)
    Springer
    Child's nervous system 16 (2000), S. 15-20 
    Details
    ISSN: 1433-0350
    Keywords: Key words Infantile brain tumors ; Childhood brain radiotherapy ; Usefulness of chemotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  To reduce the sequelae from CNS irradiation (RT), 16 children younger than 3 years with medulloblastoma-PNET (13 cases) and ependymoma (3 cases) were treated between 1987–1993 according to different postsurgical chemotherapy (CT) programs. None of these patients presented with metastases. Eleven patients were rendered disease-free by surgery, while 5 had residual tumor. Adjuvant therapy depended on patients’ age, postsurgical status and parents’ consent to radiotherapy (RT). Nine of the 16 infants remained alive in continuous complete remission from the first neoplasm (median follow-up 7 years). Three of them had been treated with CT alone and 6 with combined CT+RT (posterior fossa 4, whole CNS 2). Seven patients relapsed a median of 13 months after diagnosis, and all 7 of them died of their disease. Despite the omission of RT in 6 of the 16 patients and administration of only focal RT in 8 of the 16, the outcome of this series was satisfactory. Local failure (in 5/7 patients) was the major problem, despite the high dose of RT used in 2 of these 5. In 4 of 6 evaluable children school performance was satisfactory. One child in whom the entire CNS was irradiated developed glioblastoma multiforme 120 months after the first diagnosis of medulloblastoma.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00007279
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group (2000)
    Springer
    Annals of oncology 11 (2000), S. 1445-1449 
    Details
    ISSN: 1569-8041
    Keywords: alveolar soft part sarcoma ; pediatric tumors ; soft tissue sarcoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background:Alveolar soft part sarcoma (ASPS) is a rare malignanttumor and little is known about its clinical features and management. Wereport on a series of 19 pediatric patients managed over 20 years. Patients and methods:Primary conservative surgery was performedin all patients and was radical in nine, non-radical in three; seven patientsunderwent biopsy alone (3 unresectable tumors, 4 metastatic disease). In twocases radical surgery was performed after primary chemotherapy. Radiotherapywas delivered to 8 patients, chemotherapy to 15. Results:After a median follow-up of 74 months, the five-yearsurvival was 80% for the whole series, 91% for patients withlocalized disease, 100% for patients with tumor ≤5 cm, and31% for those 〉5 cm; 16 of 19 patients were alive (12 of 12 withgrossly-resected tumor in first continuous remission). Chemotherapy achievedtwo partial remission among seven evaluable patients. Conclusions:Pediatric ASPS has a more favorable prognosis thanits adult counterpart. In this series, tumor size correlates with metastaticdisease at onset and is the major factor influencing survival. Surgery is themainstay of therapy. The effectiveness of adjuvant therapy remains to beestablished, though radiotherapy may be advisable in cases of inadequatesurgery.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1026579623136
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...