PubMed · 3078262
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S Schewe. [Lymphoma].. https://pubmed.ncbi.nlm.nih.gov/3078262/
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A neuroblastoma cell line displaying genetically unique features was established from a stage III case of a 20-month-old girl. Southern blotting by the probe pTNB6, which contains exon 1 of the N-myc gene, showed that the primary tumor had in total 4 aberrant bands beside the normal amplified band. The established cell line NB(TU)1 had an aberrant N-myc band (9.0 kb) in addition to the normal band (2.9 kb). Cytogenetic analysis revealed that NB(TU)1 has a composite karyotype composed of at least 7 related karyotypes, which are pseudo-diploid and contain complex chromosomal abnormalities, including translocations, deletions and homogeneously staining regions (HSRs). Such extensive abnormalities were considered to be prominent among known neuroblastoma cell lines, and it was suggested that NB(TU)1 had acquired a certain type of genetic instability. Analysis of N-myc bands in 11 clones of NB(TU)1 showed that the intensity ratio of the normal-sized band (2.9 kb) and the aberrant one (9.0 kb) markedly varied among clones. Moreover, 3 clones showed an additional band with the size of 3.7 kb, which was detectable neither in the parent NB(TU)1 nor in the primary tumor. Thus, NB(TU)1 was shown to be composed of heterogeneous cell components. To further detect such ongoing chromosomal instability, we examined micronuclei formation. NB(TU)1 yielded a larger number of micronuclei than 5 other neuroblastoma cell lines. We conclude that NB(TU)1 has acquired genetic instability detectable by both Southern blotting and cytogenetic analysis.
We established and characterized a new human synovial sarcoma cell line, SN-SY-1, derived from a monophasic fibrous synovial sarcoma excised from the abdominal wall of a 21-year-old man. We maintained the cell line for over 35 passages in vitro. The SN-SY-1 cells in vitro exhibit spindle and polygonal shapes and join to form an epithelial plaque. SN-SY-1 maintained a consistent karyotype: 46,t(X;18)(p11.2;q11.2), Y, t(6;8) (q13-15;q11-13), the same as that of the primary tumor specimen. RT-PCR assay for chimeric SYT-SSX transcripts followed by digestion with restriction enzymes revealed SYT-SSX1 fusion protein. An immunohistochemical study revealed expression of bcl-2 protein in the SN-SY-1 cell line, in vitro and in vivo. However, DNA analysis showed no rearrangement of the bcl-2 gene, and expression of bcl-2 mRNA was low. Why bcl-2 protein was expressed in this synovial sarcoma was not clarified.
BACKGROUND: Despite the excellent prognosis for neuroblastoma 4S (NBL 4S; with S indicating "special"), 10-25% of these patients nevertheless do not survive. Since the first description of this subgroup of disseminated neuroblastoma with a favorable natural outcome, treatment modalities have become milder. Current treatment strategies range from observation with supportive care to full cycles of chemotherapy, radiation therapy, and/or surgical removal of the primary tumor. METHODS: A recent case of NBL 4S seen at the Sophia Children's Hospital led the authors to review their patient charts from 1971 onward, as well as the literature, for the treatment modalities used and the outcome in treated versus nontreated patients. RESULTS: In addition to the presented case and five additional cases from the authors' patient files, the literature contained 113 reported cases. Of a total of 119 cases, 33 patients died, 12 as a result of hepatomegaly with renal impairment and/or respiratory failure. All but 1 of these patients were diagnosed in the first 4 weeks of life. Of the 33 patients who died, 45% progressed to Stage 4 metastatic disease (15 of 33), a finding that appeared to be unrelated to age. N-myc amplification data were available in 30 cases. Seventeen patients had < or = 3 gene copies; 12% of these patients (2 of 17) died. In the N-myc-amplified group of patients with > 3 gene copies, 69% (9 of 13) died and another patient progressed to Stage 4 with short follow-up. CONCLUSIONS: The data presented here suggest an important role for age as a prognostic factor. The very young NBL 4S patient (age < 4 weeks at diagnosis) was at high risk of dying of (respiratory) complications as a result of massive hepatomegaly. In contrast, disease progression to Stage 4 appears to be unrelated to age, but is strongly related to the presence of biologic markers in the tumor. The authors propose a therapeutic approach for very young patients and for those with unfavorable biology.