Retrospective
study on amplification of N-myc and c-myc genes in pediatric solid tumors
and its association with prognosis and tumor differentiation.
Lab Invest. 1988 Sep;59(3):321-7.
DNA was
extracted from formalin-fixed and paraffin-embedded tissues of 85 patients
with pediatric malignant solid tumors which had been resected at surgery
or obtained at autopsy during a 24-year period. The tumors examined
included 25 rhabdomyosarcomas, 12 Wilms' tumors, 10 hepatoblastomas and 37
neuroblastoma group tumors. Neuroblastoma group tumors were subclassified
into 25 neuroblastomas and 12 ganglio neuroblastomas among which 6
composite ganglio neuroblastomas were included. Sample blocks were
selected from both tumors and normal tissues in the majority of cases. We
were able to reliably detect N- and c-myc gene amplification in tumor DNA
by dot blot-hybridization. The N-myc gene showed approximately from 3- to
500-fold amplification in 19 of 33 cases of stage IV neuroblastoma group
tumor. All of these 33 patients had been intensively treated with
chemotherapy and/or radiotherapy. The c-myc was amplified 8-fold in 1 case
of rhabdo myosarcoma, but neither N-myc nor c-myc was amplified in any
cases of Wilms' tumor or hepatoblastoma. We retrospectively examined the
association among N-myc gene amplification, prognosis, and histologic
subtype in 33 patients with stage IV neuroblastoma group tumors. The
survival of the patients with N-myc gene amplification was shorter than
that of the patients without amplification of N-myc (p less than 0.05).
There was no significant difference in prognosis between the 2 histologic
subtypes; neuroblastoma and ganglioneuroblastoma, and the cases of tumors
with amplified N-myc showed shorter survivals for each subtype (p less
than 0.05). In every case of neuroblastoma group tumor, the copy number of
the N-myc gene was the same among primary site and multiple metastatic
tumors, even when the lesions showed differences in histologic subtype
like neuroblastoma and ganglioneuroblastoma.
Solid tumors in
the child. New advances and the importance of histological
sub-classifications. II. Tumors of the peripheral nervous
system, soft tissue, liver and pancreas.Ann
Pathol. 1988;8(2):126-35.
According
to immunohistochemical, ultrastructural features of neural
cells, and identical 11; 22 chromosome translocation, at least
some extra osseous Ewing's sarcoma, as well as the malignant
small cell tumor of the thoracopulmonary region (Askin's
tumor) are actually classified as peripheral neuroepitheliomas.
Embryonal rhabdomyosarcoma, including the botryoid variant, is
now, when treated with appropriate chemotherapy, a tumor of
relatively favorable histology. Its prognosis is still
primarily related to clinical stage and location of tumor. The
alveolar subtype of rhabdomyosarcoma (including its solid
variant) has a less favorable prognosis. Hepatoblastoma
(epithelial or mixt variants) have the same long term
survival, also mainly related to stage. Pancreatoblastoma is a
tumor with well differentiated cytopathology and prolonged
course, compared with other pancreatic tumors. |
