Survival and late effects on development of patients with infantile brain tumor.

BACKGROUND: Most infants with brain tumor may have a poor prognosis. The aim of the present study was to retrospectively analyze the survival and outcome with regard to mental and physical development in 11 subjects with brain tumor; these tumors were diagnosed when the patients were under 1 year of age. METHODS: The histological diagnoses of these tumors were astrocytoma, n = 3; pineocytoma, n = 2; teratoma, n = 1; ependymoma, n = 1; atypical teratoid/rhabdoid tumor, n = 1; glioblastoma, n = 1; medulloblastoma, n = 1; and choroid plexus papilloma, n = 1. Surgical resection was performed in eight patients, and adjuvant chemotherapy was administered to all except one patient with choroid plexus papilloma. Radiotherapy was additionally performed for four of the 10 chemotherapy patients. RESULTS: Six patients survived. Among the surviving patients, five were under no treatment for 50-167 months after the diagnosis (median duration, 89 months), while one received chemotherapy for 20 months. Five patients exhibited mental retardation, and one patient experienced normal development after surgical removal of his choroid plexus papilloma. Diencephalic syndrome developed in one patient with pilomyxoid astrocytoma that necessitated hormone replacement therapy, and bodyweight over +2 SD was observed in two patients. The remaining five patients died 11-111 months after diagnosis (median duration, 24 months). CONCLUSION: The prognosis of infantile brain tumor with regard to mortality and developmental outcome remains poor. Furthermore, survivors require comprehensive medical and social support for an extended period.

Establishment and characterization of a novel human desmoplastic small round cell tumor cell line, JN-DSRCT-1.

The exact nature of the desmoplastic small round cell tumor (DSRCT) remains controversial. More detailed analyses might be facilitated by the establishment of permanent DSRCT cell lines. To date, however, no human DSRCT cell line has been reported. In this study, we report the establishment of a new human cell line, JN-DSRCT-1, from the pleural effusion of a 7-year-old boy with pulmonary metastasis from a typical intra-abdominal DSRCT. JN-DSRCT-1 cells were small round or spindle shaped with oval nuclei and have been maintained continuously in vitro for over 190 passages during more than 40 months. Histologic features of the heterotransplanted tumors in severe combined immunodeficiency mouse were essentially the same as those of the original DSRCT, revealing nests or clusters of small round cells embedded in an abundant desmoplastic stroma. Both in vitro and in vivo, the cells exhibited immunopositive reactions for vimentin, desmin, cytokeratins (AE1/AE3 and CAM 5.2), epithelial membrane antigen, neuron-specific antigen, and CD57 (Leu-7). JN-DSRCT-1 cells exhibited a pathognomonic t(11;22)(p13;q12) translocation by cytogenetic analysis. In addition, RT-PCR and sequencing analysis revealed a chimeric transcriptional message of the Ewing's sarcoma gene exon 10 fused to the Wilms' tumor gene exon 8. To our knowledge, this is the first permanent human DSRCT cell line. The JN-DSRCT-1 cell line, which exhibits the unique morphologic and genetic characteristics of DSRCT, will be extremely useful for a variety of important studies such as the pathogenic mechanism, biologic behavior, and therapeutic model of human DSRCT.

[Transfusion of fresh frozen plasma for massive intraoperative bleeding before and after the new guidelines].

"Guidelines on Blood Products Use" published in 1999 recommends restricting the use of fresh frozen plasma (FFP) solely to correct multiple coagulation factor deficiencies. We retrospectively studied the use of FFP in patients with massive intraoperative bleeding before and after publication of the new guidelines. There were 22 patients whose blood loss was more than their circulating blood volume (7% of body weight) in the past two years. The mean blood loss was 1.4 times of the circulating blood volume both before and after publication of the new guidelines. FFP was given to all 22 patients. The mean dose of FFP decreased from 26.8 ml.kg-1 to 17.8 ml.kg-1 after publication of the new guidelines but the difference was not statistically significant. The volume infused was more than that recommended to improve blood coagulation in massive bleeding, i.e., 8-10 ml.kg-1.