Prognostic significance of DNA ploidy in childhood astrocytomas.

The ability to divide subsets of children with astrocytoma into prognostic groups is limited because only a few clinical and pathologic variables are available. This study evaluated DNA ploidy as a potential prognostic factor in 30 children with diagnosed gliomas and examined the correlation of flow cytometric analysis to other parameters such as sex, age at diagnosis, histologic grading, localization of tumor, and completeness of surgical resection. Seventeen children with low-grade glioma and 13 with high-grade glioma were retrospectively reviewed; mean age of the patients was 8.2 years, and mean follow-up of the population was 7.6 years. The tumor was localized to the cerebrum in 19 patients, the cerebellum in 7 patients, the brain stem in 3 patients, and the spine in 1 patient. Fourteen patients underwent complete excision and 16 patients underwent partial excision. DNA diploidy was demonstrated in 21 patients and aneuploidy in 9 patients. Twenty children had no evidence of disease and 10 died of disease. Of the patients with diploid tumors, 81% survived, compared to only 33% survival among patients with aneuploid tumors (p < .011). By Cox regression analysis with age, gender, type of excision, grade, location of tumor, and ploidy as independent variables, ploidy was a statistically significant predictor of survival (p = .043). This investigation provides further evidence that flow cytometry may have prognostic value in children with gliomas. Thus, a larger number of tumors can be studied to extend and validate these observations.

[Optic glioma in children with type 1 neurofibromatosis].

During the period 1985-95 we treated 5 girls and 13 boys with optic glioma associated with Type 1 neurofibromatosis (median age 3 years, range 2-10 years). 6 were treated with radiotherapy alone, 1 with surgery and radiation therapy and 1 with chemotherapy in order to postpone irradiation to an older age; 1 is being followed with no therapy. All children are alive, 2-10 years from diagnosis (mean follow-up time 5.4 years). 3 had improvement of vision following therapy and in 4 visual ability stabilized, including the child being followed without treatment. In 1 vision deteriorated despite therapy.

Case report: late aggressive meningioma following prophylactic cranial irradiation for acute lymphoblastic leukaemia.

The clinical, radiological and pathological findings in a 28-year-old female patient who developed aggressive meningioma 20 years after prophylatic cranial irradiation (PCI) for acute lymphoblastic leukaemia (ALL) are described here. Only four cases of late atypical/aggressive meningioma following PCI were detected in a thorough search of the literature. The high cure rate in childhood ALL, attributable to aggressive chemotherapy and PCI, is capable of inducing secondary brain tumour, including aggressive meningioma.

Fibromatosis (desmoid tumor) following radiation therapy for Hodgkin's disease.

Fibromatosis (desmoid tumor) can sometimes develop at sites of previous surgery or previous trauma, but it is very rarely detected at sites of prior irradiation. We report two cases of fibromatosis following radiotherapy for Hodgkin's disease. The fibromatoses, developing in the radiation fields, were detected at the posterior area of the neck 11 years after irradiation in one case and in the peritoneal cavity 3 years after laparotomy and irradiation in the second case. The tumors did not differ clinically or histologically from those seen in patients who did not undergo irradiation. Although rare, fibromatosis should enter the differential diagnosis of masses developing in irradiated fields in patients with treated Hodgkin's disease.