[Intracranial, intrarachindial ependymomas. V. Ependymomas of the posterior cerebral fossa].
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[Intracranial, intrarachindial ependymomas. VI. Intraspinal ependymomas].
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[Intracranial intrarachindial ependymomas. III. Radiotherapy of ependymomas].
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[Intracranial, intrarachindial ependymomas. IV. Subtentorial intracranial ependymomas].
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Ependymomas and ependymoblastomas in children.
The authors analyze histologically verified cases of ependymoma and ependymoblastoma (malignant ependymoma) occurring in children in Connecticut from 1935 to 1973. Of the 488 central nervous system tumors diagnosed in that period, 44 (9%) of the 467 intracranial neoplasms and five (24%) of the 21 intraspinal tumors were of ependymal origin. An increase in the incidence of ependymomas was noted since the mid-1950's. The mean ages at diagnosis of ependymomas and ependymoblastomas were 5.6 and 5.0 years respectively. The male to female ratio was 0.6:1 for ependymomas and 1.7:1 for ependymoblastomas. Epencymomas were found above and below the tentorium with similar frequency; however, viturally all of the epencymoblastomas occurred supratentorially. Presenting symptoms and physical findings were reviewed. A significant difference (p less than 0.05) was noted in the seizure rates of supratentorial ependymomas (9%) and ependymoblastomas (38%). A significantly increased survival (p less than 0.05) was associated with supratentorial ependymal neoplasms relative to infratentorial from 42 months following diagnosis onward. Contrary tp the reports of no clinical difference between ependymomas and ependymoblastomas, children with supratentorial ependymomas were noted to have a significantly longer survival (p less than 0.05) than those with similarly situated ependymoblastomas, with the difference noted from 18 months following diagnosis onward. The children treated by operation and irradiation had a significantly greater survival (p less than 0.05) than those treated by other methods; furthermore, with this treatment, longer survivals were noted in the ependymoma patients as compared to those with epencymoblastomas. This difference became significant (p less than 0.05) at 27 months after diagnosis. Operative mortality decreased from 40% to 17% in the last decade of the study as compared to the previous decade. Steroid therapy may have contributed to this decreased operative mortality, but it had no statistically significant effect on length of survival. The clinical course of intracranial ependymal neoplasms in adults and children was compared and appeared to be essentially the same.
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Predicting the recurrence of ependymomas from the bromodeoxyuridine labeling index.
The usefulness of histopathological grading in predicting the prognosis of patients with ependymomas is controversial. To clarify the discrepancy between the histological malignancy and the prognosis of these tumors, we estimated the proliferative potential of 32 intracranial and intraspinal ependymomas and correlated the findings with the clinical behavior. Each patient received an intraoperative infusion of bromodeoxyuridine (BUdR, 200 mg/m2 i.v.) before tumor removal; the BUdR labeling index (LI), or percentage of BUdR-labeled cells, was determined immunohistochemically in excised specimens. The mean BUdR LI (+/- SD) of intracranial malignant ependymomas was 4.1 +/- 2.8%. Nonmalignant intracranial and intraspinal ependymomas and subependymomas had mean LIs of 1.5 +/- 0.9%, 1.1 +/- 0.3%, and less than 1%, respectively. Overall, 44% of the tumors recurred. There were no statistically significant differences in the recurrence rates of intracranial and intraspinal ependymomas, including subependymomas (43% and 44%, respectively), or of intracranial ependymomas with LIs greater than 1.0% and less than 1.0% (67% and 44%, respectively). However, the early recurrence rate (within 24 months after treatment) of tumors with LIs greater than 1.0% was higher than that of tumors with LIs of less than 1.0% (100% vs. 25%, P less than 0.05). The BUdR LI also showed a statistically significant inverse correlation with the time to recurrence. These findings indicate that BUdR LI reflects the proliferative potential of individual ependymomas and can be used to help predict the recurrence and estimate the prognosis of these tumors.
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Ependymoma: a follow-up study of 101 cases.
One hundred and one patients with histologically confirmed ependymomas were studied over a 22-year period. Choroid plexus papilloma and sub-ependymoma were not included. About half of the tumors were intracranial, with the majority of these infratentorial. The intraspinal tumors were equally divided between intramedullary and the "cauda" group. The majority of the intracranial tumors occurred in children, while almost all the intraspinal tumors were in adults. The histologic classification consisted of "typical ependymoma" (cellular, papillary and myxopapillary patterns) and "anaplastic ependymoma". The intracranial and intramedullary tumors showed a predominantly cellular pattern, while the myxopapillary type was found only in the "cauda" group. The histology seems to be of limited value in assessing the prognosis in an individual patient with ependymoma. The postoperative prognosis was poor in the intracranial tumors, although radiotherapy increased the survival time without affecting the eventual fatal outcome. The prognosis in the intraspinal group was much better, with three-fourths of the patients living for at least 10 years. No patient with an anaplastic tumor survived for more than 6 years.
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3D genome mapping identifies subgroup-specific chromosome conformations and tumor-dependency genes in ependymoma.
Ependymoma is a tumor of the brain or spinal cord. The two most common and aggressive molecular groups of ependymoma are the supratentorial ZFTA-fusion associated and the posterior fossa ependymoma group A. In both groups, tumors occur mainly in young children and frequently recur after treatment. Although molecular mechanisms underlying these diseases have recently been uncovered, they remain difficult to target and innovative therapeutic approaches are urgently needed. Here, we use genome-wide chromosome conformation capture (Hi-C), complemented with CTCF and H3K27ac ChIP-seq, as well as gene expression and DNA methylation analysis in primary and relapsed ependymoma tumors, to identify chromosomal conformations and regulatory mechanisms associated with aberrant gene expression. In particular, we observe the formation of new topologically associating domains ('neo-TADs') caused by structural variants, group-specific 3D chromatin loops, and the replacement of CTCF insulators by DNA hyper-methylation. Through inhibition experiments, we validate that genes implicated by these 3D genome conformations are essential for the survival of patient-derived ependymoma models in a group-specific manner. Thus, this study extends our ability to reveal tumor-dependency genes by 3D genome conformations even in tumors that lack targetable genetic alterations.
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The cytogenetic basis for classifying ependymomas.
The phylogeny of ependymal cells and astrocytes can be traced to a single primitive progenitor the ependymoglia or the tanycyte, respectively. Ependymoglia cells have ependymal perikarya having astrocyte-like processes that terminate subpially in primitive glial footplates. Such cells prevail in primitive nervous systems, but they also persist regionally in the mature mammalian brain. Their fine structure has been studied in many species. An electronmicroscopic study of 8 ependymomas reveals that the neoplastic cells possess features characteristic of primitive ependymoglia; in particular they possess cell processes filled with glial filaments, terminating submesenchymally in a primitive, piston-shaped footplate. The perivascular pseudorosettes of ependymomas are the equivalents of these cell poles. The dominant phenomenon of ependymoma structure appears to be a reversion of cellular organization to the stage of primitive ependymoglia cells. On reviewing 43 ependymomas and 71 astrocytomas 11 neoplasms were found having a tissue structure reminiscent of the evolution of piloid astrocytes from ependymoglia or tanycytes, respectively. These features correspond to transitional stages seen in normal primitive brains. Tumors of this type may be characterized as a tanycytic variant of ependymomas. They appear to be relatively common in the spinal cord and present a source of confusion with piloid astrocytomas.
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[Cytophotometric investigations of the nuclear DNA content in ependymomas and plexuspapillomas (author's transl)].
Report on Feulgen-cytophotometric DNA investigations in 10 ependymomas and 2 plexuspapillomas. All ependymomas represent in their karyograms aneuploidic stem lines. In most of them one observes duplication peaks (G2 cells) as expression of proliferation behaviour. The histological benign ependymomas exhibit stem lines between the hyperdiploid and hypotetraploid values. With increasing malignancy the stem lines are elevated in higher ploidy levels and a reduction of a predominant stem line is recognizable. One of the ependymomas represents a distribution of DNA values like in a "mosaic" tumour. The measurements in the plexuspapillomas in good conformity with the histologic picture reflect the DNA distribution of a benign euploid neoplasm.
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DNA sequences similar to those of simian virus 40 in ependymomas and choroid plexus tumors of childhood.
BACKGROUND: Ependymomas and papillomas of the choroid plexus occur in early childhood. The ubiquitous human polyomaviruses, BK virus and JC virus, have been associated with the induction of these neoplasms in animal models. A related monkey polyomavirus, simian virus 40 (SV40), is highly tumorigenic in rodents and also induces choroid plexus papillomas. METHODS: We tested the possibility that polyomaviruses were associated with these tumors in humans. Tumors from 31 children--20 with choroid plexus neoplasms and 11 with ependymomas--were evaluated for the presence of polyomavirus T-antigen gene sequences by means of amplification with the polymerase chain reaction. RESULTS: Ten of the 20 choroid plexus tumors and 10 of the 11 ependymomas contained amplification products that preferentially hybridized to probes specific for SV40 viral DNA rather than BK or JC viral DNA. In two specimens, DNA sequencing demonstrated that the amplified sequence was identical to the sequence of that region of the SV40 gene. In three other specimens, amplification with SV40-specific primers revealed a 574-bp segment of the SV40 viral gene. In 7 of 11 tumors examined by immunohistochemical staining, viral T antigen was expressed in the nuclei of the neoplastic cells. CONCLUSIONS: Half of the choroid plexus tumors and most of the ependymomas that we studied contained and expressed a segment of T-antigen gene related to SV40. These results suggest that SV40 or a closely related virus may have an etiologic role in the development of these neoplasms during childhood, as in animal models.
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Seeding of intracranial ependymomas in children.
19 cases of intracranial ependymomas were reviewed to determine the incidence of seeding. 4 patients demonstrated conclusive evidence of seeding in sites remote from the operative and irradiated field, 3 of whom had high-grade ependymomas. Clinical-pathological correlation disclosed a greater tendency to seeding and a worse prognosis among patients with high-grade ependymomas. A more aggressive therapeutic approach is indicated when high-grade ependymomas are involved.
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Long-term follow-up in 39 patients with an ependymoma after surgery and irradiation.
1954 to 1989, 33 patients with intracranial ependymomas had primary treatment in our hospital with surgery and postoperative radiotherapy. Five- and ten-year survival rates were 48% and 34% in 25 patients with a high-grade ependymoma and for low-grade ependymomas was maintained at 78% for both the five- and ten-year follow-up period. Cumulative frequency of local or intracranial recurrence was 70% (high-grade) and 20% (low-grade). Despite the fact, that only three of 25 patients with high-grade ependymomas had spinal irradiation with 19.5 to 30 Gy, there was only a single patient with clinical signs of spinal metastases simultaneously with an intracranial recurrence. Twelve out of 13 living patients without signs of tumor have been classified according to the neurological performance: Performance was good or very good in five patients, satisfying in three with an independent life, fair in two patients working in a sheltered office and dependent on some assistance and poor in two patients. Published results are discussed and compared with the own observations and our current treatment policy is given.
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[Prognostic factors and results after the combined surgical and radiotherapy treatment of ependymomas].
Between 1978 and 1991, 31 patients with primary (n = 28) and recurrent (n = 3) ependymoma received external radiation after initial surgery. There were 26 cases of intracranial and five cases of spinal ependymoma. Histological grading revealed low-grade in 23 and high-grade tumors in eight patients. Craniospinal irradiation was given to 13 of 28 patients, whole cranial radiation with boost to posterior fossa in three and local treatment to twelve patients. Six of 28 patients received chemotherapy. Dose to the primary, tumor localisation, grading and extend of surgery did not significantly impact on survival and relapse-free survival. Age was a marginally significant prognostic factor for survival. In patients with intracranial ependymoma (n = 24) survival and relapse-free survival at five years were 54% and 42%, respectively. Patterns of recurrence showed a local recurrence alone in eight, a CNS-relapse with local recurrence in four and local recurrence with distant metastases in one case. Spinal seeding occurred in two of 13 after craniospinal irradiation and two of twelve patients after local treatment. The main problem in the treatment of ependymoma remains local control, the use of spinal irradiation does not seem to improve treatment results.
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Ependymomas of childhood. I. Histological survey and clinicopathological correlation.
We have conducted a correlative histological and clinical study on 34 cases of childhood ependymomas encountered at the Children's Memorial Hospital from 1953 to 1974. The cases were classified into grades A, B and C according to the extent of cytological differentiation and the correlation between the histological grades and the clinical outcome was obtained. It is concluded that the biological behavior of an ependymoma could be predicted to a large extent by the degree of cytological differentiation and by its anatomical location. A large share of the infratentorial ependymomas are well differentiated (grade A) and prognosis in this group is good when complete surgical removal could be accomplished. Most of the supratentorial ependymomas belong to the intermediate grade B and the malignant grade C; prognosis in these two grades is less predictable, approximately two thirds of these patients died within 3 years. The most malignant form is the ependymoblastoma. This is a highly primitive neoplasm histologically similar to medulloblastoma but shows evidence of ependymal differentiation. Most of the ependymoblastomas are located in the supratentorial region and the average length of survival is 20 months.
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Improved survival of patients with intracranial ependymomas by irradiation: doseselection and field extension.
Intracranial ependymomas, treated with postoperative irradiation, have a 50% 5-year survival. Although this rate is good, the issue remains whether the quantity and qualityof survival can be improved by the proper selection of treatment factors, i.e. field size and dose-time relationship. Factors influencing survival and the effect of doseand field selection are considered in 28 intracranial ependymomas. Nineteen spinal primaries are also presented for comparison. Only 10% of the patients treated with dosesbelow 1350 rets are alive, vs. 56% of the patients treated with higher doses. Only21% of the patients treated with partial brain irradiation survived, vs. 57% of those treated with whole brain irradiation. Although an apparent 5-year survival difference was found for the low-grade (63%) vs. high grade (13%) tumors, high-dose whole brain irradiation had a significant effect only on the survival of thehigh-grade tumors. Local recurrences accounted for the majority of theraputic failures. Ten consecutive patients were treated with elctive craniospinal irradiation and exhibited a 3-year survival of 70%, vs. 35% in those treated conventionally to the brain only. Elective craniospinal irradiation, advocated for the high-grade, particularly infratentorial, ependymomas, has currently yieldedhigher survival, lower recurrences, good tolerance, no complications, and excellent quality of survival. Further documentation of this treatment approach is suggested.
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Cytogenetic and loss of heterozygosity studies in ependymomas, pilocytic astrocytomas, and oligodendrogliomas.
Cytogenetic and/or loss of heterozygosity studies were performed on 13 ependymomas, 11 pilocytic astrocytomas, and 18 oligodendrogliomas. Loss of chromosome 22 was the most frequent genetic abnormality among the ependymomas. We found no consistent genetic abnormality in pilocytic astrocytomas. The most common genetic abnormality in oligodendrogliomas was loss of a portion of chromosome 19. Each informative oligodendroglioma had loss of alleles mapped to the long arm (q) of chromosome 19. One oligodendroglioma had an apparent homozygous deletion of the D19S8 locus. Our results, when combined with those in the literature, indicate that chromosomes 9, 11, and 22 may harbor genes important for the pathogenesis of ependymomas and that 19q probably harbors a gene important for the pathogenesis of oligodendrogliomas.
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Extraneural metastases in ependymoma.
Ependymomas, glial neoplasms usually arising in the posterior fossa or spinal cord, rarely metastasize outside the central nervous system. We have reviewed all 81 ependymomas evaluated at MSKCC between 1956-1989. Five (6.2%) had extraneural metastases (ENM). The primary tumor was in spinal cord in 3 patients and the cerebral hemisphere in 2. Two tumors were histologically anaplastic; 3 were histologically benign. The 5 patients were 3, 3, 3.5, 16 and 37 years old. Time from initial diagnosis to development of ENM was 0, 15, 35, 40, and 288 months. At the time of ENM the primary tumor was progressing in 4/5 patients. Prior therapy had included resection plus radiation therapy (RT) (1), RT plus chemotherapy (1), resection plus RT plus chemotherapy (2). One patient had not received prior therapy because ENM were present at diagnosis. The sites of ENM included lung and thoracic lymph nodes (2), pleura and peritoneum (2), and liver (1). Both patients with peritoneal ENM had had ventriculoperitoneal shunts. ENM did not correlate with histologic grade, age, or degree of surgical resection. When patients with ependymoma develop signs or symptoms of systemic disease such as abdominal pain, cough, or adenopathy, ENM should be considered.
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