Cerebral primitive neuroectodermal tumors in childhood

Tomita, Tadanori; McLone, David G.; Yasue, Masaharu

doi:10.1007/bf00163707

Cited by 59 publications

(31 citation statements)

References 31 publications

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“…The most frequent presenting symptom in this series was elevated intracranial pressure which has also been noted in other series [13,15,16]. Tumors were located in the supratentorial area in 24 patients and in the infratentorial area in 6 patients.…”

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confidence: 61%

Primitive Cerebral Neuroectodermal Tumors Excluding Medulloblastomas: A Retrospective Study of 30 Cases

et al. 1998

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We present a retrospective study of 30 cases of primitive cerebral neuroectodermal tumors (PNET), excluding medulloblastomas, referred to us postoperatively for additional therapy to evaluate prognostic factors and treatment efficiency. The histologic types were: pinealoblastomas (n = 7); ependymoblastomas (n = 2); medulloepitheliomas (n = 4), and other PNET (n = 17). The tumor was located in the supratentorial area in 24 patients and in the posterior fossa in 6 patients. Among the supratentorial tumors, 8 were metastatic. Maximal surgical resection was performed. Sixteen of 30 patients had no measurable disease after surgery and were considered as standard-risk (SR) cases, and 14 with a local residue or metastasis as high-risk (HR) cases. The objective of postsurgical treatment was to avoid radiotherapy in children below 4 years of age. It consisted of radiotherapy alone in 6 patients, chemotherapy alone in 17, and radiotherapy with chemotherapy in 7. Furthermore, high-dose chemotherapy (busulfan, thiotepa) and autologous bone marrow transplantation, performed in 6 patients, yielded a response rate of 3/6. Event-free survival (EFS) of SR patients was 37% at 3 years (95% confidence interval (CI) 14–60%) and overall survival 44% (95% CI 26–62%). Only 1 of the HR patients achieved a complete remission and all of them died early. The critical prognostic factors appear to be the completeness of initial surgical resection and absence of metastasis. These tumors have a poor prognosis. Novel strategies (high-dose chemotherapy) are needed to improve their outcome because the children concerned are very young and the effects of radiotherapy are particularly deleterious when tumors are situated in the supratentorial area.

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confidence: 61%

Primitive Cerebral Neuroectodermal Tumors Excluding Medulloblastomas: A Retrospective Study of 30 Cases

et al. 1998

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“…21) In children with cerebral PNETs, there was no correlation between extent of resection and survival in those children treated with radiation therapy. 35) However, gross total resection was associated with better local control. 35) Similarly, an adult series of medulloblastomas of the posterior fossa did not demonstrate an improvement in progression free survival with gross total resection, although complete resection was associated with a decreased rate of local recurrence.…”

Section: Extent Of Resectionmentioning

confidence: 99%

“…35) However, gross total resection was associated with better local control. 35) Similarly, an adult series of medulloblastomas of the posterior fossa did not demonstrate an improvement in progression free survival with gross total resection, although complete resection was associated with a decreased rate of local recurrence. 14) The extent of surgical resection may be influenced by many factors: goal of surgery (biopsy, debulking, complete resection), surgeon's skill and experience, tumor invasion and involvement with adjacent structures, and physician beliefs about the effectiveness of alternative therapies (irradiation therapy, chemotherapy).…”

Section: Extent Of Resectionmentioning

confidence: 99%

Management and Survival of Pineoblastoma: An Analysis of 34 Adults From the Brain Tumor Registry of Japan

Lee

Wakabayashi

Yoshida

2005

Neurol. Med. Chir.(Tokyo)

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Pineoblastoma is a rare tumor in adults, and factors influencing survival are poorly understood. Data from the Brain Tumor Registry of Japan (BTRJ) was analyzed to examine patient, tumor, and treatment characteristics associated with increased survival in adults with pineoblastomas. All pineoblastoma cases in adults aged 16 years or older were identified in the BTRJ. Data were extracted on demographics, presentation, tumor characteristics, treatments, and outcomes. Kaplan-Meier plots, the log rank method, and p value º0.15 was used to screen variables for inclusion in a multivariate Cox regression estimating survival. In the final Cox multivariate model, all variables with p values º0.05 were considered significant predictors of survival, and all variables with p values 0.05-0.099 were considered trends. The BTRJ contained 34 adults with pineoblastomas diagnosed from 1969-1998. The patients were predominantly male (22 patients), with a median age of 35 years (range 16-66 years). Median survival from diagnosis was 25.7 months, with a median follow up of 20.5 months. Median surgical resection was 75-94%, and five of the 34 patients had gross total resection. Twenty-nine of the 34 patients received cranial irradiation therapy with a median dose of 50 Gy (range 30-70 Gy). In the final multivariate model, cranial irradiation AE40 Gy (p ＝ 0.014) and gross total resection (p ＝ 0.034) were associated with improved survival. There was a trend towards improved survival for women (p ＝ 0.099). Adult pineoblastoma patients have poor survival prognosis. Cranial irradiation therapy using at least 40 Gy and complete surgical resection are associated with improved survival.

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“…A 3-year survival of 60%, simi lar to that of medulloblastoma, has been reported, al though most others have reported worse survival [4,[15][16][17][18]. There is a direct relationship between patient age at the time of diagnosis and outcome; younger patients sur vive for a shorter period than older patients.…”

Section: Discussionmentioning

confidence: 91%

“…There is a direct relationship between patient age at the time of diagnosis and outcome; younger patients sur vive for a shorter period than older patients. Length of survival apparently bears no correlation with differentia tion of tumor cells along glial or neuronal lines [2,16,17]. Gangeme et al [15] reported that patients with cystic tumors seem to survive longer than those with solid tumors.…”

Section: Discussionmentioning

confidence: 99%

Prognostic Factors in Children with Supratentorial (Nonpineal) Primitive Neuroectodermal Tumors

et al. 1995

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Supratentorial primitive neuroectodermal tumors (S-PNETs), which have also been called cerebral neuroblastomas, have been considered to be the hemispheric equivalent of posterior fossa medulloblastomas. Twenty-seven children with S-PNETs (excluding pineoblastomas) which were confirmed by central pathology review were treated on the CCG-921 protocol from 1986 to 1992. After operation, all patients were staged with CSF cytology and spinal myelography or magnetic resonance scans and were treated with craniospinal irradiation and chemotherapy. Data from these 27 patients have been reviewed to evaluate neurosurgical treatment, survival, and prognostic variables that correlate with survival. Overall survival at 5 years was 34% (SE 20%) and progression-free survival (PFS) was 31% (SE 18%), which is lower than the survival of patients with posterior fossa PNETs (medulloblastomas). PFS was significantly worse in children 1.5–3 years of age at diagnosis and in those with evidence of tumor dissemination at the time of diagnosis. Large preoperative tumors were more likely to be associated with greater than 1.5 cm² residual tumor postoperatively. Neurosurgeons estimated that less than 1.5 cm² of residual tumor was present in 52% of the cases; postoperative scans confirmed that in 58%. For children with less than 1.5 cm² residual tumor, postoperative survival at 4.0 years was 40% (SE 22%); for those with greater than 1.5 cm² residual tumor, survival was 13% (SE 8%). The difference did not reach statistical significance, due to small numbers in this series, though a trend did exist (p = 0.19). Large series will be required to clarify the effects of extent of resection on survival.

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Cerebral primitive neuroectodermal tumors in childhood

Cited by 59 publications

References 31 publications

Primitive Cerebral Neuroectodermal Tumors Excluding Medulloblastomas: A Retrospective Study of 30 Cases

Primitive Cerebral Neuroectodermal Tumors Excluding Medulloblastomas: A Retrospective Study of 30 Cases

Management and Survival of Pineoblastoma: An Analysis of 34 Adults From the Brain Tumor Registry of Japan

Prognostic Factors in Children with Supratentorial (Nonpineal) Primitive Neuroectodermal Tumors

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