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World Neurosurgery Dec 2017Tumors arising from the pineal region account for approximately 1% of intracranial neoplasms. We present a case of a previously healthy 5-year-old boy with an acute... (Review)
Review
BACKGROUND
Tumors arising from the pineal region account for approximately 1% of intracranial neoplasms. We present a case of a previously healthy 5-year-old boy with an acute onset of headache. A magnetic resonance imaging (MRI) scan showed a pineal mass with aqueduct compression. The patient was scheduled for tumor resection. An endoscopic third ventriculostomy was performed in advance for the treatment of hydrocephalus. Afterwards, MRI showed a relevant regression of the pineal mass without specific treatment. Consequently, surgery was cancelled and further MRI follow-up showed a regression of the mass and a constant tumor mass over a period of 30 months. Spontaneous regression of malignant tumors is a rare phenomenon with an incidence of 1 of 60,000-100,000 cases. Only a few cases with spontaneous regression of pineal tumors have been reported.
METHODS
We performed a systematic literature review according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines on spontaneously regressing pineal lesions and found 13 cases in the literature.
RESULTS
Six hypotheses for explaining tumor regression were found, comprising treatment with steroids, effects of diagnostic irradiation, treatment of hydrocephalus, pineal apoplexy, surgical trauma, and immunologic mechanisms. None of these mechanisms was evidentiary. However, in all reported cases, some kind of treatment (e.g. treatment of hydrocephalus, application of steroids, and so on) has been performed before tumor regression.
CONCLUSIONS
The clinician has to bear in mind that regression of pineal tumors might be triggered by use of steroids, for example, and in cases of improvement of the patient's presenting symptoms, new MRI scans should be performed.
Topics: Brain Neoplasms; Cerebral Aqueduct; Child, Preschool; Consciousness Disorders; Diffusion Magnetic Resonance Imaging; Headache; Humans; Hydrocephalus; Magnetic Resonance Imaging; Male; Neoplasm Regression, Spontaneous; Pineal Gland; Pinealoma; Ventriculostomy; Vomiting
PubMed: 28844909
DOI: 10.1016/j.wneu.2017.08.106 -
Journal of Neuro-oncology Feb 2012Supratentorial primitive neuroectodermal tumors (sPNET) are rare childhood brain tumors. There is no standard strategy for treating relapsed sPNETs. The role of high... (Review)
Review
Supratentorial primitive neuroectodermal tumors (sPNET) are rare childhood brain tumors. There is no standard strategy for treating relapsed sPNETs. The role of high dose chemotherapy with hematopoietic stem cell rescue (HDC with HSCR) in treating relapsed sPNET is controversial. A systematic review of the literature regarding outcome of patients with relapsed sPNET treated with HDC and HSCR was performed to examine the potential predictive factors that would justify its use in this subset of patients. Forty-six patients were identified fulfilling the inclusion criteria. Of those, 15 patients were infants and 15 were pineoblastomas. With a median follow-up of 40 months (range 3-123 months) 15 patients were reported alive. Thirteen patients out of the 15 survivors did not receive craniospinal irradiation (CSRT). The 12 month overall survival (OS) of the cohort was 44.2 ± 7.5 months. Twelve-month OS for children less than 36 months was 66.7 ± 12.2 months while for older children it was 27.8 ± 10.6 (P = 0.003). Twelve-month OS was 20.0 ± 10.3 for those patients with pineoblastoma versus 54.6 ± 9.0 for those with non-pineal sPNETs (P < 0.001). Cox regression analysis revealed pineal location as the only independent adverse prognostic factor. In conclusion high dose chemotherapy with HSCR might lead to survival primarily in younger children with relapsed sPNET even in the absence of concomitant use of radiotherapy, whereas the outcome in older children and/or in pineal location is extremely poor with this modality.
Topics: Combined Modality Therapy; Databases, Bibliographic; Drug Therapy; Follow-Up Studies; Hematopoietic Stem Cell Transplantation; Humans; Neuroectodermal Tumors; Proportional Hazards Models; Retrospective Studies; Supratentorial Neoplasms; Survival Analysis
PubMed: 21850536
DOI: 10.1007/s11060-011-0690-6 -
Journal of Neurosurgery Aug 2010Pineocytoma is a rare tumor, and the current literature on these tumors is primarily composed of case reports and small case series. Thus, recommendations on appropriate... (Review)
Review
OBJECT
Pineocytoma is a rare tumor, and the current literature on these tumors is primarily composed of case reports and small case series. Thus, recommendations on appropriate treatment of these tumors are highly varied. Therefore, the authors performed a systematic review of the literature on tumor control after surgery for pineocytoma to determine the relative benefits of aggressive resection and postoperative adjuvant radiotherapy.
METHODS
A comprehensive search of the published English-language literature was performed to identify studies citing outcome data of patients undergoing surgery for pineocytoma. Determination of rates of progression-free survival (PFS) was performed using Kaplan-Meier analysis.
RESULTS
Sixty-four articles met the criteria of the established search protocol, which combined for a total of 166 patients. Twenty-one percent of these patients had undergone a biopsy procedure, 38% had undergone subtotal resection (STR), 42% had undergone gross-total resection, and 28% were treated with radiation therapy. The 1- and 5-year PFS rates for the resection group versus the biopsy group were 97 and 90% (1 year), and 89 and 75% (5 years), respectively (p < 0.05, log-rank test). The 1- and 5-year PFS rates for the GTR group versus the group undergoing STR combined with radiation therapy were 100 and 94% (1 year), and 100 and 84% (5 years), respectively (p < 0.05, log-rank test). There was no significant difference in PFS for STR only compared with STR in addition to radiation therapy.
CONCLUSIONS
Gross-total resection is the ideal treatment for pineocytoma and might represent a cure for these lesions. When gross-total resection is not possible, adjuvant radiation therapy after STR is of questionable benefit for these patients.
Topics: Brain Neoplasms; Combined Modality Therapy; Humans; Kaplan-Meier Estimate; Pineal Gland; Pinealoma; Radiotherapy, Adjuvant
PubMed: 20136388
DOI: 10.3171/2009.12.JNS091683 -
Ophthalmology Oct 2003To assess the risk of retinoblastoma developing in children with microscopic chromosomal with mosaic deletions involving 13q14. (Review)
Review
PURPOSE
To assess the risk of retinoblastoma developing in children with microscopic chromosomal with mosaic deletions involving 13q14.
DESIGN
Case report and systematic literature review.
PARTICIPANTS
Data on 29 patients with a mosaic and 107 patients with a nonmosaic somatic deletion of chromosome 13q14 were compared.
MAIN OUTCOME MEASURES
Age at diagnosis, frequency, and laterality of retinoblastoma.
CASE REPORT
A dysmorphic baby, who carried a chromosomal deletion involving 13q14 in 34% of peripheral blood lymphocytes, had neuroradiologic evidence of retinoblastoma at the age of 2 weeks. She developed trilateral retinoblastoma, a pineal neuroblastic tumor, at the age of 10 months. The diagnosis of her tumor was delayed because of misjudgment of risk of retinoblastoma developing.
RESULTS
Meta-analysis revealed no difference between children with mosaic and nonmosaic chromosomal deletion of 13q14 regarding the age at diagnosis, laterality of tumor, and presence of family history for retinoblastoma. A lower percentage of somatic cells with mosaic deletion did not predict a higher age at diagnosis or unilateral tumors. No statistically significant difference was noted regarding the presence of mental retardation, dysmorphic features, and anomalies of internal organs between mosaic and nonmosaic deletions. Only 7% (95% confidence interval, 1-23) of 29 patients who had a mosaic chromosomal deletion including 13q14 were not reported to develop retinoblastoma.
CONCLUSIONS
Whenever a 13q14 deletion is diagnosed, immediate ophthalmologic evaluation is recommended to ensure prompt diagnosis of retinoblastoma. Mosaic and nonmosaic chromosomal deletions of 13q14 do not differ regarding the risk and type of retinoblastoma developing.
Topics: Brain Neoplasms; Chromosome Deletion; Chromosomes, Human, Pair 13; Fatal Outcome; Female; Humans; Infant; Magnetic Resonance Imaging; Mosaicism; Pineal Gland; Pinealoma; Retinal Neoplasms; Retinoblastoma; Risk Factors; Tomography, X-Ray Computed
PubMed: 14522775
DOI: 10.1016/S0161-6420(03)00484-6