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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 -
Neurosurgical Review Mar 2019We conducted a systematic review of 72 studies to characterize trilateral retinoblastomas. Kaplan-Meier analysis was used to estimate survival, and statistical...
We conducted a systematic review of 72 studies to characterize trilateral retinoblastomas. Kaplan-Meier analysis was used to estimate survival, and statistical significance was assessed by using a log-rank test. We analyzed 211 cases of trilateral retinoblastomas. The average age of onset of retinoblastoma was 0.79 ± 1.38 years, and the average latency period between the onset of retinoblastomas and trilateral retinoblastomas was 1.49 ± 1.76 years. The brain tumors were found before the retinoblastoma diagnosis in 6 cases (3.1%), concurrently in 61 cases (32.1%), and after the retinoblastoma diagnosis in 123 cases (64.7%). Pineal tumors were found in 155 cases (73.4%) and sellar tumors in 46 cases (21.8%). The overall median survival was 10.3 months (95% CI, 8.5-13) and the 5-year survival rate was 15.7%. Central nervous system symptoms were variable and associated with shorter survival in univariate and multivariate analyses. The survival time in patients who received high-dose chemotherapy with stem cell transplant was significantly longer (p = 0.0067) than that of with or without conventional chemotherapy. Twelve long-term survivors were reported, and of these, six patients were treated with high-dose chemotherapy with stem cell transplant and six patients were treated with conventional chemotherapy. It is important that survivors continue to undergo regular medical surveillance in order to detect trilateral retinoblastoma at a potentially curative stage. Trilateral retinoblastoma patients with an irradiation history had shorter survival than those without irradiation history for retinoblastoma. High-dose chemotherapy should be considered as a potential treatment option for trilateral retinoblastomas.
Topics: Child, Preschool; Female; Humans; Infant; Infant, Newborn; Kaplan-Meier Estimate; Male; Retinal Neoplasms; Retinoblastoma
PubMed: 28815312
DOI: 10.1007/s10143-017-0890-4 -
International Journal of Radiation... May 2016Because it spares many normal tissues and reduces the integral dose, proton therapy (PT) is the preferred tumor irradiation technique for treating childhood cancer.... (Review)
Review
Because it spares many normal tissues and reduces the integral dose, proton therapy (PT) is the preferred tumor irradiation technique for treating childhood cancer. However, to the best of our knowledge, no systematic review of the clinical effectiveness of PT in children has been reported in the scientific literature. A systematic search for clinical outcome studies on PT published between 2007 and 2015 was performed in Medline (through OVID), EMBASE, and the Cochrane Library. Twenty-three primary studies were identified, including approximately 650 patients overall. The median/mean follow-up times were limited (range, 19-91 months). None of the studies were randomized, 2 were comparative, and 20 were retrospective. Most suffered from serious methodologic limitations, yielding a very low level of clinical evidence for the outcomes in all indications. For example, for retinoblastoma, very low-level evidence was found that PT might decrease the incidence of second malignancies. For chondrosarcoma, chordoma, craniopharyngioma, ependymoma, esthesioneuroblastoma, Ewing sarcoma, central nervous system germinoma, glioma, medulloblastoma, osteosarcoma, and rhabdomyosarcoma, there was insufficient evidence to either support or refute PT in children. For pelvic sarcoma (ie, nonrhabdomyosarcoma and non-Ewing sarcoma), pineal parenchymal tumor, primitive neuroectodermal tumor, and "adult-type" soft tissue sarcoma, no studies were identified that fulfilled the inclusion criteria. Although there is no doubt that PT reduces the radiation dose to normal tissues and organs, to date the critical clinical data on the long-term effectiveness and harm associated with the use of PT in the 15 pediatric cancers under investigation are lacking. High-quality clinical research in this area is needed.
Topics: Adolescent; Child; Child, Preschool; Follow-Up Studies; Humans; Infant; Infant, Newborn; Neoplasms; Organ Sparing Treatments; Organs at Risk; Proton Therapy; Radiation Injuries; Retrospective Studies; Time Factors; Young Adult
PubMed: 27084646
DOI: 10.1016/j.ijrobp.2015.10.025 -
World Neurosurgery Feb 2016Magnetic resonance imaging (MRI) is the imaging modality of choice for the clinical management of brain tumors, and the majority of scanners operate with static magnetic... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Magnetic resonance imaging (MRI) is the imaging modality of choice for the clinical management of brain tumors, and the majority of scanners operate with static magnetic field strengths of 1.5 or 3.0 Tesla (T). During the past decade, ultrahigh field (UHF) MRI has been investigated for its clinical applicability. This meta-analysis evaluates studies pertaining to the application of UHF MRI to patients with brain tumors.
METHODS
The authors performed a systematic review of the literature. Articles relating to application of UHF MRI to brain anatomy and brain tumors with living subjects were included. Studies were grouped into 1 of 3 categories based on area of focus: "Anatomical Structures Involved with Brain Tumors," "Tumor characterization," and "Treatment Monitoring." Comparison studies with extractable outcomes measure data were analyzed for performance of UHF MRI versus clinical field strengths (1.5 T and 3 T).
RESULTS
Twenty-four studies (361 subjects) met inclusion criteria. The field of study was heterogeneous and rigorous statistical analysis was not possible. Overall, 279 patients with brain tumors scanned at UHF MRI have been reported. Of these, glioma and glioblastoma multiforme are the most commonly studied lesions (38.9% and 24.4%, respectively). In comparison studies between UHF MRI and clinical field strengths, 24 of 51 patients had outcome measures that were better with UHF MRI, 17 of 24 were equivalent at both field strengths, and 9 were worse at UHF MRI. The most common causes of a worse performance were susceptibility artifacts and magnetic field inhomogeneities (3 of 9). Imaging of the pituitary gland, pineal gland veins, cranial nerves, and tumor microvasculature were all shown to be feasible.
CONCLUSIONS
UHF MRI shows promise to improve detection and characterization of brain tumors, preoperative planning for neurosurgical resection, and longitudinal monitoring of the effects of radiation and antibody-based therapies. Technical innovations are needed to overcome field inhomogeneity and susceptibility artifacts in certain regions of the skull. Finally, larger studies comparing 1.5 T, 3.0 T, and 7.0 T or greater will determine whether UHF MRI gains acceptance as a clinical standard.
Topics: Brain Neoplasms; Humans; Magnetic Resonance Imaging
PubMed: 26409071
DOI: 10.1016/j.wneu.2015.09.048 -
American Journal of Ophthalmology Dec 2015To estimate the incidence of trilateral retinoblastoma in patients with retinoblastoma. (Meta-Analysis)
Meta-Analysis Review
PURPOSE
To estimate the incidence of trilateral retinoblastoma in patients with retinoblastoma.
DESIGN
Systematic review and meta-analysis.
METHODS
We searched Medline and Embase for scientific literature published between January 1966 and July 2015 that assessed trilateral retinoblastoma incidence. We used a random-effects model for the statistical analyses.
RESULTS
We included 23 retinoblastoma cohorts from 26 studies. For patients with bilateral retinoblastoma the unadjusted chance of developing trilateral retinoblastoma across all cohorts was 5.3% (95% confidence interval [CI]: 3.3%-7.7%); the chance of pineal trilateral retinoblastoma was 4.2% (95% CI: 2.6%-6.2%) and the chance of nonpineal trilateral retinoblastoma was 0.8% (95% CI: 0.4%-1.3%). In patients with hereditary retinoblastoma (all bilateral cases, and the unilateral cases with a family history or germline RB1 mutation) we found a trilateral retinoblastoma incidence of 4.1% (95% CI: 1.9%-7.1%) and a pineal trilateral retinoblastoma incidence of 3.7% (95% CI: 1.8%-6.2%). To reduce the risk of overestimation bias we restricted analysis to retinoblastoma cohorts with a minimum size of 100 patients, resulting in adjusted incidences of 3.8% (95% CI: 2.4%-5.4%), 2.9% (95% CI: 1.9%-4.2%), and 0.7% (95% CI: 0.3%-1.2%) for any, pineal, and nonpineal trilateral retinoblastoma, respectively, among patients with bilateral retinoblastoma. Among hereditary retinoblastoma we found an adjusted trilateral retinoblastoma incidence of 3.5% (95% CI: 1.2%-6.7%) and a pineal trilateral retinoblastoma incidence of 3.2% (95% CI: 1.4%-5.6%).
CONCLUSION
The estimated incidence of trilateral retinoblastoma is lower than what is reported in previous literature, especially after exclusion of small cohorts that were subject to overestimation bias in this context.
Topics: Global Health; Humans; Incidence; Retinal Neoplasms; Retinoblastoma
PubMed: 26374932
DOI: 10.1016/j.ajo.2015.09.009 -
Neuro-Chirurgie 2015The sitting position for pineal tumour removal remains controversial as regards the number of potential complications despite good surgical conditions. (Review)
Review
INTRODUCTION
The sitting position for pineal tumour removal remains controversial as regards the number of potential complications despite good surgical conditions.
METHOD
A systematic review of the literature was conducted in order to record the most frequent complications observed in this position, their incidence and prevention.
RESULTS
Venous air embolism, hypotension, pneumocephalus, macroglossia, quadriplegia and nerve injuries are the most frequent complications observed. Their incidence can be dramatically decreased with an accurate anesthesiological and neurosurgical management.
CONCLUSION
In training teams, the sitting position remains the gold standard for pineal tumour removal.
Topics: Anesthetics; Embolism, Air; Humans; Intraoperative Complications; Monitoring, Intraoperative; Neurosurgical Procedures; Pineal Gland
PubMed: 25676910
DOI: 10.1016/j.neuchi.2014.10.110 -
The Lancet. Oncology Sep 2014About 5% of children with retinoblastoma from germline mutation of the RB1 gene are at risk of developing trilateral retinoblastoma--intraocular retinoblastoma combined... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
About 5% of children with retinoblastoma from germline mutation of the RB1 gene are at risk of developing trilateral retinoblastoma--intraocular retinoblastoma combined with a histologically similar brain tumour, most commonly in the pineal gland. We aimed to provide a systematic overview of published data for trilateral retinoblastoma, and to analyse how survival has changed.
METHODS
We searched Medline and Embase for scientific literature published between Jan 1, 1966, and April 14, 2014, that assessed trilateral retinoblastoma cases. We undertook a meta-analysis of survival with the Kaplan-Meier method and Cox proportional hazards regression, stratified on the basis of the original study, to account for between-study heterogeneity.
FINDINGS
We included 90 studies, with 174 patients with trilateral retinoblastoma. 5-year survival after pineal trilateral retinoblastoma increased from 6% (95% CI 2-15) in patients diagnosed before 1995, to 44% (26-61; p<0·0001) in those diagnosed from 1995 onwards. Before 1995, no patients with non-pineal trilateral retinoblastoma survived, but from 1995 onwards, 5-year survival was 57% (30-77; p=0·035). Hazard ratios (HR) adjusted for the presence of leptomeningeal metastases and trilateral retinoblastoma location, suggested that both conventional (HR 0·059, 95% CI 0·016-0·226; p<0·0001) and high-dose chemotherapy with stem-cell rescue (0·013, 0·002-0·064; p<0·0001) most strongly contributed to this improvement. Absence of leptomeningeal metastases (HR 2·13, 95% CI 0·98-4·60; p=0·055) were associated with improved survival. Non-pineal trilateral retinoblastomas were larger than pineal tumours (median 30 mm [range 6-100] vs 22 mm [7-60]; p=0·012), but both had similar outcomes since 1995.
INTERPRETATION
Our results suggest that improvements in overall survival are attributable to improved chemotherapy regimens and early detection of pineal trilateral retinoblastoma. As such, successful treatment of trilateral retinoblastoma should include screening at least at the time of retinoblastoma diagnosis and chemotherapy, which would preferably be a high-dose regimen with autologous stem-cell rescue.
FUNDING
None.
Topics: Antineoplastic Combined Chemotherapy Protocols; Child; Child, Preschool; Combined Modality Therapy; Disease-Free Survival; Early Detection of Cancer; Female; Hematopoietic Stem Cell Transplantation; Humans; Infant; Kaplan-Meier Estimate; Male; Pineal Gland; Prognosis; Proportional Hazards Models; Radiotherapy, Adjuvant; Retinal Neoplasms; Retinoblastoma; Risk Assessment; Survival Analysis; Treatment Outcome
PubMed: 25126964
DOI: 10.1016/S1470-2045(14)70336-5 -
Neuro-Chirurgie 2015The natural history of pineal cysts still remains unclear. Incidental pineal cysts have become more common which raises the question of their management. Symptomatic... (Review)
Review
OBJECTIVE
The natural history of pineal cysts still remains unclear. Incidental pineal cysts have become more common which raises the question of their management. Symptomatic pineal cysts may require a surgical solution but therapeutic indications have not yet been clearly established.
METHOD
From 1986 to 2012, 26 patients with pineal cysts were identified. Their medical records were retrospectively assessed focusing on the initial symptoms, imaging characteristics of the cyst, management strategy, operative technique and their complications, as well as the latest follow-up. A systematic review of the literature is also presented.
RESULTS
Twenty-six patients with pineal cysts were identified. The mean age was 23.5 years ranging from 7 to 49 years. Symptoms included intracranial hypertension with obstructive hydrocephalus in 18 cases and oculomotor anomalies in 12 cases. Two adult cases presented with non-specific headaches and did not require surgery. Twenty patients were operated via a suboccipital transtentorial approach with total removal of the cyst in 70% of the cases, while the remaining 4 cases were treated with an intraventricular endoscopic marsupialization associating a third ventriculostomy. Four patients required a preoperative ventriculo-peritoneal shunt due to life-threatening obstructive hydrocephalus. Overall, peri-operative mortality was nil. In the two non-operated patients, the cyst remained stable and no recurrences were observed in all operated patients with a mean follow-up of 144 months.
CONCLUSION
In the majority of incidental pineal cysts, a clinical and imaging follow-up is sufficient but occasionally not required especially in adults as very rare cases of increase in size have been reported.
Topics: Brain Neoplasms; Central Nervous System Cysts; Humans; Hydrocephalus; Neoplasm Recurrence, Local; Ventriculoperitoneal Shunt; Ventriculostomy
PubMed: 24907165
DOI: 10.1016/j.neuchi.2013.08.010 -
Clinical Neuroradiology Mar 2015Atypical teratoid/rhabdoid tumor (AT/RT) occurs in children less than 3 years old, and has a very poor prognosis. AT/RT seldom occurs in adult. We have experienced four... (Review)
Review
PURPOSE
Atypical teratoid/rhabdoid tumor (AT/RT) occurs in children less than 3 years old, and has a very poor prognosis. AT/RT seldom occurs in adult. We have experienced four cases of AT/RT at our institute. The purpose of this study is to evaluate the radiological image findings of adult-onset AT/RT and to conduct a systematic review.
METHODS
Image findings of four AT/RTs in our institute were retrospectively evaluated by an experienced neuroradiologist. If the images were unavailable, image findings were evaluated from the former image interpretation report. We assembled papers of adult-onset AT/RT (n = 38) and evaluated the image findings.
RESULTS
AT/RT occurs in a variety of sites (spinal region, pineal region, suprasellar region, jugular foramen, and so on). High density on computed tomography (CT) was seen in 10 of 11 cases; mixed intensity in T2-weighted image was seen in 13 of 18 cases; and high intensity on diffusion-weighted image (DWI) was seen in 3 of 3 cases. Contrast enhancement was observed in all cases in which images were available.
CONCLUSIONS
We have experienced four adult-onset AT/RT cases at our institute and have evaluated image findings through systematic review. The image findings of high density on CT, high intensity on DWI, with low apparent diffusion coefficient, and a heterogenous component should lead to an inclusion of AT/RT in the differential diagnosis of a tumor; these findings may be able to suggest AT/RT; however, they cannot make the diagnosis.
Topics: Adult; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Rhabdoid Tumor; Teratoma; Tomography, X-Ray Computed; Young Adult
PubMed: 24477665
DOI: 10.1007/s00062-013-0282-2