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World Neurosurgery Apr 2024To assess the efficacy and surgical outcomes of the simultaneous single-trajectory endoscopic biopsy and third ventriculostomy (ETV) in pineal region tumors. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To assess the efficacy and surgical outcomes of the simultaneous single-trajectory endoscopic biopsy and third ventriculostomy (ETV) in pineal region tumors.
METHODS
A systematic review and meta-analysis adhering to Cochrane Standards and PRISMA framework were conducted. PubMed, Embase, and Web Of Science databases were searched until December 2023. Outcomes included rate of histopathologic diagnosis success, ETV success, complications, required VPS, and mortality.
RESULTS
Seventeen studies (N = 388) met inclusion criteria. Histopathologic diagnosis success rate was 90% for general population (95% CI: 86%-95%; I = 42%) and 94% for pediatric patients (95% CI: 89%-98%; I = 19%). ETV Success rate was 93% (95% CI: 88%-97%; I = 60%). An estimated risk of postoperative ETV complications was found to be 16% for the general population (95% CI: 5%-28%; I = 90%) and 5% for pediatric patients (95% CI: 0%-13%; I = 51%). The risk of requiring VPS was estimated as 2% (95% CI: 0%-4%; I = 39%) and for the pediatric population it was 7% (95% CI: 0%-16%; I = 69%). Mortality risk was found to be 1% (95% CI: 0%-3%; I = 0%).
CONCLUSIONS
Simultaneous endoscopic biopsy and ETV demonstrated high diagnostic and therapeutic success rates. The procedure's safety profile, with low mortality and complications, supports its role in treating hydrocephalus associated to pineal region tumors. Subgroup analyses revealed higher diagnostic success rates and required VPS in the pediatric population, whilst it had lower complication rates.
Topics: Child; Humans; Ventriculostomy; Neuroendoscopy; Third Ventricle; Pinealoma; Biopsy; Postoperative Complications; Hydrocephalus; Brain Neoplasms; Pineal Gland; Treatment Outcome; Retrospective Studies
PubMed: 38340795
DOI: 10.1016/j.wneu.2024.02.015 -
Journal of Pineal Research Dec 2023Pineal region tumors (PTs) represent extremely rare pathologies, characterized by highly heterogeneous histological patterns. Most of the available evidence for Gamma... (Review)
Review
Pineal region tumors (PTs) represent extremely rare pathologies, characterized by highly heterogeneous histological patterns. Most of the available evidence for Gamma Knife radiosurgical (GKSR) treatment of PTs arises from multimodal regimens, including GKSR as an adjuvant modality or as a salvage treatment at recurrence. We aimed to gather existing evidence on the topic and analyze single-patient-level data to address the efficacy and safety of primary GKSR. This is a systematic review of the literature (PubMed, Embase, Cochrane, Science Direct) and pooled analysis of single-patient-level data. A total of 1054 original works were retrieved. After excluding duplicates and irrelevant works, we included 13 papers (n = 64 patients). An additional 12 patients were included from the authors' original series. A total of 76 patients reached the final analysis; 56.5% (n = 43) received a histological diagnosis. Confirmed lesions included pineocytoma WHO grade I (60.5%), pineocytoma WHO grade II (14%), pineoblastoma WHO IV (7%), pineal tumor with intermediate differentiation WHO II/III (4.7%), papillary tumor of pineal region WHO II/III (4.7%), germ cell tumor (2.3%), neurocytoma WHO I (2.3%), astrocytoma WHO II (2.3%) and WHO III (2.3%). Presumptive diagnoses were achieved in the remaining 43.5% (n = 33) of cases and comprised of pineocytoma (9%), germ cell tumor (6%), low-grade glioma (6%), high-grade glioma (3%), meningioma (3%) and undefined in 73%. The mean age at the time of GKSR was 38.7 years and the mean lesional volume was 4.2 ± 4 cc. All patients received GKSR with a mean marginal dose of 14.7 ± 2.1 Gy (50% isodose). At a median 36-month follow-up, local control was achieved in 80.3% of cases. Thirteen patients showed progression after a median time of 14 months. Overall mortality was 13.2%. The median OS was not reached for all included lesions, except high-grade gliomas (8mo). The 3-year OS was 100% for LGG and pineal tumors with intermediate differentiation, 91% for low-grade pineal lesions, 66% for high-grade pineal lesions, 60% for germ cell tumors (GCTs), 50% for HGG, and 82% for undetermined tumors. The 3-year progression-free survival (PFS) was 100% for LGG and pineal intermediate tumors, 86% for low-grade pineal, 66% for high-grade pineal, 33.3% for GCTs, and 0% for HGG. Median PFS was 5 months for HGG and 34 months for GCTs. The radionecrosis rate was 6%, and cystic degeneration was observed in 2%. Ataxia as a presenting symptom strongly predicted mortality (odds ratio [OR] 104, p = .02), while GCTs and HGG histology well predicted PD (OR: 13, p = .04). These results support the efficacy and safety of primary GKSR treatment of PTs. Further studies are needed to validate these results, which highlight the importance of the initial presumptive diagnosis for choosing the best therapeutic strategy.
Topics: Humans; Pinealoma; Radiosurgery; Brain Neoplasms; Melatonin; Pineal Gland; Glioma; Neoplasms, Germ Cell and Embryonal
PubMed: 37705383
DOI: 10.1111/jpi.12910 -
World Neurosurgery Mar 2022Pineal region metastases are rare but often cause severe neurologic deficits. Surgical resection and chemoradiotherapy can provide therapeutic benefit. We investigated... (Review)
Review
BACKGROUND
Pineal region metastases are rare but often cause severe neurologic deficits. Surgical resection and chemoradiotherapy can provide therapeutic benefit. We investigated the literature to analyze clinical characteristics, management strategies, and survival of adult patients with pineal region metastases.
METHODS
PubMed, Embase, Scopus, and Cochrane were searched following the PRISMA guidelines, including studies reporting clinical outcomes of patients with pineal region metastases. Clinical presentation, management, and survival were reviewed.
RESULTS
We included 31 studies comprising 47 patients. Lung cancer (29.8%) and carcinomas of unknown origin (14.9%) were the most frequent primary tumors. In 48.9% of patients, symptomatic pineal metastases preceded primary tumor diagnosis. Headache (67.4%) and confusion (46.5%) were the most common symptoms. Parinaud syndrome (46.5%) and hydrocephalus (87.2%) were noted. Biopsy (65.9%) was preferred over resection (34.1%), and shunting strategies used were endoscopic third ventriculostomy (43.9%) and ventriculoperitoneal (26.8%). Eleven patients (32.3%) received adjuvant chemotherapy and 32 (68%) received radiotherapy. Posttreatment improvement in symptoms (56.6%) and hydrocephalus (80.5%) were noted. In patients who received adjuvant chemotherapy/radiotherapy, significant improvement in posttreatment performance status occurred with both biopsy (P < 0.001) and resection (P = 0.007). No survival differences were reported between surgery and biopsy (P = 0.912) or between complete and partial resection (P = 0.220). Overall survival was neither influenced by surgical approach (P = 0.157) nor by shunting strategy (P = 0.822). Mean follow-up was 8 months and median overall survival 3 months. Only 2 cases (4.8%) of pineal metastasis showed recurrence.
CONCLUSIONS
Pineal region metastases carry significant morbidity. Biopsy or surgical resection, combined with adjuvant chemotherapy/radiotherapy and/or shunting, may significantly improve performance status.
Topics: Adult; Brain Neoplasms; Humans; Hydrocephalus; Pineal Gland; Pinealoma; Ventriculostomy
PubMed: 34999267
DOI: 10.1016/j.wneu.2022.01.005 -
Neurosurgical Review Apr 2022Pineal parenchymal tumor of intermediate differentiation (PPTID) is a WHO grade II and III tumor arising from pineal parenchymal cells. PPTID is a rare tumor accounting... (Review)
Review
Pineal parenchymal tumor of intermediate differentiation (PPTID) is a WHO grade II and III tumor arising from pineal parenchymal cells. PPTID is a rare tumor accounting for less than 1% of all primary central nervous system neoplasms. Therefore, reports describing the clinical characteristics and biological features of PPTID are lacking. Moreover, the therapeutic strategy remains controversial. The current study aimed to evaluate treatment results and problems of contemporary therapeutic modalities of PPTID based on its features compared with other pineal parenchymal tumors. A comprehensive systematic literature review of 69 articles was performed, including articles on PPTID (389 patients) and similar tumors. Patient demographics, disease presentation, imaging characteristics, biological features, and current therapeutic options and their results were reviewed. We found that histopathological findings based on current WHO classification are well associated with survival; however, identifying and treating aggressive PPTID cases with uncommon features could be problematic. A molecular and genetic approach may help improve diagnostic accuracy. Therapeutic strategy, especially for grade III and aforementioned uncommon and aggressive tumors, remains controversial. A combination therapy involving maximum tumor resection, chemotherapy, and radiotherapy could be the first line of treatment. However, although challenging, a large prospective study would be required to identify ways to improve the clinical results of PPTID treatment.
Topics: Brain Neoplasms; Combined Modality Therapy; Humans; Pineal Gland; Pinealoma; Prospective Studies
PubMed: 34668090
DOI: 10.1007/s10143-021-01674-3 -
Clinical Neurology and Neurosurgery Feb 2021Papillary tumors of the pineal region (PTPR) are a rare tumor entity first described in 2003, later codified in the 2007 WHO tumor classification system. PTPRs most...
Papillary tumors of the pineal region (PTPR) are a rare tumor entity first described in 2003, later codified in the 2007 WHO tumor classification system. PTPRs most commonly occur in the third and fourth decades, with exceedingly rare presentations in pediatric populations. Herein, we present an additional case of a 10-year-old female found to have PTPR in conjunction with Trisomy 21 managed successfully with cerebrospinal fluid diversion and gross total resection (GTR). Three years after resection she has returned to baseline without recurrence. We also performed a comprehensive review of the current literature discussing the diagnosis, treatment, and pathophysiologic correlations in children. Diagnosis and management of PTPRs is a topic that is increasingly garnering attention in the literature given the recent characterization of this tumor entity. However, relatively little is known about the presentation of PTPRs in pediatric populations. In adults, PTPRs have been linked with several chromosomal and genetic abnormalities; however this correlation is limited in pediatric literature. Although GTR is the mainstay for treatment, the application of adult treatment protocols may not be advisable due to age and the developmental changes of the CNS in children.
Topics: Adolescent; Brain Neoplasms; Child; Female; Humans; Male; Neoplasm Recurrence, Local; Pediatrics; Pineal Gland; Pinealoma
PubMed: 33360024
DOI: 10.1016/j.clineuro.2020.106404 -
Clinical Neurology and Neurosurgery Mar 2020Papillary tumor of the pineal region (PTPR) is a rare neuroepithelial brain tumor, characterized by a high risk of local recurrence (greater than 70 % at 6 years). The...
Papillary tumor of the pineal region (PTPR) is a rare neuroepithelial brain tumor, characterized by a high risk of local recurrence (greater than 70 % at 6 years). The aim of our study was to review the available literature on radiotherapy for PTPR in order to evaluate timings, schedules, outcomes and toxicities of this treatment modality. In our review, 72.4 % (84) of the patients diagnosed with PTPR received radiation therapy. There is heterogeneity in the dose prescription, ranging from 45 Gy (25 × 1.8 Gy) to 60 Gy (30 × 2 Gy) for 3D Conformal Radiation Therapy and from 12 Gy to 36 Gy for Stereotactic Radiosurgery. Being considered as a grade II or III tumor, PTPR should receive higher total radiation dose in the adjuvant setting. Our analysis showed a very limited treatment-related toxicity with an expected 10-y OS of 72.5 %. At 5-years from the diagnosis, about 60 % of the patients experienced a local recurrence, whereas at 10 years the rate is higher than 80 %. In the literature, conflicting data about radiotherapy for PTPR are reported, in particular regarding disease progression. Although radiotherapy represents a fundamental treatment in the management of PTPR, prospective studies are required to better define its impact on overall survival and progression-free survival.
Topics: Brain Neoplasms; Humans; Neoplasm Grading; Neoplasm Recurrence, Local; Neurosurgical Procedures; Pineal Gland; Pinealoma; Progression-Free Survival; Radiosurgery; Radiotherapy, Adjuvant; Radiotherapy, Conformal; Salvage Therapy; Survival Rate; Treatment Outcome
PubMed: 31896492
DOI: 10.1016/j.clineuro.2019.105646 -
Neurosurgery Sep 2019Clinical outcomes and biological behavior of papillary tumors of the pineal region (PTPR) are still under investigation. The best therapeutic strategy has not been...
BACKGROUND
Clinical outcomes and biological behavior of papillary tumors of the pineal region (PTPR) are still under investigation. The best therapeutic strategy has not been defined.
OBJECTIVE
To perform a comprehensive patient-level analysis of all PTPR cases and identify their clinical features, treatment options, and prognostic factors.
METHODS
A search of the medical databases for case series and reports on PTPRs from January 2003 to June 2017 was performed. Data addressing PTPR's clinical presentation, imaging, treatment, and histological features were. Variables associated with the primary outcome of 36-mo survival were identified through Cox regression models.
RESULTS
The initial search yielded 1164 studies, of which 71 were included (60 case reports and 11 case series), containing 177 patients (mean age 33.0 ± 15.3 yr and 53.2% male). Intracranial hypertension and hydrocephalus prevailed as the clinical picture. Surgery was performed on 82.0% and gross total resection (GTR) was achieved on 71.4%. A total of 56.8% recurred after a median 29 mo (quartiles 10.5-45.5). The 36-mo survival rate was 83.5% (95% confidence interval [CI] 76.2-89.2%). Good functional outcomes (Glasgow Outcome Scale 4/5) were observed in 60.0%. The variables of interest were inconsistently reported and the multivariable analysis final sample was 133 patients. After adjustment for age, tumor size (each additional centimeter, hazard ratio [HR] 1.99, 95% CI 1.12-3.53, P = .019) and surgical treatment (HR 0.16, 95% CI 0.05-0.45, P = .001) were associated with 36-mo survival.
CONCLUSION
Tumor size and surgery are associated with improvement in 36-mo survival. We did not observe any significant benefits from GTR or adjuvant treatments.
Topics: Adult; Brain Neoplasms; Female; Humans; Male; Middle Aged; Papilloma; Pineal Gland; Pinealoma; Prognosis; Young Adult
PubMed: 30989225
DOI: 10.1093/neuros/nyz062 -
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 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