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Neuroimaging Clinics of North America Feb 2017A review of pediatric pineal region tumors is provided with emphasis on advanced imaging techniques. The 3 major categories of pineal region tumors include germ cell... (Review)
Review
A review of pediatric pineal region tumors is provided with emphasis on advanced imaging techniques. The 3 major categories of pineal region tumors include germ cell tumors, pineal parenchymal tumors, and tumors arising from adjacent structures such as tectal astrocytomas. The clinical presentation, biochemical markers, and imaging of these types of tumors are reviewed.
Topics: Brain Neoplasms; Child; Diffusion Magnetic Resonance Imaging; Humans; Magnetic Resonance Spectroscopy; Neuroimaging; Pineal Gland
PubMed: 27889025
DOI: 10.1016/j.nic.2016.08.002 -
Neuro-oncology Advances 2022The management of pineal parenchymal tumors remains controversial.
Survival and associated predictors for patients with pineoblastoma or pineal parenchymal tumors of intermediate differentiation older than 3 years: Insights from the National Cancer Database.
BACKGROUND
The management of pineal parenchymal tumors remains controversial.
METHODS
The 2004-2017 National Cancer Database was queried for cases (age >3 years) with histologically confirmed pineal parenchymal tumors of intermediate differentiation (PPTID, n = 90) or pineoblastoma (n = 106).
RESULTS
Within the PPTID group, median age was 41 years; 49% were males. Five- and 10-year survival were 83% and 78%, respectively. Adjuvant radiation and chemotherapy were administered in 64% and 17% patients, respectively. The effect of radiation with or without chemotherapy (HR 1.15, = .81, and HR 1.31, = .72, respectively), and extent of resection (HR = 1.07, = .93) was not significant. Within the pineoblastoma group, median age was 25 years; 51% were males. Five- and 10-year survival were 66% and 42%, respectively. Adjuvant radiation and chemotherapy were administered in 72% and 51%, respectively. In multivariable analysis, patients with pineoblastoma who received both radiation and chemotherapy (n = 39) had significantly lower hazard of death (HR 0.35, 95% CI 0.14-0.85, = .02) compared to those who received radiation alone (n = 20) or no adjuvant treatment (n = 19). Finally, females in the pineoblastoma group were found to have a lower hazard of death compared to males (HR 0.24, 95% CI 0.10-0.58, = .001); this comparison trended toward statistical significance in the PPTID subgroup (HR 0.40, 95% CI 0.14-1.08, = .07).
CONCLUSIONS
Survival rates were higher in patients with PPTID vs patients with pineoblastoma. Adjuvant chemoradiation was associated with improved survival in pineoblastoma and females had lower hazards of death. Further research should identify specific patient profiles and molecular subgroups more likely to benefit from multimodality therapy.
PubMed: 35611271
DOI: 10.1093/noajnl/vdac057 -
Acta Neuropathologica Feb 2020Pineoblastoma is a rare embryonal tumor of childhood that is conventionally treated with high-dose craniospinal irradiation (CSI). Multi-dimensional molecular evaluation...
Pineoblastoma is a rare embryonal tumor of childhood that is conventionally treated with high-dose craniospinal irradiation (CSI). Multi-dimensional molecular evaluation of pineoblastoma and associated intertumoral heterogeneity is lacking. Herein, we report outcomes and molecular features of children with pineoblastoma from two multi-center, risk-adapted trials (SJMB03 for patients ≥ 3 years; SJYC07 for patients < 3 years) complemented by a non-protocol institutional cohort. The clinical cohort consisted of 58 patients with histologically diagnosed pineoblastoma (SJMB03 = 30, SJYC07 = 12, non-protocol = 16, including 12 managed with SJMB03-like therapy). The SJMB03 protocol comprised risk-adapted CSI (average-risk = 23.4 Gy, high-risk = 36 Gy) with radiation boost to the primary site and adjuvant chemotherapy. The SJYC07 protocol consisted of induction chemotherapy, consolidation with focal radiation (intermediate-risk) or chemotherapy (high-risk), and metronomic maintenance therapy. The molecular cohort comprised 43 pineal parenchymal tumors profiled by DNA methylation array (n = 43), whole-exome sequencing (n = 26), and RNA-sequencing (n = 16). Respective 5-year progression-free survival rates for patients with average-risk or high-risk disease on SJMB03 or SJMB03-like therapy were 100% and 56.5 ± 10.3% (P = 0.007); respective 2-year progression-free survival rates for those with intermediate-risk or high-risk disease on SJYC07 were 14.3 ± 13.2% and 0% (P = 0.375). Of patients with average-risk disease treated with SJMB03/SJMB03-like therapy, 17/18 survived without progression. DNA-methylation analysis revealed four clinically relevant pineoblastoma subgroups: PB-A, PB-B, PB-B-like, and PB-FOXR2. Pineoblastoma subgroups differed in age at diagnosis, propensity for metastasis, cytogenetics, and clinical outcomes. Alterations in the miRNA-processing pathway genes DICER1, DROSHA, and DGCR8 were recurrent and mutually exclusive in PB-B and PB-B-like subgroups; PB-FOXR2 samples universally overexpressed the FOXR2 proto-oncogene. Our findings suggest superior outcome amongst older children with average-risk pineoblastoma treated with reduced-dose CSI. The identification of biologically and clinically distinct pineoblastoma subgroups warrants consideration of future molecularly-driven treatment protocols for this rare pediatric brain tumor entity.
Topics: Adolescent; Age Factors; Brain Neoplasms; Child; Child, Preschool; Cohort Studies; DNA Methylation; Female; Humans; Male; Pineal Gland; Pinealoma; Proto-Oncogene Mas; Risk Factors; Survival Rate; Young Adult
PubMed: 31802236
DOI: 10.1007/s00401-019-02106-9 -
Advances in Experimental Medicine and... 2023Pineal region tumors fall into five broad categories: benign pineal region tumors, glial tumors, papillary tumors, pineal parenchymal tumors, and germ cell tumors....
Pineal region tumors fall into five broad categories: benign pineal region tumors, glial tumors, papillary tumors, pineal parenchymal tumors, and germ cell tumors. Genetic and transcriptional studies have identified key chromosomal alterations in germinomas (RUNDC3A, ASAH1, LPL) and in pineocytomas/pineoblastomas (DROSHA/DICER1, RB1). Pineal region tumors generally present with symptoms of hydrocephalus including nausea, vomiting, papilledema, and the classical Parinaud's triad of upgaze paralysis, convergence-retraction nystagmus, and light-near pupillary dissociation. Workup requires neuroimaging and tissue diagnosis via biopsy. In germinoma cases, diagnosis may be made based on serum or CSF studies for alpha-fetoprotein or beta-HCG making the preferred treatment radiosurgery, thereby preventing the need for unnecessary surgeries. Treatment generally involves three steps: CSF diversion in cases of hydrocephalus, biopsy through endoscopic or stereotactic methods, and open surgical resection. Multiple surgical approaches are possible for approach to the pineal region. The original approach to the pineal region was the interhemispheric transcallosal first described by Dandy. The most common approach is the supracerebellar infratentorial approach as it utilizes a natural anatomic corridor for access to the pineal region. The paramedian or lateral supracerebellar infratentorial approach is another improvement that uses a similar anatomic corridor but allows for preservation of midline bridging veins; this minimizes the chance for brainstem or cerebellar venous infarction. Determination of the optimal approach relies on tumor characteristics, namely location of deep venous structures to the tumor along with the lateral eccentricity of the tumor. The immediate post-operative period is important as hemorrhage or swelling can cause obstructive hydrocephalus and lead to rapid deterioration. Adjuvant therapy, whether chemotherapy or radiation, is based on tumor pathology. Improvements within pineal surgery will require improved technology for access to the pineal region along with targeted therapies that can effectively treat and prevent recurrence of malignant pineal region tumors.
Topics: Humans; Pinealoma; Pineal Gland; Brain Neoplasms; Glioma; Hydrocephalus; Ribonuclease III; DEAD-box RNA Helicases
PubMed: 37452938
DOI: 10.1007/978-3-031-23705-8_6 -
Neuro-Chirurgie 2015Pineal tumor management in pediatric patients must be based on close co-operation between oncologists, surgeons, radiation oncologists, neurologists, ophthalmologists,... (Review)
Review
Pineal tumor management in pediatric patients must be based on close co-operation between oncologists, surgeons, radiation oncologists, neurologists, ophthalmologists, and endocrinologists. Radiation therapy (RT) remains critical in most situations and should be assessed as soon as the diagnosis is made, in order to optimize the radiation technique. This paper will focus on RT modalities, indications, as well as modalities in main pediatric pineal tumors (germ cell tumors and pineal parenchyma tumors). RT modalities are presently being debated and new RT techniques (intensity-modulated RT, proton therapy etc.) that are now available for pineal lesions need to be evaluated. Radiation strategies are also controversial for germ cell tumors: cranio-spinal radiation versus chemotherapy followed by focal radiation, which also requires discussion.
Topics: Brain Neoplasms; Combined Modality Therapy; Humans; Neoplasms, Germ Cell and Embryonal; Pineal Gland; Pinealoma; Treatment Outcome
PubMed: 25612810
DOI: 10.1016/j.neuchi.2014.11.002 -
Neuro-Chirurgie 2015Germ cell tumors (GCTs) classically occur in gonads. However, they are the most frequent neoplasms in the pineal region. The pineal location of GCTs may be caused by the... (Review)
Review
Germ cell tumors (GCTs) classically occur in gonads. However, they are the most frequent neoplasms in the pineal region. The pineal location of GCTs may be caused by the neoplastic transformation of a primordial germ cell that has mismigrated. The World Health Organization (WHO) recognizes 5 histological types of intracranial GCTs: germinoma and non-germinomatous tumors including embryonal carcinoma, yolk sac tumor, choriocarcinoma and mature or immature teratoma. Germinomas and teratomas are frequently encountered as pure tumors whereas the other types are mostly part of mixed GCTs. In this situation, the neuropathologist has to be able to identify each component of a GCT. When diagnosis is difficult, use of recent immunohistochemical markers such as OCT(octamer-binding transcription factor)3/4, Glypican 3, SALL(sal-like protein)4 may be required. OCT3/4 is helpful in the diagnosis of germinomas, Glypican 3 in the diagnosis of yolk sac tumors and SALL4 in the diagnosis of the germ cell nature of an intracranial tumor. When the germ cell nature of a pineal tumor is doubtful, the finding of an isochromosome 12p suggests the diagnosis of GCT. The final pathological report should always be confronted with the clinical data, especially the serum or cerebrospinal fluid levels of β-human chorionic gonadotropin (HCG) and alpha-fetoprotein.
Topics: Biomarkers, Tumor; Brain Neoplasms; Glypicans; Humans; Neoplasms, Germ Cell and Embryonal; Pineal Gland; Pinealoma; Teratoma
PubMed: 24726316
DOI: 10.1016/j.neuchi.2013.06.006 -
Neuro-oncology Practice Apr 2022Pineoblastoma (PB) is a rare malignant brain tumor originating in the pineal gland. Here, we provide a comprehensive epidemiological analysis of PB in the United States...
BACKGROUND
Pineoblastoma (PB) is a rare malignant brain tumor originating in the pineal gland. Here, we provide a comprehensive epidemiological analysis of PB in the United States from 2000 to 2017.
METHODS
Data on 1133 patients with PB were acquired from the Central Brain Tumor Registry of the United States, in collaboration with the Centers for Disease Control and Prevention and the National Cancer Institute, from 2000 to 2017. Age-adjusted incidence rates (AAIRs) per 100 000 and incidence rate ratios (IRRs) were reported for age, sex, race, and ethnicity. Using the National Program of Cancer Registries survival database, median survival and hazard ratios (HRs) were evaluated for overall survival from 2001 to 2016.
RESULTS
Incidence was highest in ages 0-4 years (AAIR: 0.049, 95% CI: 0.042-0.056), decreasing as age increased. Incidence was higher among patients who are Black compared to patients who are White (IRR: 1.71, 95% CI: 1.48-1.98, < .001), and was impacted by age at diagnosis, with Black-to-White incidence highest in children ages 5-9 years (IRR: 3.43, 95% CI: 2.36-4.94, < .001). Overall survival was lower for males (HR: 1.39, 95% CI: 1.07-1.79, = .013). All age groups, excluding those over 40, had improved survival compared to ages 0-4 years. Those who received surgical intervention had better survival compared to those who did not receive surgical treatment.
CONCLUSION
PB incidence is highest among children and patients who are Black, and there may be a potential interaction between these factors. Survival is worse among males, young children, and elderly adults, and those who received no surgery. Comprehensive, population-based statistics provide critical information on PB characteristics that could be useful in impacting patient care and prognosis.
PubMed: 35371520
DOI: 10.1093/nop/npac009 -
Child's Nervous System : ChNS :... Sep 2023Surgical resection of pineal region tumors is challenging because of close proximity to the vein of Galen draining system and the quadrigeminal plate. Surgical resection...
PURPOSE
Surgical resection of pineal region tumors is challenging because of close proximity to the vein of Galen draining system and the quadrigeminal plate. Surgical resection usually is performed through the narrow corridor by piecemeal resection and en bloc resection is difficult in cases of large tumors. Moreover, in cases of hypervascular tumors, surgical resection through the narrow corridor could entail massive intraoperative bleeding. The effectiveness of neoadjuvant chemotherapy and second-look surgery for pineal region tumors for maximal safe resection was evaluated.
METHODS
Retrospective institutional review of pediatric patients with pineal region tumors who underwent second look surgery after neoadjuvant chemotherapy was performed.
RESULTS
Nine patients underwent surgical resection after neoadjuvant chemotherapy over the period of September 2017 to February 2022. The mean age was 7.7 years (ranged from 1.4 to 15.3 years). Three patients underwent partial resection via open craniotomy, and 6 underwent endoscopic biopsy as an initial surgery. The histopathological diagnoses were germ cell tumors in 5 patients (yolk sac tumors in 2, germinoma in 2, choriocarcinoma in 1), /rhabdoid tumor (AT/RT) in 2, medulloblastoma in 1, and high-grade glioneuronal tumor in 1. After several courses of chemotherapy, the second-look surgery was performed. The tumor volume was reduced in 8 patients (89%) after chemotherapy except for 1 case of growing teratoma syndrome. The tumor was extended laterally to the ambient cistern in 2 patients, and posteriorly to the tentorial surface of the cerebellum in 3 patients. The lesion was approached through occipital transtentorial approach in 8 patients and infratentorial supracerebellar approach in 1. Intraoperatively, the high vascularity of the tumor was not observed in all cases. Gross total resection (8 patients, 89%) or near total resection (1, 11%) was achieved in all cases. No complications were observed postoperatively in all cases. Eight patients subsequently underwent additional chemo-radiation therapy according to the initial diagnosis. All patients are alive with no evidence of recurrence with a mean follow-up of 33 months.
CONCLUSIONS
Neoadjuvant chemotherapy and second-look surgery for pediatric pineal region tumors was considered to be effective in reducing the tumor volume and vascularity, which facilitates the safe maximal tumor resection.
Topics: Humans; Child; Infant; Child, Preschool; Adolescent; Second-Look Surgery; Retrospective Studies; Pinealoma; Brain Neoplasms; Pineal Gland; Cerebellar Neoplasms
PubMed: 36181520
DOI: 10.1007/s00381-022-05676-4 -
Chinese Medical Journal Feb 2023
Topics: Humans; Child; Child, Preschool; Pinealoma; Prognosis; Brain Neoplasms; Pineal Gland
PubMed: 36989486
DOI: 10.1097/CM9.0000000000002063 -
Journal of the Egyptian National Cancer... Sep 2021The survival of pineoblastoma patients is low, particularly in infants and those with metastatic disease. This study aimed to analyze the prognostic factors affecting...
BACKGROUND
The survival of pineoblastoma patients is low, particularly in infants and those with metastatic disease. This study aimed to analyze the prognostic factors affecting the outcome of Pineoblastoma in different age groups.
METHODS
A retrospective study included 33 patients. Twenty-two patients older than 3 years had upfront surgery, followed by induction CSI then 6 cycles of chemotherapy. Eleven patients younger than 3 years underwent surgery, followed by induction chemotherapy then radiation therapy. Focal irradiation (54 Gy) was administrated in six patients, and CSI (23.4 Gy) with booster dose 30.6 Gy to the tumor bed in two patients followed by 4 cycles of chemotherapy.
RESULTS
Patient's age showed a significant impact on the outcome (P value = 0.001 for EFS and 0.002 for OS). The metastases' presence did not impact the outcome negatively. The survival of patients with metastatic disease did not differ between age groups. However, age had a significant impact on the outcome of M0 disease, with 3-year EFS and OS of 65.3% and 74%, respectively, in the older group compared to 0% for both rates in younger patients. CSI showed a positive impact on survival. For all cases, the 3-year OS and EFS were 46.7% and 44.4%, respectively.
CONCLUSIONS
A multimodality approach is needed to treat this aggressive disease. Inadequate dose intensity affected our patients' outcome negatively. A more aggressive approach using high-dose chemotherapy or CSI may be required to improve infantile pineoblastoma's dismal outcome. Focal radiotherapy is not an efficacious treatment in infants due to its high-metastatic potential. Molecular typing should be considered to label patients who need a more intensified approach.
Topics: Antineoplastic Combined Chemotherapy Protocols; Brain Neoplasms; Combined Modality Therapy; Humans; Infant; Pineal Gland; Pinealoma; Prognosis; Retrospective Studies; Treatment Outcome
PubMed: 34541625
DOI: 10.1186/s43046-021-00083-3