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Obstetric Medicine Sep 2021Pineoblastoma is an extremely rare intracranial neoplasm, with increased risk of craniospinal metastasis. There is only one case reported in the literature who presented...
Pineoblastoma is an extremely rare intracranial neoplasm, with increased risk of craniospinal metastasis. There is only one case reported in the literature who presented during pregnancy. Described here is a woman who presented at five months of gestation with recurrence of pineoblastoma, who had previously defaulted adjuvant therapy following surgical decompression. The challenges in the diagnosis and treatment of pineoblastoma and its effects on pregnancy are also discussed.
PubMed: 34646348
DOI: 10.1177/1753495X20958339 -
Brain Pathology (Zurich, Switzerland) Jan 2000The WHO classification of CNS tumors divides pineal parenchymal tumors (PPT) into pineocytoma (PC), pineoblastoma (PB) and mixed pineocytoma-pineoblastoma or PPT with... (Review)
Review
The WHO classification of CNS tumors divides pineal parenchymal tumors (PPT) into pineocytoma (PC), pineoblastoma (PB) and mixed pineocytoma-pineoblastoma or PPT with intermediate differentiation. The reported incidence of mixed/intermediate PPT varies and this may reflect the difficulty in classifying tumors of this type. In an attempt to overcome the problem of the classification of PPT with intermediate differentiation, we describe the relationship between histological features and patient survival in a large cooperative series of 66 PPT from 12 neurosurgical centres. All tumors were studied with both light microscopy and immunohistochemically using antibodies against glial markers or neural/neuroendocrine markers. Our series included 11 PC, 39 mixed/intermediate PPT and 16 PB. A number of mitoses greater than 6 and the presence of necrosis were associated with a poorer outcome, while positive immunostaining for neurofilaments was associated with a better survival. We propose a new prognostic grading of 4 grades, grade I for PC, grade II for PPT with fewer than 6 mitoses and positive immunolabelling for neurofilaments, grade III for PPT with either 6 or more than 6 mitoses or fewer than 6 mitoses but without immunostaining for neurofilaments and grade IV for PB.
Topics: Adolescent; Adult; Aged; Brain Neoplasms; Child; Child, Preschool; Female; Humans; Immunohistochemistry; Infant; Male; Middle Aged; Pineal Gland; Pinealoma; Prognosis
PubMed: 10668895
DOI: 10.1111/j.1750-3639.2000.tb00242.x -
Frontiers in Oncology 2020Pineoblastoma (PB) is a rare neoplasm of the central nervous system. This analysis aimed to identify factors and establish a predictive model for the prognosis of adult...
Pineoblastoma (PB) is a rare neoplasm of the central nervous system. This analysis aimed to identify factors and establish a predictive model for the prognosis of adult patients with PB. Data for 213 adult patients with PB (Surveillance, Epidemiology, and End Results database) were randomly divided into primary and validation cohorts. A predictive model was established and optimized based on the Akaike Information Criterion and visualized by a nomogram. Its predictive performance (concordance index and receiver operating characteristic curve) and clinical utility (decision curve analyses) were evaluated. We internally and externally validated the model using calibration curves. Multivariate Cox regression analysis identified age, year of diagnosis, therapy, tumor size, and tumor extension as independent predictors of PB. The model exhibited great discriminative ability (concordance index of the nomogram: 0.802; 95% confidence interval: 0.78-0.83; area under the receiver operating characteristic curve: ranging from 0.7 to 0.8). Calibration plots (probability of survival) showed good consistency between the actual observation and the nomogram prediction in both cohorts, and the decision curve analyses demonstrated great clinical utility of the nomogram. The nomogram is a useful and practical tool for evaluating prognosis and determining appropriate therapy strategies.
PubMed: 32793463
DOI: 10.3389/fonc.2020.01021 -
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 -
The Neuroradiology Journal Apr 2015Diffusion weighted imaging (DWI) has been shown to be helpful in characterizing tumor cellularity, and predicting histology. Several works have evaluated this technique...
BACKGROUND
Diffusion weighted imaging (DWI) has been shown to be helpful in characterizing tumor cellularity, and predicting histology. Several works have evaluated this technique for pineal tumors; however studies to date have not focused on pediatric pineal tumors.
OBJECTIVE
We evaluated the diffusion characteristics of pediatric pineal tumors to confirm if patterns seen in studies using mixed pediatric and adult populations remain valid.
MATERIALS AND METHODS
This retrospective study was performed after Institutional Review Board approval. We retrospectively evaluated all patients 18 years of age and younger with pineal tumors from a single institution where preoperative diffusion weighted imaging as well as histologic characterization was available.
RESULTS
Twenty patients (13 male, 7 female) with pineal tumors were identified: seven with pineoblastoma, four with Primitive Neuroectodermal Tumor (PNET), two with other pineal tumors, and seven with germ cell tumors including two germinomas, three teratomas, and one mixed germinoma-teratoma. The mean apparent diffusion coefficient (ADC) values in pineoblastoma (544 ± 65 × 10⁻⁶ mm²/s) and pineoblastoma/PNET (595 ± 144 × 10⁻⁶ mm²/s) was lower than that of the germ cell tumors (1284 ± 334 × 10⁻⁶ mm²/s; p < 0.0001 vs pineoblastoma). One highly cellular germinoma had an ADC value of 694 × 10⁻⁶ mm²/s.
CONCLUSION
ADC values can aid in differentiation of pineoblastoma/PNET from germ cell tumors in a population of children with pineal masses.
Topics: Adolescent; Brain Neoplasms; Child; Child, Preschool; Diffusion Magnetic Resonance Imaging; Female; Humans; Infant; Male; Pineal Gland; Pinealoma; Reproducibility of Results; Retrospective Studies; Sensitivity and Specificity
PubMed: 25963154
DOI: 10.1177/1971400915581741 -
International Journal of Surgery... Dec 2023The pineal region tumors are challenging for neurosurgeons and can lead to secondary hydrocephalus. The introduction of the exoscope has provided clinical interventions...
BACKGROUND
The pineal region tumors are challenging for neurosurgeons and can lead to secondary hydrocephalus. The introduction of the exoscope has provided clinical interventions with high image quality and an ergonomic system for pineal region tumor operations. In this study, the authors describe the exoscopic approach used to facilitate the surgical resection of pineal region tumors and relieve hydrocephalus.
MATERIALS AND METHODS
In this retrospective cohort study, we consecutively reviewed the clinical and radiological data of 25 patients with pineal region lesions who underwent three-dimensional exoscopic tumor resection at a single center.
RESULTS
The patient cohort consisted of 16 males and 9 females, with an average age of 34.6 years (range, 6-62 years; 8 cases aged ≤18). Pathological examination confirmed eight pineal gland tumors, four gliomas, nine germ cell neoplasms, two ependymomas, and two metastatic tumors. Preoperative hydrocephalus was present in 23 patients. Prior to tumor resection, external ventricular drainage (EVD) with Ommaya reservoir implantation was performed in 17 patients. Two patients received preoperative endoscopic third ventriculostomy (ETV), and five patients received a ventriculoperitoneal (VP) shunt, including one who received both procedures. Gross total resection was achieved in 19 patients (76%) in the 'head-up' park bench position using the exoscope. Eight patients (31.6%) with third ventricle invasion received subtotal resection, mainly in glioma cases, which was higher than those without invasion (0%), but not statistically significant ( P =0.278, Fisher's exact test). No new neurological dysfunction was observed after surgery. Two patients (8%) developed intracranial and pulmonary infections, and two patients (8%) suffered from pneumothorax. Hydrocephalus was significantly relieved in all patients postoperatively, and four patients with relapse hydrocephalus were cured during the long-term follow-up. Postoperative adjuvant management was recommended for indicated patients, and a mean follow-up of 24.8±14.3 months showed a satisfied outcome.
CONCLUSIONS
The exoscope is a useful tool for pineal region tumor resection and hydrocephalus relief, particularly with posterior third ventricle invasion, as total resection could be achieved without obvious complication. The special superiority of the exoscope for the indicated pineal region tumors should be highlighted.
Topics: Male; Female; Humans; Adult; Pinealoma; Retrospective Studies; Treatment Outcome; Neoplasm Recurrence, Local; Pineal Gland; Glioma; Ventriculostomy; Third Ventricle; Hydrocephalus; Brain Neoplasms
PubMed: 37755386
DOI: 10.1097/JS9.0000000000000707 -
Unedited microneurosurgery of a high-grade pineal parenchymal tumor of intermediate differentiation.Surgical Neurology International 2018WHO Grade II-III pineal parenchymal tumors of intermediate differentiation (PPTIDs) were included in the 2007 World Health Organization Classification of Central Nervous...
BACKGROUND
WHO Grade II-III pineal parenchymal tumors of intermediate differentiation (PPTIDs) were included in the 2007 World Health Organization Classification of Central Nervous System Tumors as pineal parenchymal tumors between pineocytomas and pineoblastomas. PPTIDs comprise more than 20-60% of all pineal parenchymal tumors (PPT) s and are characterized by moderately high cellularity, mild-to-moderate nuclear atypia, and low-to-moderate mitotic activity. Moreover, PPTID includes transitional cases in which pineocytomatous and pineoblastoma features are associated. Synaptophysin and neuron-specific enolase are usually positive, with variable reactivity to neurofilament protein, chromogranin A, retinal S-antigen, and S-100 protein. PPTID Grades II and III can be distinguished on the basis of mitotic activity (higher in high-grade PPTID) and neurofilament protein immunoreactivity (higher in low-grade PPTID). Herein, we present the microsurgical management of a histologically confirmed high-grade PPTID.
CASE DESCRIPTION
A patient with high grade PPTID underwent sitting praying position and right paramedian supracerebellar infratentorial approach. The lesion was identified after lateral opening of the quadrigeminal cistern, followed by removal of its cystic component. Tissue samples were obtained under high microscopic magnification, and internal debulking of the tumor was performed with ring microforceps and bipolar forceps in the right hand and a thumb-regulated suction tube in the left hand. Continuous water irrigation provided us a clean surgical field and the recognition of small bleeding vessels. Moreover, water dissection technique was applied to recognize the cleavage plane of the tumor. Bipolar coagulation forceps were used to shrink the tumor and remove it by piecemeal reduction aiming to identify the anterior and lateral limits of the lesion. The poorly differentiated borders between the tumor and the surrounding parenchyma were determined under microscopic vision. Small vessels feeding the tumor were coagulated and cut. The most critical surgical stage was related with removal of some tumor remnants attached to the internal cerebral veins. A meticulous and skillful dissection was essential aiming to preserve these vascular structures. The final steps included meticulous hemostasis with electrocoagulation, Tachosil, and Surgicel. The postoperative course was uneventful. The patient underwent adjuvant radiotherapy and currently is alive, free of tumor recurrence 4 years after surgery.
CONCLUSION
This unedited video offers all detailed aspects that are, as the senior author JH considers, essential for a neurosurgeon when performing an efficient and safe surgery for a high-grade PPTID.
VIDEOLINK
http://surgicalneurologyint.com/videogallery/pineal-tumor-2.
PubMed: 30603232
DOI: 10.4103/sni.sni_353_18 -
BioRxiv : the Preprint Server For... Apr 2024Mutations in the microRNA processing genes and drive several cancers that resemble embryonic progenitors. To understand how microRNAs regulate tumorigenesis, we...
Mutations in the microRNA processing genes and drive several cancers that resemble embryonic progenitors. To understand how microRNAs regulate tumorigenesis, we ablated or in the developing pineal gland to emulate the pathogenesis of pineoblastoma, a brain tumor that resembles undifferentiated precursors of the pineal gland. Accordingly, these mice develop pineal tumors marked by loss of microRNAs, including the let-7/miR-98-5p family, and de-repression of microRNA target genes. Pineal tumors driven by loss of or mimic tumors driven by loss, as they exhibit upregulation of S-phase genes and homeobox transcription factors that regulate pineal development. Blocking proliferation of these tumors facilitates expression of pinealocyte maturation markers, with a concomitant reduction in embryonic markers. Select embryonic markers remain elevated, however, as the microRNAs that normally repress these target genes remain absent. One such microRNA target gene is the oncofetal transcription factor , which regulates expression of pro-growth genes, and inhibiting their signaling impairs tumor growth. Thus, we demonstrate that tumors driven by loss of microRNA processing may be therapeutically targeted by inhibiting downstream drivers of proliferation.
PubMed: 38712047
DOI: 10.1101/2024.04.23.590638 -
Neuro-oncology Advances 2022
PubMed: 35821675
DOI: 10.1093/noajnl/vdac092 -
Journal of Neuro-oncology Jan 2024To provide a treatment-focused review and develop basic treatment guidelines for patients diagnosed with pineal anlage tumor (PAT).
PURPOSE
To provide a treatment-focused review and develop basic treatment guidelines for patients diagnosed with pineal anlage tumor (PAT).
METHODS
Prospectively collected data of three patients with pineal anlage tumor from Germany was combined with clinical details and treatment information from 17 published cases.
RESULTS
Overall, 20 cases of PAT were identified (3 not previously reported German cases, 17 cases from published reports). Age at diagnosis ranged from 0.3 to 35.0 (median: 3.2 ± 7.8) years. All but three cases were diagnosed before the age of three years. For three cases, metastatic disease at initial staging was described. All patients underwent tumor surgery (gross-total resection: 9, subtotal resection/biopsy: 9, extent of resection unknown: 2). 15/20 patients were alive at last follow-up. Median follow-up for 10/15 surviving patients with available follow-up and treatment data was 2.4 years (0.3-6.5). Relapse was reported for 3 patients within 0.8 years after diagnosis. Five patients died, 3 after relapse and 2 from early postoperative complications. Two-year-progression-free- and -overall survival were 65.2 ± 12.7% and 49.2 ± 18.2%, respectively. All 4 patients who received intensive chemotherapy including high-dose chemotherapy combined with radiotherapy (2 focal, 2 craniospinal [CSI]) had no recurrence. Focal radiotherapy- and CSI-free survival rates in 13 evaluable patients were 46.2% (6/13) and 61.5% (8/13), respectively.
CONCLUSION
PAT is an aggressive disease mostly affecting young children. Therefore, adjuvant therapy using intensive chemotherapy and considering radiotherapy appears to comprise an appropriate treatment strategy. Reporting further cases is crucial to evaluate distinct treatment strategies.
Topics: Adolescent; Adult; Child; Child, Preschool; Humans; Infant; Young Adult; Brain Neoplasms; Neoplasm Recurrence, Local; Pineal Gland; Pinealoma; Recurrence; Supratentorial Neoplasms; Treatment Outcome
PubMed: 38253790
DOI: 10.1007/s11060-023-04547-5