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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 -
Operative Neurosurgery (Hagerstown, Md.) Jun 2021
Topics: Brain Neoplasms; Hemangioblastoma; Humans; Pineal Gland; Pinealoma
PubMed: 33956942
DOI: 10.1093/ons/opab127 -
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 -
Pediatric Blood & Cancer Apr 2022Pineal anlage tumor is a rare pediatric tumor with clinical and histological features overlapping with pineoblastoma. Two patients with pineal anlage tumor, a...
Pineal anlage tumor is a rare pediatric tumor with clinical and histological features overlapping with pineoblastoma. Two patients with pineal anlage tumor, a 13-month-old female and an 11-month-old male, underwent subtotal resection, high-dose chemotherapy with autologous stem cell rescue, and radiation. Neither had tumor progression 50 months after diagnosis. The tumors underwent next-generation sequencing on a panel of 340 genes. Chromosomal copy gains and losses were present and differed between the tumors. No mutations or amplifications, including none specific to pineoblastoma, were identified.
Topics: Brain Neoplasms; Child; Chromosome Aberrations; Female; Humans; Infant; Male; Mutation; Pineal Gland; Pinealoma; Supratentorial Neoplasms
PubMed: 35129878
DOI: 10.1002/pbc.29596 -
Neurology India 2021Pineal parenchymal tumors account for less than 0.3% of all CNS tumors and "Pineal parenchymal tumor of intermediate differentiation" (PPTID; World Health Organization... (Review)
Review
BACKGROUND
Pineal parenchymal tumors account for less than 0.3% of all CNS tumors and "Pineal parenchymal tumor of intermediate differentiation" (PPTID; World Health Organization (WHO) grades II and III) exhibit intermediary differentiation and prognosis. However "Papillary tumor of the pineal region" (PTPR; WHO grades II and III) is a distinct entity.
OBJECTIVES
This combination of rarity and apparent similarity often leads to perplexity regarding the treatment and prognosis among neurosurgeons. In this review, we have tried to elucidate the differences in clinical as well as treatment modalities and outcomes of these two entities.
METHODS
We used the PubMed Database to search for all relevant articles using the keywords "pineal parenchymal tumor of intermediate differentiation" and "Papillary tumor of the pineal region." Articles having details regarding demographic and clinical variables along with treatment and outcomes were chosen for this study. Full text of these articles was analyzed, and data tabulated.
RESULTS
A total of 25 articles for PPTID and 45 for PTPR were found suitable for inclusion in this study. The studies were either case reports or small retrospective series with only one systemic review for each pathology. Despite the poor quality of data, some trends were apparent. Surgical resection offered a survival benefit in both pathologies. Radiotherapy was effective in increasing the survival in PPTID, while there was little to no effect in PPTR. Chemotherapy was not found to be beneficial in either.
CONCLUSION
Both of these tumors have moderate growth rate and potential for malignant behavior. This continuum of characteristics makes their optimal treatment strategy difficult and confusing. The discussion on comprehensive literature review should give information for neurosurgeons to decide on optimal treatment strategies.
Topics: Brain Neoplasms; Humans; Pineal Gland; Pinealoma; Prognosis; Retrospective Studies
PubMed: 34747779
DOI: 10.4103/0028-3886.329550 -
Child's Nervous System : ChNS :... Sep 2023Pineal region tumours (PRTs) are more common in children and represent a wide variety of lesions. The practise of a radiation test dose is obsolete and a...
INTRODUCTION
Pineal region tumours (PRTs) are more common in children and represent a wide variety of lesions. The practise of a radiation test dose is obsolete and a biochemical/histological diagnosis is recommended before further therapy. Many patients present with hydrocephalus. Advances in neuroendoscopic techniques have allowed safe and effective management of this obstructive hydrocephalus with an opportunity to sample cerebrospinal fluid (CSF) and obtain tissue for histopathology. Definitive surgery is required in less than a third. Endoscopic visualisation and assistance is increasingly used for radical resection, where indicated.
METHODOLOGY
Our experience of endoscopic surgery for paediatric PRTs from 2002 to 2021 is presented. All patients underwent MRI with contrast. Serum tumour markers were checked. If negative, endoscopic biopsy and endoscopic third ventriculostomy (ETV) were performed; and CSF collected for tumour markers and abnormal cells. For radical surgery, endoscope-assisted microsurgery procedures were performed to minimise retraction, visualise the extent of resection and confirm haemostasis.
RESULTS
M:F ratio was 2:1. The median age of presentation was 11 years. Raised ICP (88.88%) was the commonest mode of presentation. Nineteen patients had pineal tumours, one had a suprasellar and pineal tumour, one had disseminated disease, while six had tectal tumours. The ETB diagnosis rate was 95.45%, accuracy rate was 83.3% and ETV success rate was 86.96%.
CONCLUSION
Neuroendoscopy has revolutionised the management of paediatric PRTs. It is a safe and effective procedure with good diagnostic yield and allows successful concurrent CSF diversion, thereby avoiding major surgeries and shunt implantation. It is also helpful in radical resection of lesions, where indicated.
Topics: Child; Humans; Neuroendoscopy; Third Ventricle; Hydrocephalus; Pinealoma; Supratentorial Neoplasms; Ventriculostomy; Brain Neoplasms; Pineal Gland; Treatment Outcome; Retrospective Studies
PubMed: 35665837
DOI: 10.1007/s00381-022-05561-0 -
Indian Journal of Pathology &... 2023Pineocytoma is a rare tumor. It is rare for pineocytoma to present as leptomeningeal metastasis. We present a rare case of pineocytoma with malignant transformation and...
Pineocytoma with malignant transformation to pineal parenchymal tumor with intermediate differentiation and leptomeningeal dissemination after subtotal tumor resection and adjuvant radiotherapy.
Pineocytoma is a rare tumor. It is rare for pineocytoma to present as leptomeningeal metastasis. We present a rare case of pineocytoma with malignant transformation and leptomeningeal metastasis after subtotal tumor resection and adjuvant radiotherapy. This case was a 58-year-old male with an unsteady gait for 2 months. Enhanced brain magnetic resonance imaging revealed a heterogeneous mass involving the pineal region. The initial pathological diagnosis of pineocytoma was confirmed after subtotal tumor resection. Two years after adjuvant radiotherapy to the primary site, the magnetic resonance imaging showed C2 and T2 metastatic lesions, with the final pathological diagnosis being pineal parenchymal tumor (PPT) with intermediate differentiation after the removal of T2 intramedullary tumor. After that adjuvant radiotherapy at the cervical and thoracic spinal cord was completed. There was no recurrence of the tumor 1 year after the radiotherapy. We report a rare case of pineocytoma with malignant transformation to PPT with intermediate differentiation and leptomeningeal dissemination.
Topics: Male; Humans; Middle Aged; Pinealoma; Brain Neoplasms; Radiotherapy, Adjuvant; Pineal Gland; Spinal Cord; Cell Transformation, Neoplastic
PubMed: 36656225
DOI: 10.4103/ijpm.ijpm_817_21 -
European Journal of Radiology Feb 2023To evaluate the effectiveness of diffusion-weighted imaging (DWI) and susceptibility-weighted imaging (SWI) for differentiation between germinoma and other pineal region...
PURPOSE
To evaluate the effectiveness of diffusion-weighted imaging (DWI) and susceptibility-weighted imaging (SWI) for differentiation between germinoma and other pineal region tumors.
METHOD
This retrospective study consisted of 72 patients with pathologically proven pineal region tumors between January 2010 and August 2020. Tumors were classified as germinomas (40), non-germinomatous germ cell tumors (11) (NGGCT), pineal parenchymal tumors (10) (PPT), and other types of tumors (11). Visual scale score, ADC values and SWI intratumoral susceptibility signal (ITSS) score were analyzed and compared to histopathology data.
RESULTS
The mean apparent diffusion coefficient (ADCmean) and minimum apparent diffusion coefficient (ADCmin) ratio of germinoma were significantly lower than NGGCT. ADCmean or ADCmin cut-off ratio of ≤ 1.48 or ≤ 1.32 allowed for discrimination between germinoma and NGGCT with sensitivity and specificity of 100 % and 63.6 %. An ADCmin cut-off ratio of ≥ 0.93 allowed for discrimination between germinoma and PPT with sensitivity and specificity of 60 % and 80.0 %. ADCmin cut-off ratio of ≤ 1.15 allowed for discrimination of germinoma from other types of tumors with sensitivity and specificity of 87.5 % and 54.5 %.
CONCLUSIONS
ADC ratio can differentiate germinoma from other types of pineal region tumors. Our initial results suggest that ITSS score was not significantly correlated with specific histology subtype.
Topics: Humans; Pinealoma; Retrospective Studies; Magnetic Resonance Imaging; Diffusion Magnetic Resonance Imaging; Germinoma; Neoplasms, Germ Cell and Embryonal; Cell Differentiation; Brain Neoplasms; Pineal Gland
PubMed: 36584565
DOI: 10.1016/j.ejrad.2022.110663 -
Brain Tumor Research and Treatment Oct 2023This study aims to elucidate clinical features, therapeutic strategies, and prognosis of pineal parenchymal tumors (PPT) by analyzing a 30-year dataset of a single...
BACKGROUND
This study aims to elucidate clinical features, therapeutic strategies, and prognosis of pineal parenchymal tumors (PPT) by analyzing a 30-year dataset of a single institution.
METHODS
We reviewed data from 43 patients diagnosed with PPT at Seoul National University Hospital between 1990 and 2020. We performed survival analyses and assessed prognostic factors.
RESULTS
The cohort included 10 patients with pineocytoma (PC), 13 with pineal parenchymal tumor of intermediate differentiation (PPTID), and 20 with pineoblastoma (PB). Most patients presented with hydrocephalus at diagnosis. Most patients underwent an endoscopic third ventriculostomy and biopsy, with some undergoing additional resection after diagnosis confirmation. Radiotherapy was administered with a high prevalence of gamma knife radiosurgery for PC and PPTID, and craniospinal irradiation for PB. Chemotherapy was essential in the treatment of grade 3 PPTID and PB. The 5-year progression-free survival rates for PC, grade 2 PPTID, grade 3 PPTID, and PB were 100%, 83.3%, 0%, and 40%, respectively, and the 5-year overall survival rates were 100%, 100%, 40%, and 55%, respectively. High-grade tumor histology was associated with lower survival rates. Significant prognostic factors varied among tumor types, with World Health Organization (WHO) grade and leptomeningeal seeding (LMS) for PPTID, and the extent of resection and LMS for PB. Three patients experienced malignant transformations.
CONCLUSION
This study underscores the prognostic significance of WHO grades in PPT. It is necessary to provide specific treatment according to tumor grade. Grade 3 PPTID showed a poor prognosis. Potential LMS and malignant transformations necessitate aggressive multimodal treatment and close-interval screening.
PubMed: 37953449
DOI: 10.14791/btrt.2023.0033 -
Journal of Neuro-oncology Nov 2022Pineal parenchymal tumor of intermediate differentiation (PPTID) is a neoplasm of pinealocyte origin and of intermediate differentiation (WHO grade 2 or 3). Treatment... (Review)
Review
PURPOSE
Pineal parenchymal tumor of intermediate differentiation (PPTID) is a neoplasm of pinealocyte origin and of intermediate differentiation (WHO grade 2 or 3). Treatment selection and prognostication is challenging for this rare, recently characterized tumor. In this single study, we review our clinical experience in patients with PPTID as well as pooled data from two other institutions.
METHODS
This is a retrospective analysis of patients seen at Mayo Clinic, with additional data pooled from two similar studies at outside institutions for comparison and further analysis.
RESULTS
Ten adult patients (6 male) were identified. Median age at diagnosis was 36 years (range 13-73 years). Patients were followed between 3 and 88 months with no reported deaths. The most common presenting symptom was headache, followed by nausea. Nine patients had local disease without neuroaxial dissemination at diagnosis, one of which had tumor recurrence at 36 months. Two patients underwent gross total resection (GTR) without adjuvant radiation without recurrence (PFS 4 and 46 months). In our combined analysis, we reviewed 36 patients from three different institutions. Four patients presented with disseminated disease. GTR was achieved for 16 patients. Twenty-eight patients received adjuvant radiation therapy and 16 patients experienced disease recurrence. Median overall survival was 44 months.
CONCLUSION
Our single institution experience and combined multi-institution analysis suggest GTR is associated with improved outcomes. The role of adjuvant radiation therapy and utility of CSI compared to focal RT is less unclear. Prospective study is required to identify optimal adjuvant therapy selection.
Topics: Adult; Humans; Male; Adolescent; Young Adult; Middle Aged; Aged; Pinealoma; Retrospective Studies; Neoplasm Recurrence, Local; Pineal Gland; Brain Neoplasms
PubMed: 36301435
DOI: 10.1007/s11060-022-04174-6