-
World Neurosurgery May 2023Obtaining a prompt diagnosis, avoiding indwelling ventriculoperitoneal shunt, and enhancing the predictive value of pathologic examinations are only some of the... (Review)
Review
Obtaining a prompt diagnosis, avoiding indwelling ventriculoperitoneal shunt, and enhancing the predictive value of pathologic examinations are only some of the advantages conferred by a simultaneous third ventriculostomy and tumor biopsy in patients with pineal region tumors. The objective of this study was to retrospectively search the literature on concomitant, single burr hole endoscopic third ventriculostomy (ETV) and tumor biopsy (TB) for pineal region tumors and to analyze the feasibility, surgical safety, and benefits of these 2 combined procedures. Consequently, a comprehensive, systematic literature search was performed in compliance with the updated PRISMA 2020 guidelines within electronic databases MEDLINE/PubMed, EMBASE, PLOS, and Cochrane Library. Statistical analysis was performed with IBM SPSS 28.0.1.1(14), using Kendall's and Spearman's tests, with a P < 0.05 considered significant. A total of 25 studies were selected and included in this review, for a total of 368 patients (mean age 20.6 years; range 1-86 years; SD 17.5). More than two-thirds of the procedures were operated with a rigid endoscope and 27.6% were performed with either a flexible endoscope, a combination of the 2, or not otherwise specified. Germinoma represented the most frequent diagnosis (20.1%) followed by astrocytoma (12.9%) and pineocytoma (9.9%). The single-entry approach allowed a correct histologic diagnosis in 88.7% of the examined cases. Summing up, concomitant ETV and TB represent a valuable option for the management of non-communicating hydrocephalus and the initial assessment of pineal region tumors. The histologic confirmation rate was 88.7% in the examined cohort, with only 10% of the biopsies yielding inconclusive results.
Topics: Humans; Young Adult; Adult; Ventriculostomy; Retrospective Studies; Feasibility Studies; Third Ventricle; Pineal Gland; Pinealoma; Neuroendoscopy; Biopsy; Hydrocephalus; Brain Neoplasms
PubMed: 36764448
DOI: 10.1016/j.wneu.2023.01.082 -
International Journal of Surgery Case... Feb 2023Intracranial germinomas are germ cell tumors that commonly develop in the pineal or neurohypophysis regions. As ectopic germinomas are rarely observed within the...
INTRODUCTION AND IMPORTANCE
Intracranial germinomas are germ cell tumors that commonly develop in the pineal or neurohypophysis regions. As ectopic germinomas are rarely observed within the cerebrum and are associated with atypical image findings, diagnosis is challenging.
CASE PRESENTATION
A 14-year-old boy was admitted to our hospital with complaints of vomiting and headache. Gadolinium-enhanced magnetic resonance imaging revealed ring-enhancing lesions in his left frontal lobe and basal ganglia. Susceptibility-weighted imaging indicated that the subependymal veins passing through the lesion centers were engorged, while electrophoretic analysis of cerebrospinal fluid identified oligoclonal bands (OCBs); both were typical of multiple sclerosis (MS). Tumor biopsy revealed many cells with atypical mitotic figures and nuclear enlargements, suggesting malignant disease. As the tumor rapidly proliferated, we opted for surgical excision of the lesions. Histopathological analyses revealed "two-cell patterns" characteristic of germinoma. Immunohistochemistry was positive for placental alkaline phosphatase and c-KIT. The definitive diagnosis was germinoma. After chemoradiotherapy, the patient was discharged without neurological deficits.
CLINICAL DISCUSSION
OCBs and several magnetic resonance imaging features (including open ring enhancement, T2 hypointense rims, mild mass effects, mild perilesional edema, peripheral restriction around the lesion, and vessel-like structures running through the lesion center) are useful diagnostic signs for the radiological discrimination of MS from germinoma. However, owing to these factors, some cases are difficult to diagnose.
CONCLUSION
Our case report of an unusual ectopic cerebral germinoma illustrates the difficulty of distinguishing it from MS. Therefore, we recommend proper tissue sampling in such cases, especially in adolescent patients, to make definitive germinoma diagnoses.
PubMed: 36696842
DOI: 10.1016/j.ijscr.2023.107884 -
Chinese Neurosurgical Journal Jan 2023The goal of this study is to show the feasibility and benefits of using the simultaneous biportal endoscopic procedure to treat pineal tumors in patients with...
BACKGROUND
The goal of this study is to show the feasibility and benefits of using the simultaneous biportal endoscopic procedure to treat pineal tumors in patients with obstructive hydrocephalus.
METHODS
We retrospectively reviewed three patients with pineal tumors and acute obstructive hydrocephalus who were treated in one session with a frameless stereotactic guided simultaneous biportal endoscopic third ventriculostomy and endoscopic tumor biopsy performed through two separate ports using one rigid ventriculoscope.
RESULTS
In the three patients, ventriculostomy and endoscopic biopsies were conducted. There was no death or morbidity throughout the 45-min procedure. All of the patients' histological findings were confirmed. Germinoma was diagnosed in two patients who recieved postoperative radiotherapy, and the third patient diagnosed with a pineocytoma. Magnetic resonance imaging with flow-sensitive sequences was used to confirm ventriculostomy patency in all patients 6 months after the surgery.
CONCLUSION
Biportal endoscopic approach enables better visual control of both procedures. Furthermore, it allows the surgeon to safely pass the ventriculoscope via the foramen of monro, even if it is narrow. Moreover, during endoscopic tumor biopsy and third ventriculostomy, the intracranial pressure can be smoothly managed using the outlet tubes accessible. This treatment may be an alternative to traditional uniportal endoscopic operations in certain patients.
PubMed: 36624512
DOI: 10.1186/s41016-022-00313-0 -
Child's Nervous System : ChNS :... Feb 2023Intracranial germ cell tumor (iGCT) is a rare disorder and often occurs during childhood and adolescence. iGCTs are frequently localized in pineal region and... (Review)
Review
INTRODUCTION
Intracranial germ cell tumor (iGCT) is a rare disorder and often occurs during childhood and adolescence. iGCTs are frequently localized in pineal region and hypothalamic-neurohypophyseal axis (HNA). In spite of well-established clinical and pathological entity, histogenesis of iGCTs remains unsettled. Current theories of histogenesis of iGCTs include germ cell theory (from primordial germ cells (PGCs) of aberrant migration) and stem cell theory (transformed embryonic stem (ES) cells). In order to comprehend the histogenesis, we revisit the origin, migration, and fate of the human PGCs, and their transformation processes to iGCT.
DISCUSSION
In "germ cell theory," transformation of ectopic PGCs to iGCT is complex and involves multiple transcription factors. Germinoma is derived from ectopic PGCs and is considered a prototype of all GCTs. Non-germinomatous germ cell tumors (NGGCTs) develop from more differentiated counterparts of embryonic and extra-embryonic tissues. However, there is a distinct genomic/epigenomic landscape between germinoma and NGGCT. ES cells transformed from ectopic PGCs through molecular dysregulation or de-differentiation may become the source of iGCT. "Stem cell theory" is transformation of endogenous ES cells or primitive neural stem cell to iGCTs. It supports histological diversity of NGGCTs because of ES cell's pluripotency. However, neural stem cells are abundantly present along the subependymal zone; therefore, it does not explain why iGCTs almost exclusively occur in pineal and HNA locations. Also, the vast difference of methylation status between germinoma and NGGCT makes it difficult to theorize all iGCTs derive from the common cellular linage.
CONCLUSION
Transformation of PGCs to ES cells is the most logical mechanism for histogenesis of iGCT. However, its detail remains an enigma and needs further investigations.
Topics: Adolescent; Humans; Brain Neoplasms; Neoplasms, Germ Cell and Embryonal; Germinoma; Embryonic Stem Cells
PubMed: 36595083
DOI: 10.1007/s00381-022-05808-w -
Journal of Cancer Research and... Jan 2023Germ cell tumor of the central nervous system (CNS) is an infrequent entity consisting of only 0.2%-1.7% of all primary CNS tumors. The pineal gland is the commonest... (Review)
Review
Germ cell tumor of the central nervous system (CNS) is an infrequent entity consisting of only 0.2%-1.7% of all primary CNS tumors. The pineal gland is the commonest location of CNS germinoma. Traditionally, radiotherapy alone has been used to treat localized pineal germinoma, which has delivered a very high cure rate. Spinal drop metastases from pineal germinoma can develop after a long time from diagnosis and primary treatment. Currently, craniospinal irradiation is the standard of care in metastatic pineal germinoma with spinal drop metastases along with systemic chemotherapy. Very few cases of pineal germinoma with spinal drop metastases have been published in the literature. We report a pineal gland germinoma case with spinal drop metastases in an 18-year-old boy and reviewed the published literature.
Topics: Male; Humans; Adolescent; Pineal Gland; Germinoma; Brain Neoplasms; Neoplasms, Germ Cell and Embryonal
PubMed: 38384076
DOI: 10.4103/jcrt.jcrt_657_22 -
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 -
Pediatric Blood & Cancer Mar 2023This study analysed the clinical features of a cohort of children with intracranial germ cell tumours (IC-GCTs). We retrospectively reviewed timelag between symptoms...
AIMS
This study analysed the clinical features of a cohort of children with intracranial germ cell tumours (IC-GCTs). We retrospectively reviewed timelag between symptoms onset, clinic-radiological findings, diagnosis and outcomes.
METHODS
Symptoms at diagnosis were divided into four groups: (1) raised intracranial pressure (RICP); (2) visual impairment; (3) endocrinopathies; (4) other. Total diagnostic interval (TDI), defined as the interval between symptom onset (including retrospective recall of symptoms) and definitive diagnosis of IC-GCT, was calculated and compared to survival rates.
RESULTS
Our cohort included 55 children with median follow-up of 78.9 months (0.5-249.9). The majority (63.6%) had germinomas and 10.9% were metastatic at diagnosis. IC-GCTs were suprasellar (41.8%), pineal (36.4%), bifocal (12.7%) or in atypical sites (9.1%). The most common presenting symptoms were related to RICP (43.6%); however, by the time of tumour diagnosis, 50.9% of patients had developed endocrine dysfunctions. All pineal GCTs manifested with RICP or visual impairment. All suprasellar GCTs presented with endocrinopathies. TDI ranged between 0.25 and 58.5 months (median 4 months). Pineal GCTs had the shortest TDI (median TDI 1 month versus 24 months in suprasellar GCTs, p < .001). TDI > 6 months was observed in 47.3% of patients and was significantly associated with endocrine presenting symptoms. No statistically significant difference was found in progression-free survival and overall survival between patients with TDI > 6 months and with TDI ≤ 6 months.
CONCLUSION
Approximately half of the IC-GCT patients in this cohort had TDI > 6 months. These presented mostly with endocrine deficits. TDI > 6 months was not associated with increased relapse or mortality rates.
Topics: Child; Humans; Retrospective Studies; Brain Neoplasms; Neoplasm Recurrence, Local; Neoplasms, Germ Cell and Embryonal; Endocrine System Diseases; Vision Disorders
PubMed: 36582128
DOI: 10.1002/pbc.30168 -
Neurological Research Apr 2023Neuroendoscopic resection supracerebellar infratentorial (SCIT) approach is adequate for some indicated pineal region tumors with the natural infratentorial corridor.... (Review)
Review
OBJECTIVE
Neuroendoscopic resection supracerebellar infratentorial (SCIT) approach is adequate for some indicated pineal region tumors with the natural infratentorial corridor. We described this full endoscopic approach through a modified 'head-up' park-bench position to facilitate the procedure.
METHODS
We reviewed the clinical and radiological data of four patients with pineal region lesions who underwent pure endoscopic tumor resection through the SCIT approach with this modified position. The related literature concerning fully endoscopic pineal region tumor resection was also reviewed.
RESULTS
This cohort included four patients with pineal region tumors. External ventricular drainage (Ommaya reservoir) was performed in three patients with hydrocephalus in advance. The average tumor volume was 19.2 ± 17.2 cm. Pathological examination confirmed two mixed germinomas, one glioblastoma multiforme, and one hemangioblastoma. Gross total resection (GTR) was achieved in all patients, and all patients recovered well without neurological deficits or surgical complications. Hydrocephalus was relieved among all patients.
CONCLUSIONS
The pure endoscopic SCIT approach could enable safe and effective resection of pineal region tumors, even for relatively large lesions. The endoscope could provide a panoramic view and illumination of the deep-seated structures. Compared with the sitting position, this modified ergonomic position could be implemented easily.
Topics: Humans; Sitting Position; Neurosurgical Procedures; Pinealoma; Pineal Gland; Neuroendoscopy; Brain Neoplasms
PubMed: 36509700
DOI: 10.1080/01616412.2022.2146266 -
Computers in Biology and Medicine Jan 2023Pineal region tumors (PRTs) are highly histologically heterogeneous. Germinoma is the most common PRT and is treatable with radiotherapy and chemotherapy. A non-invasive...
BACKGROUND
Pineal region tumors (PRTs) are highly histologically heterogeneous. Germinoma is the most common PRT and is treatable with radiotherapy and chemotherapy. A non-invasive system that helps identify germinoma in the pineal region could reduce lab exams and traumatic therapies.
METHODS
In this retrospective study, 122 patients with histologically confirmed PRTs and pre-operative multi-modal MR images were included. Radiomics features were extracted from different ROIs and image sequences separately. A computational framework that combines a few classification and feature selection algorithms were used to predict histology with radiomics features and demographics. We systemically benchmarked performance of models with feature matrices from all possible combinations of ROIs and image sequences. The Area under the ROC Curve (AUC) was then used to evaluate model performance.
RESULTS
Models with demographics and radiomics features outperform radiomics-only or demographics-only models. The best demographical-radiomics model reached the highest AUC of 0.88 (CI95%: 0.81-0.96). Through the comprehensive evaluation of possible sequence combinations in the differential diagnosis of pineal tumor, T1 and T2 emerged as the most informative sequences for the task. There is imbalanced usage of feature classes as we analyze their proportion in all models.
CONCLUSIONS
The demographical-radiomics model can accurately and efficiently identify germinomas in the pineal region. The preference for MRI sequences, radiomics feature classes, features selection and classification algorithms provide a valuable reference for future attempts at developing classifiers on medical images.
Topics: Humans; Retrospective Studies; ROC Curve; Magnetic Resonance Imaging; Machine Learning; Germinoma
PubMed: 36470145
DOI: 10.1016/j.compbiomed.2022.106366 -
Radiology Case Reports Jan 2023Germinoma is a rare CNS germ cell tumor preferentially affecting children and young adults. Intracranial germinomas arise typically in the neurohypophysis and pineal...
Germinoma is a rare CNS germ cell tumor preferentially affecting children and young adults. Intracranial germinomas arise typically in the neurohypophysis and pineal region and occasionally in the basal ganglia and thalamus. Germinomas in the basal ganglia and thalamus are characterized by the ipsilateral cerebral and brainstem hemiatrophy with slowly progressive neurological deficits, which is due to tumor infiltration into the thalamocortical and corticospinal tract and induction of anterograde and retrograde Wallerian degeneration. We report an 11-year-old boy with a mass located in the fornix incidentally discovered on the first work-up of his minor head injury. Imaging findings revealed the ipsilateral atrophy of the mammillary body and the fornix. Stereotactic brain biopsy was performed and the final diagnosis was germinoma. The ipsilateral atrophy of the mammillary body and the fornix implied the transsynaptic degeneration via the Papez circuit. We discuss the unique nature of germinomas and underlying pathological mechanisms.
PubMed: 36425391
DOI: 10.1016/j.radcr.2022.10.064