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Radiology Case Reports Sep 2022Tumors of the pineal region are a rare clinical entity, comprising approximately 3%-8% of pediatric tumors. Based on their histopathological features, they are typically...
Tumors of the pineal region are a rare clinical entity, comprising approximately 3%-8% of pediatric tumors. Based on their histopathological features, they are typically classified as pineal parenchymal tumors and germ cell tumors, with the latter being more prevalent. Clinical presentation is heterogeneous, with symptoms arising either due to tumor invasion or compression of adjacent neurovascular structures and increased intracranial pressure. Imaging studies are paramount in evaluating pineal region lesions and establishing an accurate diagnosis, with MRI representing the gold standard. Herein, we present the case of a 16-year-old boy presented with recurrent headaches. A head MRI revealed a pineal gland lesion. Histopathological examination confirmed the diagnosis, and the patient underwent a successful gross total resection (GTR) of the tumor. This case report seeks to draw attention to the elusive clinical presentation and management of this infrequently encountered tumor, as well as emphasize the importance of considering pineal gland tumors in the differential diagnosis of recurrent, chronic headaches in pediatric patients.
PubMed: 35755097
DOI: 10.1016/j.radcr.2022.05.024 -
American Journal of Ophthalmology Case... Jun 2018To describe a unilateral ocular paraneoplastic syndrome in pineal germinoma.
PURPOSE
To describe a unilateral ocular paraneoplastic syndrome in pineal germinoma.
OBSERVATIONS
A 24-year-old male presented with diplopia, excessive thirst, and frequent urination. Cranial MRI showed a mass in pineal gland. Dorsal midbrain syndrome signs were present. Examination showed optic disc edema and segmental retinal periphlebitis in right and normal fundus in left eye. Rheumatologic work-up was negative. Brain biopsy confirmed pineal germinoma. Retinal findings were attributed to paraneoplastic syndrome. Resolution of optic disc edema and retinal periphlebitis occurred following chemotherapy and focal irradiation.
CONCLUSIONS
and importance: To date there are two published case reports on bilateral optic disc edema and retinal periphlebitis occurring as a paraneoplastic syndrome in pineal germinoma. This is the first report on a patient with pineal germinoma who had unilateral paraneoplastic involvement characterized by optic disc edema and retinal periphlebitis and who showed complete resolution of ocular disease after treatment of underlying tumor.
PubMed: 29780941
DOI: 10.1016/j.ajoc.2018.03.013 -
Cureus Jul 2022Germ cell tumors are rare tumors that most commonly occur in the pediatric population. Their usual location is in the pineal gland and above the suprasellar cistern....
Germ cell tumors are rare tumors that most commonly occur in the pediatric population. Their usual location is in the pineal gland and above the suprasellar cistern. Pure germinomas, the most common type of germ cell tumor, are exquisitely sensitive to radiation, and rarely recur after radiotherapy treatment. We present a case of a pure germinoma that (1) occurred in the cerebellar hemisphere, (2) in the fifth decade of life, and (3) after being adequately treated with radiotherapy.
PubMed: 36060381
DOI: 10.7759/cureus.27535 -
Chinese Neurosurgical Journal 2018The pineal region tumors are surrounded by important structures. Neuroendoscopy has been increasingly used at home and abroad. This study is to simulate pure...
BACKGROUND
The pineal region tumors are surrounded by important structures. Neuroendoscopy has been increasingly used at home and abroad. This study is to simulate pure neuroendoscopic infratentorial supracerebellar approach for resection of pineal region tumor from the cadaveric head, and discuss the advantages and safety through this corridor.
METHODS
The anatomical structure for resection of pineal region tumor was visualized through pure neuroendoscopic infratentorial supracerebellar approach in three cadaveric heads. Three cases with pineal region tumors were retrospectively analyzed and summarized between June 2017 and December 2017. All cases were operated through pure neuroendoscopic infratentorial supracerebellar approach in the first affiliated hospital of Fujian medical university.
RESULTS
The anatomical structures of pineal region can be completely visualized by pure neuroendoscopic infratentorial supracerebellar corridor in the cadaveric head. Among the three cases, the first case was total resection, the second case was subtotal resection and the last case was partial resection. The postoperative pathology revealed cavernous hemangioma, germinoma and yolk sac tumor, respectively. The patients were followed-up for 1-6 months and had normal life.The KPS (karnofsky performance status) score was 100.
CONCLUSION
The anatomical structure of the pineal region can be completely visualized and the tumor can be safely removed through pure neuroendoscopic infratentorial supracerebellar approach.
PubMed: 32922879
DOI: 10.1186/s41016-018-0127-6 -
Modern Pathology : An Official Journal... Dec 2022Central nervous system (CNS) germ cell tumors (GCTs) represent 2-3% of all primary CNS tumors. The majority are germinomas, which are radiosensitive and have an...
Central nervous system (CNS) germ cell tumors (GCTs) represent 2-3% of all primary CNS tumors. The majority are germinomas, which are radiosensitive and have an excellent prognosis. Contrarily, CNS non-germinomatous GCTs (NGGCTs) have less favorable prognosis and require more aggressive treatment. The expression of checkpoint/immune markers in CNS GCTs, particularly NGGCTs, is unknown. We previously reported a case of a patient whose intracranial NGGCT (predominantly choriocarcinoma) responded to immune checkpoint inhibition therapy. This case led us to evaluate our archive of intracranial GCTs for expression of PD-L1 and PD-1. With IRB approval, we searched the pathology archives at our institution for CNS GCTs. Demographic, radiologic, clinical, and histologic information was extracted from the medical records. Immunohistochemistry for lymphocytic markers (CD4, CD8, CD20), PD-1, and PD-L1 was performed. PD-L1 was considered positive if greater than 1% of tumor cells were positive and PD-1 was reported as a percentage of positive inflammatory cells. Fifty cases were identified, including 28 germinomas (mean age at diagnosis: 15.5 years; 17 males, 11 females), and 22 NGGCTs (mean age at diagnosis: 12.0 years, 21 males, 1 female). Germinomas were mostly suprasellar (17/28) and NGGCTs were predominantly pineal (17/22). Twenty-two germinomas (79%) were positive for PD-L1 expression, and 13 NGGCTs (57%) were positive for PD-L1. Cases of choriocarcinoma showed the most diffuse PD-L1 expression. PD-1 expression was seen in lymphocytes among 27/28 of the germinomas and 20/23 of the NGGCTs (ranging from 1-40% of lymphocytes). As expected, larger quantities of inflammatory cells were present in cases of germinoma. We demonstrate immune activity in CNS GCTs, and our results suggest that immune checkpoint inhibitors may be efficacious in the treatment of intracranial GCTs. Among NGGCTs, cases of choriocarcinoma showed the highest expression of PD-L1 in tumor cells, suggesting that this subtype may have the greatest benefit from checkpoint blockade.
Topics: Child; Male; Humans; Female; Adolescent; Programmed Cell Death 1 Receptor; B7-H1 Antigen; Neoplasms, Germ Cell and Embryonal; Germinoma; Brain Neoplasms; Central Nervous System Neoplasms; Choriocarcinoma; Central Nervous System
PubMed: 36057740
DOI: 10.1038/s41379-022-01142-3 -
Acta Cytologica 2021Neoplasms from the ventricular system share a common location but have highly variable histogenesis. Many are slowly growing tumors that behave in a benign fashion. They... (Review)
Review
BACKGROUND
Neoplasms from the ventricular system share a common location but have highly variable histogenesis. Many are slowly growing tumors that behave in a benign fashion. They can be classified as primary and secondary tumors. The most common primary tumors are ependymomas, subependymomas, subependymal giant cell astrocytomas, central neurocytomas, choroid plexus tumors, meningiomas, germinomas, pineal parenchymal tumors, papillary tumors of the pineal region, chordoid gliomas, rosette-forming glioneuronal tumors of the fourth ventricle, and craniopharyngiomas. Pilocytic astrocytomas, medulloblastomas, and atypical teratoid/rhabdoid tumors often show secondary involvement of the ventricular system.
SUMMARY
Advances in neurosurgery have facilitated access to the ventricular system increasing the number of cases in which such tumors can be biopsied. In this context, cytology has been proven to be an extremely useful diagnostic tool during intraoperative pathologic consultations. Many ventricular tumors are infrequent, and the cytologic information available is limited. In this review, we describe the cytologic features of the uncommon ventricular tumors and report on unusual findings of the more common ones. For the cytologic evaluation of brain tumors, many neuropathologists prefer formalin fixation and hematoxylin and eosin staining. In this review, we highlight the cytologic findings as seen with Diff-Quik, a very popular staining method among cytopathologists. In fact, when pathologists are unfamiliar with cytology, it is common to request the assistance of cytopathologists during the evaluation of intraoperative procedures. Key Message: Ventricular tumors of the central nervous system comprise a group of heterogeneous tumors with very different cytologic features. The cytomorphology of these tumors, including rare entities, is often very characteristic, allowing a precise recognition during intraoperative pathologic consultations. Diff-Quik is a valuable staining method that can be used alone or as a complement to hematoxylin and eosin staining. Diff-Quik allows for clear visualization of the overall architecture, cytoplasmic details, and extracellular material.
Topics: Azure Stains; Biopsy; Cerebral Ventricle Neoplasms; Coloring Agents; Diagnosis, Differential; Humans; Intraoperative Care; Methylene Blue; Neurosurgical Procedures; Predictive Value of Tests; Staining and Labeling; Xanthenes
PubMed: 33477138
DOI: 10.1159/000512723 -
Cureus Jun 2016A 19-year-old Caucasian male presented with complaints of headaches and syncope. Suspicion of hydrocephalus prompted computed tomography (CT) and magnetic resonance...
A 19-year-old Caucasian male presented with complaints of headaches and syncope. Suspicion of hydrocephalus prompted computed tomography (CT) and magnetic resonance imaging (MRI), which revealed pineal and suprasellar prominences with diffuse, thick, nodular subependymal enhancement of the lateral and third ventricles. Based on imaging, the differential diagnosis consisted primarily of malignancy, such as lymphoma, with inflammatory and infectious etiologies not excluded. Cerebrospinal fluid (CSF) samples were non-specific, and neuroendoscopic tissue biopsy histologically confirmed the diagnosis of pure germinoma. The patient was treated with radiation, and follow-up MRIs at one, three, six, and 12 months demonstrated progressive resolution of tumor burden with marked clinical improvement. Germinomas are rare germ cell tumors that are more frequently diagnosed in Asian countries. They uncommonly seed into the lateral ventricles, and only two other cases have been described with diffuse subependymal involvement. Unlike other malignant germ cell tumors, germinomas have marker negative CSF samples that are important in the normal diagnostic workup of diffuse subependymal lesions. Histopathologic correlation is required for definitive diagnosis in the United States and can be achieved with endoscopic tissue sampling. Germinomas are highly radio- and chemotherapy sensitive and have a fair prognosis with modern therapeutic techniques. Germinoma should be considered with simultaneous midline and diffuse ventricular lesions.
PubMed: 27433421
DOI: 10.7759/cureus.643 -
World Neurosurgery Apr 2024A pineal region tumor is a rare intracranial tumor, and its specific location leads to its own characteristics. This study aimed to provide some insight for medical...
BACKGROUND
A pineal region tumor is a rare intracranial tumor, and its specific location leads to its own characteristics. This study aimed to provide some insight for medical practice in the care of pineal region tumors. We investigated the key epidemiological characteristics and survival prognosis of pineal tumors based on the epidemiological data from the Surveillance, Epidemiology, and End Results database.
METHODS
Data of pineal region tumor patients from 1975 to 2019 were extracted from the Surveillance, Epidemiology, and End Results database. The data were divided into 3 pathologic groups: germ cell tumors, pineal parenchymal tumors, and other. The patients' overall survival (OS) was analyzed using the Kaplan-Meier method. The prognostic effects of the patient characteristics on OS were explored using the Cox proportional hazard model. The analysis results are presented as tabular data, Kaplan-Meier plots, forest plots, and nomograms. A calibration curve was used to verify the nomograms. All analyses were performed for all patients overall and stratified by pathological group using SPSS and R language.
RESULTS
Based on the predefined inclusion and exclusion criteria, 628 patients were included in this study, of whom 440 (70.1%) were male and 188 (29.9%) were female. Most patients were aged 0-19 years. The pathological type was germinoma for 225 patients (35.8%). Age, surgery, behavioral code, and pathology were significant factors for OS. A calibration curve was used to verify that the nomograms had a good prediction effect.
CONCLUSIONS
An intuitive nomogram was developed and verified and can predict the prognosis of patients with pineal tumors.
Topics: Humans; Male; Female; Pinealoma; Neoplasm Staging; Retrospective Studies; SEER Program; Prognosis; Nomograms; Brain Neoplasms; Pineal Gland
PubMed: 38266997
DOI: 10.1016/j.wneu.2024.01.088 -
Neurologia Medico-chirurgica Jan 2017Immature teratoma (IMT) is normally treated by resection and adjuvant therapy. The present unusual case of recurrent germinoma occurred 21 years after total resection of... (Review)
Review
Immature teratoma (IMT) is normally treated by resection and adjuvant therapy. The present unusual case of recurrent germinoma occurred 21 years after total resection of pineal IMT. A 3-year-old boy presented with headache, disturbance of consciousness, and Parinaud's syndrome. Magnetic resonance (MR) imaging revealed a pineal mass lesion, and total resection of the tumor was achieved. The histological diagnosis was mature teratoma. He did not receive further treatment, and did well without recurrence for 20 years. However, he suffered headache 21 years after resection, and MR imaging revealed a homogeneously enhanced pineal mass with low minimum apparent diffusion coefficient value and proton MR spectroscopy showed a huge lipid peak. The levels of tumor markers were not elevated. Cerebrospinal fluid (CSF) cytology found atypical cells with large nuclei and irregularly shaped nucleoli. To elucidate the relationship between the primary and recurrent tumors, we reviewed the histological specimens and CSF cytology at the initial treatment and found a subset of incompletely differentiated components resembling fetal tissues in the histological specimen and atypical large cells in the CSF. Based on these radiological and histological findings, we presume that the recurrent disease was disseminated germinoma after the resection of disseminated IMT. He received chemotherapy and craniospinal radiation therapy, and the enhanced lesion and atypical cells in the CSF disappeared. This case demonstrates that disseminated IMT can be controlled for the long term without adjuvant therapy, but may recur as germinoma. Tumor dormancy may account for this unusual course.
Topics: Brain Neoplasms; Child, Preschool; Humans; Male; Neoplasm Recurrence, Local; Teratoma; Time Factors
PubMed: 27928096
DOI: 10.2176/nmc.cr.2016-0241 -
Cureus Jan 2024Intracranial germ cell tumors (GCTs) are rare malignant tumors with a peak incidence around puberty. The pineal region is the most commonly involved area of all...
Intracranial germ cell tumors (GCTs) are rare malignant tumors with a peak incidence around puberty. The pineal region is the most commonly involved area of all intracranial GCTs. Due to the heterogeneous tumor origin, subtypes, and presentation, diagnosis and management are challenging. Complicated pineal germinomas are rarely reported in the literature. Here, we report a rare case of pineal germinoma with hydrocephalus and discuss the potential treatment approach. A 20-year-old boy presented to the hospital with vomiting and a decreased level of consciousness. The brain magnetic resonance imaging (MRI) revealed a pineal tumor. A ventriculoperitoneal shunt was placed to relieve the increased intracranial pressure. The patient underwent a suboccipital craniotomy with excisional biopsy of the pineal region tumor due to its critical location, as imaging studies alone may not be sufficient to establish a definitive diagnosis. Although there has been a rise in reported cases of germinoma tumors, there is currently no standardized therapeutic approach for treating them. Therefore, more randomized controlled cohort studies are necessary to evaluate potential treatments and develop a therapeutic approach.
PubMed: 38435909
DOI: 10.7759/cureus.53325