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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 -
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 -
Surgical Neurology International 2023Intracranial germinomas are rare tumors, accounting for 0.5-2% of primary intracranial neoplasms. While they typically occur in the pineal gland, suprasellar region,...
BACKGROUND
Intracranial germinomas are rare tumors, accounting for 0.5-2% of primary intracranial neoplasms. While they typically occur in the pineal gland, suprasellar region, basal ganglia, and thalamus, germinomas arising in the medulla oblongata are exceptionally rare. Diagnosis of medulla oblongata germinoma is challenging, potentially leading to misdiagnosis and poor prognosis.
CASE DESCRIPTION
We present a case of a 29-year-old man complaining of left leg numbness. Radiological findings revealed a contrast-enhanced lesion in the medulla oblongata. The patient underwent tumor biopsy, and intraoperative pathological diagnosis (IOD) suspected the diagnosis of medulla oblongata germinoma. He underwent chemoradiotherapy after confirming the diagnosis of germinoma. Intracranial germinoma arising in the medulla oblongata differs from germinomas in other locations due to its higher incidence in individuals in their 20s and a slight female predominance.
CONCLUSION
When encountering lesions in the medulla oblongata, germinoma should be considered as one of the differential diagnoses, and surgical strategies including IOD should be planned accordingly.
PubMed: 37941640
DOI: 10.25259/SNI_682_2023 -
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 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 -
NMC Case Report Journal 2023Primary germ cell tumors of the central nervous system (CNS) typically occur in the neurohypophysis, hypothalamus, or pineal gland and rarely in the spinal cord. We...
Primary germ cell tumors of the central nervous system (CNS) typically occur in the neurohypophysis, hypothalamus, or pineal gland and rarely in the spinal cord. We report a case of a spinal intramedullary tumor, which was first detected on magnetic resonance imaging (MRI) 41 months after the initial symptoms, with a verified pathological diagnosis of germinoma. The initial symptom was an abnormal sensation in the left plantar region that gradually worsened, resulting in severe sensory disturbance, difficulty in standing, and even bladder rectal disturbance. Repeated MRI after the onset failed to provide an imaging diagnosis. The MRI was performed 41 months after the onset and revealed a previously undiagnosed, contrast-enhancing spinal intramedullary neoplastic lesion at the Th11-12 level. Gross total resection of the tumor was successfully performed, and the pathology confirmed the diagnosis of pure germinoma. Postoperative chemotherapy, followed by local radiation, was successfully administered. Among primary germinomas of the CNS, occult germinoma that lacks imaging findings suggestive of tumors in the early stages of onset and becomes apparent over time is often reported as a primary neurohypophyseal germinoma, particularly in adolescents presenting with diabetes insipidus. In the present case, the lesion appeared to correspond to a primary occult germinoma of the intramedullary spinal cord.
PubMed: 36937498
DOI: 10.2176/jns-nmc.2022-0346 -
Medicine Aug 2019Tumors of the pineal region are rare, and metastatic carcinoma occurring in the pineal region is extremely rare. No previous reports have described pineal region...
INTRODUCTION
Tumors of the pineal region are rare, and metastatic carcinoma occurring in the pineal region is extremely rare. No previous reports have described pineal region metastasis with intraventricular seeding.
PATIENT CONCERNS
We report a case of a 51-year-old woman presented with a 1-week history of severe headache, nausea, and vomiting. Imaging examination revealed 2 lesions in the pineal region and the right lateral ventricle.
DIAGNOSIS
Pinealocytoma or germinoma was considered as the preoperative diagnosis. The postoperative pathological diagnosis was small cell neuroendocrine carcinoma. After bronchoscopic biopsy, small cell lung cancer was confirmed.
INTERVENTIONS
A right frontal craniotomy and a translateral ventricle approach were performed to remove 2 lesions completely. And regular radiotherapy and chemotherapy were initiated after surgery.
OUTCOMES
The patient was discharged from the hospital 2 weeks after operation and went to another cancer hospital for bronchoscopic biopsy, radiotherapy, and chemotherapy. Finally, the patient died 2 years after surgical treatment.
CONCLUSION
Metastatic tumors of the pineal region are very rare. For patients with pineal lesions, a diagnosis of a metastatic tumor should be considered. Retrograde cerebrospinal fluid circulation might be the reason for a secondary metastasis.
Topics: Diagnosis, Differential; Female; Humans; Lung Neoplasms; Middle Aged; Neoplasm Metastasis; Pinealoma; Small Cell Lung Carcinoma
PubMed: 31441839
DOI: 10.1097/MD.0000000000016652 -
Journal of Neurosurgery. Case Lessons Apr 2021Germinoma is the most common type of germ cell tumor that develops intracranially. Germinomas usually grow in the midline structures, such as the pineal and suprasellar...
BACKGROUND
Germinoma is the most common type of germ cell tumor that develops intracranially. Germinomas usually grow in the midline structures, such as the pineal and suprasellar regions, and are rarely found in other locations. To the best of the authors' knowledge, no previous research has reported on growth of this tumor in the cranial vault.
OBSERVATIONS
The authors reported an unusual case of primary cranial vault germinoma in a young adult. Macroscopically, the tumor had a solid-soft consistency and grayish color with brownish spots on the surface. The histological examination revealed anaplastic cells with round, hyperchromatic, pleomorphic nuclei; prominent nucleoli; and abundant, clear cytoplasm, arranged in lobules and sheets that were infiltrated by lymphocytes and separated by fibrous connective tissue. These findings were consistent with the histopathological characteristics of germinoma.
LESSONS
Primary cranial vault germinoma is a unique tumor because no previous research has reported any growth in that location. It should be considered one of the differential diagnoses of lesions located over the cranial vault. Histopathological examination is still the primary modality for diagnosing these tumors and excluding other differential diagnoses.
PubMed: 35855389
DOI: 10.3171/CASE2082 -
Cureus Apr 2024This case report delineates the radiological evaluation and diagnostic intricacies of two unique cases of pineal region meningioma, underscoring the pivotal role of...
This case report delineates the radiological evaluation and diagnostic intricacies of two unique cases of pineal region meningioma, underscoring the pivotal role of advanced imaging techniques in the accurate diagnosis and management of such rare tumors. Pineal region tumors represent a heterogeneous group of neoplasms, with meningiomas in this location being particularly uncommon, thereby posing significant challenges in diagnosis and therapeutic decision-making. The first case involves a 40-year-old female presenting with progressive headaches and visual disturbances with symptoms of increased intracranial pressure, whereas the second case describes a 30-year-old male presenting with headache, dizziness, difficulty with balance, and cognitive decline. Both patients underwent a comprehensive diagnostic workup, including magnetic resonance imaging (MRI), which revealed tumors in the pineal region exhibiting characteristics suggestive of meningioma. The MRI findings in both cases included well-defined mass lesions showing iso- to hypointense signal on T1-weighted images with robust contrast enhancement. Additionally, the radiological assessment was instrumental in differentiating these meningiomas from other pineal region tumors, such as germinomas or pineocytomas, based on their distinctive imaging features and the absence of dissemination. Surgical resection followed by histopathological examination confirmed the diagnosis of meningioma in both cases. This report highlights the critical role of radiological imaging in the early detection and differentiation of pineal region tumors, emphasizing the need for a multidisciplinary approach to achieve optimal patient outcomes.
PubMed: 38721197
DOI: 10.7759/cureus.57796