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Cureus Mar 2024Pineal parenchymal tumors (PPTs) are rare, accounting for less than 0.3% of all primary central nervous system (CNS) tumors. Pineal parenchymal tumors of intermediate...
Pineal parenchymal tumors (PPTs) are rare, accounting for less than 0.3% of all primary central nervous system (CNS) tumors. Pineal parenchymal tumors of intermediate differentiation (PPTID) (WHO grade 2 or 3) show an intermediate prognosis between pineocytoma and pineoblastoma. The clinical course is unknown, and the optimal treatment for PPTID, especially for recurrence, has not been determined. We report a case of PPTID with spinal dissemination over 10 years after treatment and survival for four years. A 56-year-old woman presented with headaches and diplopia. Computerized tomography (CT) and magnetic resonance imaging (MRI) revealed a pineal mass, but leptomeningeal dissemination was not identified on whole-spine MRI. Microsurgical gross total tumor resection (GTR) was performed, and the pathological diagnosis was PPTID (grade 3). In addition, a later study found it to harbor a mutation. She underwent whole-brain radiation therapy with a focal boost. The patient was unable to continue chemotherapy for severe myelosuppression after the first course of treatment. Eleven years after the surgery, she was unable to walk, and a whole-spine MRI revealed multiple masses at C3-4, T4, and cauda equina. Fluorodeoxyglucose-positron emission tomography (FDG-PET) revealed accumulations of the same lesions. No recurrence was observed in the brain. A biopsy of the caudal portion was performed, and the histopathological findings were the same as those of the initial surgery. Spinal dissemination was refractory to chemotherapy but responded to whole spine radiotherapy with focal boost, and she remained tumor-free for four years. We considered good local control with a combination of GTR and subsequent radiation therapy to contribute to long-term survival. The timing of spinal radiation administration is controversial because of the tendency for late cerebrospinal dissemination. The importance of long-term follow-up of the spine and head is emphasized. In PPTID cases with good local control, withholding spinal radiation until spinal dissemination occurs may become a long-term treatment plan.
PubMed: 38681294
DOI: 10.7759/cureus.57147 -
Pediatric Neurosurgery Apr 2024Pineal region tumors have historically been challenging to treat. Advances in surgical techniques have led to significant changes in care and outcomes for these...
INTRODUCTION
Pineal region tumors have historically been challenging to treat. Advances in surgical techniques have led to significant changes in care and outcomes for these patients, and this is well demonstrated by our single institution's experience over a 17-year-period in which the evolution of diagnosis, treatment, and outcomes of pineal tumors in pediatric patients will be outlined.
METHODS
We retrospectively collected data on all pediatric patients with pineal region lesions treated with surgery at Children's National Hospital (CNH) from 2005 to 2021. Variables analyzed included presenting symptoms, presence of hydrocephalus, diagnostic and surgical approach, pathology, and adverse events, among others. IRB approval was obtained (IRB: STUDY00000009), and consent was waived due to minimal risk to patients included.
RESULTS
A total of 43 pediatric patients with pineal region tumors were treated during a 17-year period. Most tumors in our series were germinomas (n = 13, 29.5%) followed by pineoblastomas (n = 10, 22.7%). Twenty seven of the 43 patients (62.8%) in our series received a biopsy to establish diagnosis, and 44.4% went on to have surgery for resection. The most common open approach was posterior interhemispheric (PIH, transcallosal) - used for 59.3% of the patients. Gross total resection was achieved in 50%; recurrence occurred in 20.9% and mortality in 11% over a median follow-up of 47 months. Endoscopic third ventriculostomy (ETV) was employed to treat hydrocephalus in 26 of the 38 patients (68.4%) and was significantly more likely to be performed from 2011 to 2021. Most (73%) of the patients who received an ETV also underwent a concurrent endoscopic biopsy. No difference was found in recurrence rate or mortality in patients who underwent resection compared to those who did not, but complications were more frequent with resection. There was disagreement between frozen and final pathology in 18.4% of biopsies.
CONCLUSION
This series describes the evolution of surgical approaches and outcomes over a 17-year-period at a single institution. Complication rates were higher with open resection, reinforcing the safety of pursuing endoscopic biopsy as an initial approach. The most significant changes occurred in the preferential use of ETVs over ventriculoperitoneal shunts. Though there has been a significant evolution in our understanding of and treatment for these tumors, in our series, the outcomes for these patients have not significantly changed over that time.
PubMed: 38679003
DOI: 10.1159/000538745 -
Current Oncology (Toronto, Ont.) Mar 2024Molecular analysis of the growing teratoma syndrome has not been extensively studied. Here, we report a 14-year-old boy with a growing mass during treatment for a mixed...
Molecular analysis of the growing teratoma syndrome has not been extensively studied. Here, we report a 14-year-old boy with a growing mass during treatment for a mixed germ cell tumor of the pineal region. Tumor markers were negative; thus, growing teratoma syndrome was suspected. A radical resection via the occipital transtentorial approach was performed, and histopathological examination revealed a teratoma with malignant features. Methylation classifier analysis confirmed the diagnosis of teratoma, and loss and 12p gain were identified by copy number variation analysis, potentially elucidating the cause of growth and malignant transformation of the teratoma. The patient remains in remission after intense chemoradiation treatment as a high-risk germ cell tumor.
Topics: Humans; Male; Teratoma; Adolescent; Brain Neoplasms; Combined Modality Therapy
PubMed: 38668041
DOI: 10.3390/curroncol31040138 -
Frontiers in Oncology 2024Pure germinomas account for 40% of pineal tumors and are characterized by the lack of appreciable tumor markers, thus requiring a tumor biopsy for diagnosis. MicroRNAs...
BACKGROUND
Pure germinomas account for 40% of pineal tumors and are characterized by the lack of appreciable tumor markers, thus requiring a tumor biopsy for diagnosis. MicroRNAs (miRNA) have emerged as potential non-invasive biomarkers for germ cell tumors and may facilitate the non-invasive diagnosis of pure pineal germinomas.
MATERIAL AND METHODS
A retrospective chart review was performed on all patients treated at the Children's Cancer Hospital Egypt diagnosed with a pineal region tumor between June 2013 and March 2021 for whom a research blood sample was available. Plasma samples were profiled for miRNA expression, and DESeq2 was used to compare between pure germinoma and other tumor types. Differentially expressed miRNAs were identified. The area under the curve of the receive;r operating characteristic curve was constructed to evaluate diagnostic performance.
RESULTS
Samples from 39 pediatric patients were available consisting of 12 pure germinomas and 27 pineal region tumors of other pathologies, including pineal origin tumors [ = 17; pineoblastoma ( = 13) and pineal parenchymal tumors of intermediate differentiation ( = 4)] and others [ = 10; low-grade glioma ( = 6) and atypical teratoid rhabdoid tumor ( = 4)]. Using an adjusted -value <0.05, three miRNAs showed differential expression (miR-143-3p, miR-320c, miR-320d; adjusted = 0.0058, = 0.0478, and = 0.0366, respectively) and good discriminatory power between the two groups (AUC 90.7%, < 0.001) with a sensitivity of 25% and a specificity of 100%.
CONCLUSION
Our results suggest that a three-plasma miRNA signature has the potential to non-invasively identify pineal body pure germinomas which may allow selected patients to avoid the potential surgical complications.
PubMed: 38665953
DOI: 10.3389/fonc.2024.1219796 -
Advances and Technical Standards in... 2024Management of lateral and third ventricular tumors has been a challenge for neurosurgeons. Advances in imaging and pathology have helped in a better understanding of the... (Review)
Review
Management of lateral and third ventricular tumors has been a challenge for neurosurgeons. Advances in imaging and pathology have helped in a better understanding of the treatment options. Technical refinement of microsurgical technique and addition of endoscopy has enabled more radical excision of tumors, when indicated, and added more safety.A proper understanding of the pathology at various ages and treatment options is continuously evolving. Many pediatric tumors are amenable to conservative surgical methods with effective complementary treatments. However, radical surgery is required in many adults as the main treatment and for many benign tumors. Various intraventricular lesions encountered and their surgical management is reviewed here for their efficacy, safety, and outcome, encompassing changes in our practice over the last 20 years.
Topics: Adult; Child; Humans; Cerebral Ventricle Neoplasms; Neurosurgeons; Third Ventricle
PubMed: 38592528
DOI: 10.1007/978-3-031-53578-9_3 -
Journal of Clinical Medicine Feb 2024Pineal parenchymal cell tumors constitute a rare group of primary central nervous system neoplasms (less than 1%). Their classification, especially the intermediate... (Review)
Review
BACKGROUND
Pineal parenchymal cell tumors constitute a rare group of primary central nervous system neoplasms (less than 1%). Their classification, especially the intermediate subtype (PPTIDs), remains challenging.
METHODS
A literature review was conducted, navigating through anatomo-pathological, radiotherapy, and neurosurgical dimensions, aiming for a holistic understanding of these tumors.
RESULTS
PPTIDs, occupying an intermediate spectrum of malignancy, reveal diverse histological patterns, mitotic activity, and distinct methylation profiles. Surgical treatment is the gold standard, but when limited to partial removal, radiotherapy becomes crucial. While surgical approaches are standardized, due to the low prevalence of the pathology and absence of randomized prospective studies, there are no shared guidelines about radiation treatment modalities.
CONCLUSION
Surgical removal remains pivotal, demanding a personalized approach based on the tumor extension. This review underscores the considerable variability in treatment approaches and reported survival rates within the existing literature, emphasizing the need for ongoing research to better define optimal therapeutic strategies and prognostic factors for PPTIDs, aiming for further and more detailed stratification among them.
PubMed: 38592098
DOI: 10.3390/jcm13051266 -
Life Sciences Jun 2024Sepsis is a life-threatening condition manifested by organ dysfunction caused by a dysregulated host response to infection. Lung, brain, liver, kidney, and heart are... (Review)
Review
Sepsis is a life-threatening condition manifested by organ dysfunction caused by a dysregulated host response to infection. Lung, brain, liver, kidney, and heart are among the affected organs. Sepsis-induced cardiomyopathy is a common cause of death among septic patients. Sepsis-induced cardiomyopathy is characterized by an acute and reversible significant decline in biventricular both systolic and diastolic function. This is accompanied by left ventricular dilatation. The pathogenesis underlying sepsis-induced cardiomyopathy is multifactorial. Hence, targeting an individual pathway may not be effective in halting the extensive dysregulated immune response. Despite major advances in sepsis management strategies, no effective pharmacological strategies have been shown to treat or even reverse sepsis-induced cardiomyopathy. Melatonin, namely, N-acetyl-5-methoxytryptamine, is synthesized in the pineal gland of mammals and can also be produced in many cells and tissues. Melatonin has cardioprotective, neuroprotective, and anti-tumor activity. Several literature reviews have explored the role of melatonin in preventing sepsis-induced organ failure. Melatonin was found to act on different pathways that are involved in the pathogenesis of sepsis-induced cardiomyopathy. Through its antimicrobial, anti-inflammatory, and antioxidant activity, it offers a potential role in sepsis-induced cardiomyopathy. Its antioxidant activity is through free radical scavenging against reactive oxygen and nitrogen species and modulating the expression and activity of antioxidant enzymes. Melatonin anti-inflammatory activities control the overactive immune system and mitigate cytokine storm. Also, it mitigates mitochondrial dysfunction, a major mechanism involved in sepsis-induced cardiomyopathy, and thus controls apoptosis. Therefore, this review discusses melatonin as a promising drug for the management of sepsis-induced cardiomyopathy.
Topics: Melatonin; Sepsis; Humans; Cardiomyopathies; Animals; Antioxidants; Cardiotonic Agents
PubMed: 38580195
DOI: 10.1016/j.lfs.2024.122611 -
Cureus Mar 2024Falcotentorial meningiomas are rare tumors, representing only 2-3% of all intracranial meningiomas. These tumors can grow rapidly, leading to severe neurological...
Falcotentorial meningiomas are rare tumors, representing only 2-3% of all intracranial meningiomas. These tumors can grow rapidly, leading to severe neurological complications. They grow at the junction of the tentorium cerebelli and the falx cerebri, in close proximity to the great vein of Galen. The surgical approach depends on several factors, such as the tumor's size, the patency of the straight sinus, and its location, either above or below the tentorium. Complete removal of the tumor in this area is difficult due to its deep location near major neurovascular structures. Various surgical approaches can be employed to remove these tumors, and the decision on which approach to use should be based on its advantages and disadvantages.
PubMed: 38576702
DOI: 10.7759/cureus.55581 -
Case Reports in Oncology 2024Intracranial germinomas mainly arise in the pineal gland or neurohypophyseal region. The basal ganglia have been reported as the site of occurrence for ectopic...
INTRODUCTION
Intracranial germinomas mainly arise in the pineal gland or neurohypophyseal region. The basal ganglia have been reported as the site of occurrence for ectopic germinomas, whereas other sites have been rarely described. We experienced a case of multifocal ectopic germinoma that arose in the septum pellucidum and the dorsal brain stem, not including the pineal gland, neurohypophysis, and basal ganglia of ectopic germinoma in a pregnant woman.
CASE PRESENTATION
The patient initially presented to our institution with complaints of diplopia in the past 14 weeks of gestation, and imaging later revealed two intracranial neoplastic lesions, with one lesion involving the septum pellucidum and the other involving the dorsal brainstem. Both tumors were partially excised via a transcortical approach. Based on the results of pathology and immunohistochemistry, the patient was diagnosed with germinoma, and the intraoperative spinal fluid cytology was class V in Papanicolaou classification. The patient received three courses of ifosfamide, carboplatin, and etoposide together with whole-brain irradiation, which resulted in complete elimination of the tumors. No evidence of recurrence was identified after 18 months.
CONCLUSION
The results illustrated the need to consider germinoma in the differential diagnosis if the lesions involve midline structures such as the septum pellucidum or dorsal brainstem. Given the rarity of germinoma arising outside the pineal gland and neurohypophyseal region, these findings provide key insights into the diagnosis and treatment of this disease.
PubMed: 38549623
DOI: 10.1159/000538088 -
Zhurnal Voprosy Neirokhirurgii Imeni N.... 2024Currently, endoscopic third ventriculostomy and simultaneous biopsy of deep midline brain tumors are a generally accepted option in neurooncology. Nevertheless,...
BACKGROUND
Currently, endoscopic third ventriculostomy and simultaneous biopsy of deep midline brain tumors are a generally accepted option in neurooncology. Nevertheless, effectiveness of this surgery and diagnostic accuracy of biopsy are not without drawbacks. An alternative to endoscopic surgery may be simultaneous microsurgical third ventriculostomy and biopsy of deep midline tumors.
OBJECTIVE
To evaluate effectiveness and safety of burr hole microsurgical third ventriculostomy in the treatment of deep midline brain tumors.
MATERIAL AND METHODS
We used transcortical (25 cases) and transcallosal (8 cases) approaches for microsurgical third ventriculostomy.
RESULTS
Initially scheduled biopsy was performed in 19 cases, partial resection in 6 cases, subtotal resection in 4 cases and total resection in 4 cases. All patients underwent microsurgical third ventriculostomy. In 12 cases, stenting of stoma was performed in addition to ventriculostomy. Biopsy was informative in all cases. Postoperative follow-up period ranged from 3 to 44 months (mean 29 months). There was no postoperative hydrocephalus and need for shunting procedure.
CONCLUSION
Burr hole microsurgery may be an alternative to endoscopic surgery for the treatment of pineal, periaqueductal and third ventricular tumors.
Topics: Humans; Ventriculostomy; Third Ventricle; Brain Neoplasms; Endoscopy; Pineal Gland; Hydrocephalus; Neuroendoscopy
PubMed: 38549405
DOI: 10.17116/neiro2024880215