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Cancer Innovation Dec 2023The aim of this study was to review clinical features of adolescent malignant germ cell tumors (MGCTs) in Beijing and analyze the peculiar characteristics of this age...
BACKGROUND
The aim of this study was to review clinical features of adolescent malignant germ cell tumors (MGCTs) in Beijing and analyze the peculiar characteristics of this age group.
METHODS
Clinical characteristics, pathological presentations, and survival outcomes of 34 patients were analyzed retrospectively.
RESULTS
Of 34 patients, 12 girls and 22 boys, 18 (52.9%) had an extra-cranial tumor, including one testicular tumor, five ovarian tumors, one sacrococcygeal tumor, and 11 mediastinal tumors. Histologically, we found immature teratomas ( = 6), yolk sac tumors ( = 5), mixed malignant tumors ( = 5), an embryonic carcinoma ( = 1), and seminoma ( = 1). Three-year event-free survival (EFS) and overall survival (OS) were 48.8% and 62.9%, respectively. Another 16 (47.1%) patients had an intracranial tumor, including nine in the pineal region, five in the suprasellar region, one in basal ganglia, and one in cerebellopontine. All patients had localized disease and an excellent outcome with 3-year EFS and OS of 93.7% and 100%, respectively.
CONCLUSIONS
Adolescent MGCTs are rare with a strong dependence on gender, and the mediastina and pineal region are the most common tumor locations. The prognosis is promising compared with that of other adolescent tumors and MGCTs in other age groups. MGCTs in mediastina have a tendency to companion with other hematological malignancies, and the prognosis is extremely poor in these patients.
PubMed: 38125762
DOI: 10.1002/cai2.87 -
Magyar Onkologia Dec 2023Considerable changes were introduced into the 5th World Health Organization (WHO) classification of central nervous system (CNS) tumors, published in 2021, including new...
Considerable changes were introduced into the 5th World Health Organization (WHO) classification of central nervous system (CNS) tumors, published in 2021, including new entities, a clearer classification of previous categories, correlating better with clinical behavior and changes in nomenclature. The number of definitions based on molecular features in addition to histopathology continued to increase. Here, we highlight the basic principles of the 5th CNS WHO classification and discuss glial, glioneuronal, neuronal, choroid plexus, embryonal and pineal tumors, as well as meningiomas in more details. We pay special attention to new entities as well as altered criteria and designations. Our primary goal is to present the "classical" pathological aspects, but the inseparable molecular pathological features are also briefly discussed, to the absolutely necessary extent for comprehension. We aim to provide a guideline to understand the modern classification of CNS tumors for practitioners of neuro-oncology and neuropathology.
Topics: Humans; Central Nervous System Neoplasms; World Health Organization; Pathology, Molecular; Brain Neoplasms
PubMed: 38109508
DOI: No ID Found -
Complementary Medicine Research 2024Migraine is one of the leading causes of morbidity and disability in the world. Its prevalence ranges from 5 to 40% in the pediatric population, with about 50% of these...
BACKGROUND
Migraine is one of the leading causes of morbidity and disability in the world. Its prevalence ranges from 5 to 40% in the pediatric population, with about 50% of these headaches continuing to adulthood. Incidental diagnosis of pineal cysts (PCs) is on the rise with the evolution of imaging techniques; the causal relationship of PC and individuals with headache however remains controversial, and complete resolution is uncommon. Conventional medicine stresses on the importance of tailored therapy in the management of pediatric migraine.
CASE REPORT
An 11-year-old female, diagnosed with migraine with an aura and PC, was benefitted by individualized classical homeopathic therapy using the homeopathic preparation Phosphorous and Natrum muriaticum. The symptoms of migraine improved, and the incidentally diagnosed PC completely resolved during the course of treatment.
CONCLUSION
Individualized classical homeopathy may have a role in effectively treating migraines and PCs in the pediatric population. There is a need for further scientific investigation with well-designed studies to prove the effectiveness of this therapeutic method, taking care to maintain the individualization principle of classical homeopathy.
UNLABELLED
Hintergrund Migräne ist eine der führenden Ursachen für Krankheitslast und Behinderung weltweit. Ihre Prävalenz bei Kindern und Jugendlichen liegt im Bereich von 5–40%; hiervon sind etwa 50% bis ins Erwachsenenalter von den Kopfschmerzen betroffen. Zufallsdiagnosen von Pinealiszysten (PC) kommen im Zuge des Fortschritts bei den bildgebenden Verfahren immer häufiger vor, jedoch ist ein kausaler Zusammenhang der PC mit Kopfschmerzen umstritten, und ein vollständige Rückbildung kommt nicht häufig vor. Die Schulmedizin betont, wie wichtig eine maßgeschneiderte Therapie bei der Behandlung der Migräne im Kindesalter ist.Fallbericht Ein 11-jähriges Mädchen, bei dem eine Migräne mit Aura und eine Pinealiszyste diagnostiziert wurde, profitierte von einer individualisierten klassisch-homöopathischen Therapie mit den homöopathischen Präparaten Phosphorus und Natrum muriaticum. Die Migränesymptome besserten sich, und die zufällig diagnostizierte Pinealiszyste bildete sich im Laufe der Behandlung vollständig zurück.Schlussfolgerung Die individualisierte klassische Homöopathie kann bei der wirksamen Behandlung von Migräne und Pinealiszysten bei Kindern und Jugendlichen eine Rolle spielen. Es bedarf weiterer wissenschaftlicher Untersuchungen mit gutem Studiendesign, um die Wirksamkeit dieser Behandlungsmethode nachzuweisen, wobei darauf zu achten ist, das Individualisierungsprinzip der klassischen Homöopathie aufrechtzuerhalten.Topics: Female; Humans; Child; Adolescent; Homeopathy; Headache; Migraine Disorders; Central Nervous System Cysts; Epilepsy
PubMed: 38096803
DOI: 10.1159/000535615 -
Hormone and Metabolic Research =... Jun 2024Melatonin (5-methoxy-acetyl tryptamine) is a sleep-inducing hormone, and the pineal gland produces it in response to the circadian clock of darkness. In the body, MT1... (Review)
Review
Melatonin (5-methoxy-acetyl tryptamine) is a sleep-inducing hormone, and the pineal gland produces it in response to the circadian clock of darkness. In the body, MT1 and MT2 receptors are mostly found, having an orthosteric pocket and ligand binding determinants. Melatonin acts by binding on melatonin receptors, intracellular proteins, and orphan nuclear receptors. It inhibits adenyl cyclase and activates phospholipase C, resulting in gene expression and an intracellular alteration environment. Melatonin signaling pathways are also associated with other intracellular signaling pathways, i. e., cAMP/PKA and MAPK/ERK pathways. Relative expression of different proteins depends on the coupling profile of G protein, accounting pharmacology of the melatonin receptor bias system, and mediates action in a Gi-dependent manner. It shows antioxidant, antitumor, antiproliferative, and neuroprotective activity. Different types of melatonin agonists have been synthesized for the treatment of sleeping disorders. Researchers have developed therapeutics that target melatonin signaling, which could benefit a wide range of medical conditions. This review focuses on melatonin receptors, pharmacology, and signaling cascades; it aims to provide basic mechanical aspects of the receptor's pharmacology, melatonin's functions in cancer and neurodegenerative diseases, and any treatments and drugs designed for these diseases. This will allow a basic comparison between the receptors in question, highlighting any parallels and differences that may exist and providing fundamental knowledge about these receptors to future researchers.
Topics: Humans; Melatonin; Signal Transduction; Animals; Receptors, Melatonin; Neoplasms; Neurodegenerative Diseases
PubMed: 38081221
DOI: 10.1055/a-2226-3971 -
Diagnostics (Basel, Switzerland) Dec 2023The natural course of pineal germ cell tumors (GCTs), particularly their post-operative progression, is not well understood. We report a rare case of pineal region GCT...
The natural course of pineal germ cell tumors (GCTs), particularly their post-operative progression, is not well understood. We report a rare case of pineal region GCT showing rapid enlargement within 2 weeks following surgical resection. A young adult male presented with progressive headache and diplopia for several weeks. Although elevation of β-human chorionic gonadotropin (β-HCG) and α-fetoprotein (AFP) levels suggested that a large pineal mass lesion observed on magnetic resonance imaging (MRI) might be a β-HCG/AFP-producing tumor, whether the mass was truly a GCT remained unclear. We performed an endoscopy-assisted suboccipital infratentorial approach with removal of the tumor that was diagnosed as germinoma via histopathological investigation. During the week preceding chemotherapy, the patient's consciousness rapidly worsened. MRI showed that the residual pineal germinoma had enlarged and even compressed the tectum and thalamus. Emergency chemotherapy and radiotherapy were prescribed, and the patient received invasive ventilation for respiratory failure. Unexpectedly, the patient recovered within a short period. Importantly, total regression of the pineal germinoma, accompanied by β-HCG and AFP levels returning to normal range, was observed 4 months after chemotherapy. These phenomena suggest that the rapid enlargement of the pineal germinoma, which might be induced by aggressive surgical cytoreduction, responds well to chemoradiotherapy.
PubMed: 38066820
DOI: 10.3390/diagnostics13233579 -
Journal of Neurosciences in Rural... 2023Pineal cysts are usually benign, fluid-filled sacs and most pineal cysts are asymptomatic. Sudden death specifically related to pineal cysts is extremely uncommon. A...
Pineal cysts are usually benign, fluid-filled sacs and most pineal cysts are asymptomatic. Sudden death specifically related to pineal cysts is extremely uncommon. A literature review of the pertinent databases, including PubMed, Google Scholar, Scopus, and Web of Science, was carried out to review the existing literature describing sudden death in patients with pineal cysts. In the evaluation of 49 articles, it was found that four reports discussed the unexpected death of patients who had pineal cysts. A total of four cases of sudden death and a pineal cyst were reported. There were 75% females and a mean age of 29 (range: 20-45). Cyst size on average was 1.3 cm (1.2-1.5). In each case, the cause of death and the involvement of important brain structures were confirmed by autopsy results. A pathological analysis of the pineal region and the surrounding brain tissue revealed a variety of lesions. Vascular malformation was found in one case, adding another layer of complexity to the study of sudden death syndrome. In this research, the authors highlight the fact that patients with pineal cysts can experience serious, even fatal, complications. Increased vigilance and early detection through neuroimaging and neurological assessments are required due to the wide variety of clinical manifestations and underlying mechanisms. To explain the mechanism and enhance the management and prevention of sudden deaths associated with pineal cysts, additional research with larger sample sizes is required.
PubMed: 38059255
DOI: 10.25259/JNRP_421_2023 -
Journal of Neurosurgery. Case Lessons Nov 2023Glioblastoma (GBM) is the most common primary brain tumor with poor patient prognosis. Spinal leptomeningeal metastasis has been rarely reported, with long intervals...
BACKGROUND
Glioblastoma (GBM) is the most common primary brain tumor with poor patient prognosis. Spinal leptomeningeal metastasis has been rarely reported, with long intervals between the initial discovery of the primary tumor in the brain and eventual spine metastasis.
OBSERVATIONS
Here, the authors present the case of a 51-year-old male presenting with 7 days of severe headache, nausea, and vomiting. Magnetic resonance imaging of the brain and spine demonstrated a contrast-enhancing mass in the pineal region, along with spinal metastases to T8, T12, and L5. Initial frozen-section diagnosis led to the treatment strategy for medulloblastoma, but further molecular analysis revealed characteristics of isocitrate dehydrogenase-wild type, grade 4 GBM.
LESSONS
Glioblastoma has the potential to show metastatic spread at the time of diagnosis. Spinal imaging should be considered in patients with clinical suspicion of leptomeningeal spread. Furthermore, molecular analysis should be confirmed following pathological diagnosis to fine-tune treatment strategies.
PubMed: 37956418
DOI: 10.3171/CASE23536 -
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 -
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 -
Surgical Neurology International 2023Leiomyosarcomas (LMSs) is a type of sarcoma that arises from smooth muscle and generally presents in the abdomen. Although intracranial LMS has been identified before,...
Giant primary intracranial multi-fossa leiomyosarcoma involving the frontal sinus, ethmoid air cells, anterior fossa, middle fossa, and intraventricular space: A case report and literature review.
BACKGROUND
Leiomyosarcomas (LMSs) is a type of sarcoma that arises from smooth muscle and generally presents in the abdomen. Although intracranial LMS has been identified before, most reported presentations have been in immunocompromised patients. Here, we present an intracranial LMS in an immunocompetent patient.
CASE DESCRIPTION
A 22-year-old male with a history of an atypical pineal parenchymal tumor of intermediate differentiation resected by suboccipital craniotomy at the age of 12 followed by adjuvant radiation therapy, presented with 3 weeks of decreased appetite, weight loss, and lethargy. He subsequently underwent transbasal approach skull base tumor resection. Histologic examination of the mass along with the patient's history of radiation was supportive of a low-grade, radiation-induced LMS arising from the anterior fossa of the skull or meninges and extends to the frontal sinus and ethmoid air cells.
CONCLUSION
Primary intracranial LMS is an extremely rare diagnosis and presenting symptoms vary with the location and size of the tumor. Due to the poor specificity of clinical symptoms, diagnosis is often based on histology. The most common treatment is surgical resection. Adjuvant chemotherapy with various agents has been found to be somewhat effective outside the central nervous system. When LMS does occur, a history of immunocompromised state or previous radiation exposure is often present. Pathological confirmation is required for an appropriate diagnosis.
PubMed: 37941634
DOI: 10.25259/SNI_647_2023