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Neurosurgery Clinics of North America Jul 2023Meningiomas are the most common intracranial brain tumor. Spheno-orbital meningiomas are a rare subtype that originate at the sphenoid wing and characteristically extend... (Review)
Review
Meningiomas are the most common intracranial brain tumor. Spheno-orbital meningiomas are a rare subtype that originate at the sphenoid wing and characteristically extend to the orbit and surrounding neurovascular structures via bony hyperostosis and soft tissue invasion. This review summarizes early characterizations of spheno-orbital meningiomas, presently understood tumor characteristics, and current management strategies.
Topics: Humans; Meningioma; Treatment Outcome; Sphenoid Bone; Neoplasm Recurrence, Local; Brain Neoplasms; Meningeal Neoplasms
PubMed: 37210130
DOI: 10.1016/j.nec.2023.02.006 -
Klinische Monatsblatter Fur... Sep 2023Malignant masses of the orbit include a large variety of neoplasms of epithelial or mesenchymal origin. The treatment of orbital malignancies is an essential...
Malignant masses of the orbit include a large variety of neoplasms of epithelial or mesenchymal origin. The treatment of orbital malignancies is an essential interdisciplinary field of medicine that integrates ENT medicine, facial surgery, plastic surgery, neurosurgery, oncology and radiology.The main symptom of malignant orbital masses is the exophthalmos. A symptom that can help to differentiate a benign from a malignant orbital mass can be the pain. The main diagnostic tool is the MRI including new sequences like DWI and DCE.After presenting symptoms and diagnostic strategies of malignant orbital masses, this article starts with the description of malignant epithelial neoplasms of the lacrimal gland. Furthermore, it describes new insights in orbital lymphomas, followed by the discussion of semimalignant orbital masses. Last but not least the text deals with malignant neoplasms of the skin that can grow secondarily in the orbit. Finally, the manuscript discusses orbital metastases.
Topics: Humans; Orbit; Orbital Neoplasms; Magnetic Resonance Imaging; Orbital Diseases; Exophthalmos
PubMed: 37586398
DOI: 10.1055/a-2129-1194 -
Blood Reviews Nov 2023The guidelines for classification, prognostication, and response assessment of myelodysplastic syndromes/neoplasms (MDS) have all recently been updated. In this report... (Review)
Review
Classification, risk stratification and response assessment in myelodysplastic syndromes/neoplasms (MDS): A state-of-the-art report on behalf of the International Consortium for MDS (icMDS).
The guidelines for classification, prognostication, and response assessment of myelodysplastic syndromes/neoplasms (MDS) have all recently been updated. In this report on behalf of the International Consortium for MDS (icMDS) we summarize these developments. We first critically examine the updated World Health Organization (WHO) classification and the International Consensus Classification (ICC) of MDS. We then compare traditional and molecularly based risk MDS risk assessment tools. Lastly, we discuss limitations of criteria in measuring therapeutic benefit and highlight how the International Working Group (IWG) 2018 and 2023 response criteria addressed these deficiencies and are endorsed by the icMDS. We also address the importance of patient centered care by discussing the value of quality-of-life assessment. We hope that the reader of this review will have a better understanding of how to classify MDS, predict clinical outcomes and evaluate therapeutic outcomes.
Topics: Humans; Neoplasms; Myelodysplastic Syndromes; Risk Assessment; Quality of Life; Prognosis
PubMed: 37704469
DOI: 10.1016/j.blre.2023.101128 -
Radiologie (Heidelberg, Germany) Mar 2024Orbital tumours include a variety of orbital diseases of different origins. In the case of malignant orbital tumours, early detection is important so that treatment can... (Review)
Review
Orbital tumours include a variety of orbital diseases of different origins. In the case of malignant orbital tumours, early detection is important so that treatment can be initiated promptly. Neuroradiological imaging, in particular magnetic resonance imaging (MRI), plays an important role in the diagnostic of orbital tumours. In adults, lymphoproliferative diseases, inflammations and secondary orbital tumours are most frequently found, whereas in children mostly dermoid cysts, optic gliomas and capillary haemangiomas are found. Optic glioma is a pilocytic astrocytoma and accounts for two thirds of all primary optic tumours. Optic nerve sheath meningiomas mostly affect middle-aged women. In childhood, retinoblastoma is the most common intraocular tumour. This is an aggressive malignant tumour which can occur unilaterally or bilaterally. Based on the imaging findings, differential diagnoses can usually be easily narrowed down using criteria such as age of manifestation, frequency, localisation and imaging characteristics.
Topics: Adult; Child; Middle Aged; Humans; Female; Orbital Neoplasms; Orbital Diseases; Optic Nerve Neoplasms; Optic Nerve Glioma; Meningeal Neoplasms; Retinal Neoplasms
PubMed: 38194103
DOI: 10.1007/s00117-023-01257-x -
Current Pain and Headache Reports Dec 2023We aim to present diagnostic considerations and management insights on Tolosa-Hunt syndrome. We highlight recent controversies in this field and emerging literature. (Review)
Review
PURPOSE OF REVIEW
We aim to present diagnostic considerations and management insights on Tolosa-Hunt syndrome. We highlight recent controversies in this field and emerging literature.
RECENT FINDINGS
The diagnostic categorization of Tolosa-Hunt syndrome remains controversial, with imaging negative cases described in the literature and an increasing literature of secondary causes falsely diagnosed as Tolosa-Hunt syndrome. Response to steroids can fulfill diagnostic criteria, but newer management strategies are available in treatment-resistant patients, such as steroid-sparing agents or radiotherapy. Tolosa-Hunt syndrome has become controversial; the entity of granulomatous inflammation of the cavernous sinus and possible extension into orbital fissure and posterior orbit of late has been re-evaluated. Recent case series and reviews have outlined multiple false positive (neoplasm or infection) and negative (no imaging findings) diagnoses. In the future, when assessing whether a patient has this entity, we caution readers to closely follow patients for secondary causes and consider this entity in differential even if MRI is non-revealing. Additionally, biopsy should be regarded as a gold standard for diagnosis and utilized, especially in uncertain cases. The diagnostic categorization of Tolosa-Hunt syndrome may need reconsideration in future versions of the ICHD.
Topics: Humans; Tolosa-Hunt Syndrome; Diagnosis, Differential; Cavernous Sinus; Magnetic Resonance Imaging; Steroids
PubMed: 38032539
DOI: 10.1007/s11916-023-01193-4 -
Nature Genetics Dec 2023Circular extrachromosomal DNA (ecDNA) in patient tumors is an important driver of oncogenic gene expression, evolution of drug resistance and poor patient outcomes....
Circular extrachromosomal DNA (ecDNA) in patient tumors is an important driver of oncogenic gene expression, evolution of drug resistance and poor patient outcomes. Applying computational methods for the detection and reconstruction of ecDNA across a retrospective cohort of 481 medulloblastoma tumors from 465 patients, we identify circular ecDNA in 82 patients (18%). Patients with ecDNA-positive medulloblastoma were more than twice as likely to relapse and three times as likely to die within 5 years of diagnosis. A subset of tumors harbored multiple ecDNA lineages, each containing distinct amplified oncogenes. Multimodal sequencing, imaging and CRISPR inhibition experiments in medulloblastoma models reveal intratumoral heterogeneity of ecDNA copy number per cell and frequent putative 'enhancer rewiring' events on ecDNA. This study reveals the frequency and diversity of ecDNA in medulloblastoma, stratified into molecular subgroups, and suggests copy number heterogeneity and enhancer rewiring as oncogenic features of ecDNA.
Topics: Humans; DNA, Circular; Medulloblastoma; Retrospective Studies; Neoplasms; Oncogenes; Cerebellar Neoplasms
PubMed: 37945900
DOI: 10.1038/s41588-023-01551-3