Characterization of Intraocular Neuroectodermal Tumors
Summary
Intraocular neuroectodermal tumours comprise a heterogeneous group of rare embryonal neoplasms arising from primitive neural crest or neuroepithelial precursors within the eye. They most commonly present in childhood but can occasionally be diagnosed in young adults. Histologically, these tumours exhibit patterns of small, round undifferentiated cells, often forming rosettes or pseudorosettes. Molecular profiling has revealed abnormalities in key developmental pathways, including alterations in DICER1 in medulloepithelioma and overexpression of stem-cell markers such as SOX2 in embryonal variants. Imaging modalities such as ultrasonography, magnetic resonance imaging and computed tomography assist in defining tumour extent, internal architecture and complications such as secondary glaucoma. Clinically, patients may present with leukocoria, proptosis, pain, cataract or neovascular glaucoma. Management strategies balance globe preservation against the risk of local invasion or metastasis, and may include enucleation, local resection, radiotherapy and systemic chemotherapy. Ongoing research seeks to refine diagnostic criteria through immunohistochemical markers and to identify novel therapeutic targets, with the ultimate goal of improving visual prognosis and survival while minimising treatment-related morbidity.
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Characterization of Intraocular Neuroectodermal Tumors publication trend
The graph below shows the total number of articles in characterization of intraocular neuroectodermal tumors across all publications each year (not limited to Nature Index journals).
Technical terms
Neuroectodermal tumour: A neoplasm derived from embryonic neural crest or neuroepithelial cells within the eye.
Medulloepithelioma: A rare congenital intraocular tumour arising from non-pigmented ciliary epithelium, often associated with DICER1 mutations.
Rosette: A circular arrangement of tumour cells around a central lumen, indicative of neuroectodermal differentiation.
Pseudorosette: A radial arrangement of tumour cells around a central neuropil without a true lumen.
Immunohistochemistry: A laboratory method using labelled antibodies to detect specific antigens in tissue sections, aiding in tumour classification.
References
- Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit. Indian Journal of Ophthalmology (2009).
- Intraocular neuroectodermal embryonal tumor in two rabbits. Veterinary Ophthalmology (2023).
- Intraocular medulloepithelioma: an unusual and challenging entity in paediatric population.. Journal of the Pakistan Medical Association (2023).
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