Diktyoma
A rare, primitive neuroectodermal tumor of the eye
Diktyoma[edit | edit source]
Diktyoma, also known as medulloepithelioma, is a rare, primitive neuroectodermal tumor that primarily affects the eye. It is most commonly found in children and is characterized by its origin from the non-pigmented ciliary epithelium of the ciliary body.
Pathophysiology[edit | edit source]
Diktyomas are believed to arise from the primitive medullary epithelium, which is a remnant of the embryonic neural tube. These tumors are composed of neuroepithelial cells that can differentiate into a variety of cell types, including neuronal, glial, and epithelial cells. The histological appearance of diktyoma is often described as resembling the embryonic neural tube, with rosettes and tubular structures.
Clinical Presentation[edit | edit source]
Patients with diktyoma typically present with symptoms related to the eye, such as decreased vision, eye pain, or visible mass in the eye. In some cases, the tumor may cause secondary glaucoma or retinal detachment.
Diagnosis[edit | edit source]
The diagnosis of diktyoma is primarily based on clinical examination and imaging studies. Ultrasound and magnetic resonance imaging (MRI) are commonly used to assess the extent of the tumor. Definitive diagnosis is made through histopathological examination of the tumor tissue, which reveals the characteristic features of medulloepithelioma.
Treatment[edit | edit source]
The treatment of diktyoma depends on the size and location of the tumor, as well as the presence of any complications. Surgical removal of the tumor is the primary treatment modality. In some cases, enucleation of the affected eye may be necessary. Adjuvant therapies, such as radiation therapy or chemotherapy, may be considered in cases where complete surgical resection is not possible.
Prognosis[edit | edit source]
The prognosis for patients with diktyoma varies depending on the extent of the disease and the success of treatment. Early detection and complete surgical removal of the tumor are associated with a better prognosis. However, the potential for local recurrence and metastasis exists, particularly in cases where the tumor is not completely resected.
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