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Oligodendroglioma of the ciliary body: a unique case report and the review of literature
© Guo et al; licensee BioMed Central Ltd. 2010
Received: 2 December 2009
Accepted: 23 October 2010
Published: 23 October 2010
To date, there is no report in the international literature of an oligodendroglioma of the ciliary body, nor is there an analysis of the possible origins of this lesion.
Here we report on a 52-year-old man admitted to our hospital with a ciliary body tumor revealed by clinical examination and ultrasound, computed tomography and magnetic resonance imaging studies. Following enucleation, pathological and immunohistochemical analyses were performed. Postoperative histopathological staining results included OLIGO-2(+) and GFAP(-), leading to a pathological diagnosis of oligodendroglioma of the ciliary body in the right eye (WHO grade II).
Since malignant gliomas derive from transformed neural stem cells, the presence of oligodendroglioma in the ciliary body supports the hypothesis that gliomas can occur wherever neural stem cells exist. Tumors of the ciliary body derived from oligodendrocytes are difficult to diagnose; pathological analyses are essential.
The list of most commonly occurring primary brain tumors includes gliomas, and the vast majority of these are astrocytomas or oligodendrogliomas. The latter usually originate from oligodendrocytes in the brain parenchyma, especially in adult patients. To our knowledge,the oligodendroglioma of the retina [1, 2] and the ciliary body astrocytoma [3–6] have been reported in the literature, but the oligodendroglioma of the ciliary body has not been reported, nor is there an analysis of the possible origins of this lesion. Here we describe the first case of this tumor type occurring in the right ciliary body of a patient who presented with severely compromised vision.
The pathogenesis of oligodendrogliomas of the ciliary body should be consistent with that for intracranial gliomas, which originate from neural stem cells. Neural stem cells are a group of cells that differentiate into neurons, glial cells and oligodendrocytes . They are found in the brain tissue of human embryos, and they persist into adulthood. Retinas differentiate from the neuroectoderm and as such can be considered extensions of the brain. Neural stem cells may be found in the retina , and in 2004 Coles found that retinal stem cells are located in the lateral half of the iris, in the pars plana and in ciliary processes . As a specific subset of neural stem cells, retinal stem cells are pluripotent. Like mitogen-responsive neural stem cells, retinal stem cells can be induced to differentiate into neurons and glial cells. The eyes of adult Ahmad rats, when placed into a mitogenic medium (containing bFGF and EGF), formed a single-cell culture, thereby confirming that neural precursor cells of ciliary pigmented epithelium can differentiate into star-shaped glial cells and oligodendrocytes .
Our case provides further confirmation that gliomas might occur wherever neural stem cells exist. Malignant gliomas derive from transformed neural stem cells. Oligodendrogliomas and astrocytomas of the ciliary body originate from oligodendrocytes and astrocytes that have differentiated from retinal stem cells. Ciliary retinal stem cells have the same properties of mobility and pluripotentiality as neural stem cells. When stimulated by carcinogenic factors, retinal stem cells can give rise to tumors of a variety of cell types. It is possible for stem cells derived from retinal ciliary epithelium to be well-differentiated and mature enough to replace dead retinal neurons when surgically transplanted[13–15]. Furthermore, ciliary retinal stem cells, when placed into an environment of retinal growth and development, have more cell differentiation potential for retinal transplantation than do other types of stem or progenitor cells. Therefore, finding an oligodendroglioma of the ciliary body provides evidence for the presence of retinal stem cells in this anatomical location. This knowledge may ultimately lead to novel approaches to treatment of numerous degenerative diseases such as retinitis pigmentosa, the advanced stages of glaucoma, age-related macular degeneration, and optic nerve atrophy.
Definitive diagnosis of oligodendroglioma of the ciliary body can be made only after results of pathological and immunohistochemical analyses are known. Although extremely rare, preoperatively oligodendroglioma should be included in the differential diagnosis of ciliary body tumors.
Written consent was obtained from the patient for publication of the case report.
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