Ciliary Body Medulloepithelioma
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Ciliary Body Medulloepithelioma

Ciliary body medulloepithelioma (CBM) is a highly unusual and ruthless cancer of the eye that originates from the ciliary body, the part of the eye in the uvea that produces the aqueous humor and enables accommodation of the lens. Here at Protheragen, we provide full support for the creation of diagnostic and therapeutic tools for ciliary body medulloepithelioma.

Overview of Ciliary Body Medulloepithelioma

Ciliary body medulloepithelioma is an uncommon intraocular tumor that usually arises from the undeveloped nonpigmented portion of the ciliary epithelium of the pars plicata. Non-hereditary, this embryonal tumor is most commonly found in children whose median age of presentation is between 2 to 5 years, although it may occur in adults in rare cases. Ciliary body medulloepithelioma is not thought to be familial but could be linked to illness that brings a predisposition to several tumors such as DICER1-related pleuropulmonary blastoma. It manifests with vision loss, eye ache, leukocoria, pain, and secondary glaucoma, which are misleadingly diagnosed as retinoblastoma or other intraocular problems like persistent hyperplastic primary vitreous.

Histopathological Analysis of Ciliary Body Medulloepithelioma.Fig.1 Histopathologic image of ciliary body medulloepithelioma by hematoxylin-eosin staining. (Coc I. R., et al., 2024)

Histopathological Examination of Ciliary Body Medulloepithelioma

Histopathology aids in identifying ciliary body medulloepithelioma. Nonteratoid and teratoid forms of primitive medulloepithelioma are distinguished by their cytologic patterns. Nonteratoid medulloepitheliomas have cords and sheets of medullary epithelial cells. The teratoid form has heteroplastic elements, which may contain cartilage, muscle, or even brain tissue. Malignancy is marked by poorly differentiated cells, heightened mitotic activity, and invasion of adjacent tissues. Commonly positive immunohistochemical markers, such as neuron-specific enolase (NSE), glial fibrillary acidic protein (GFAP), and vimentin, which further support the tumor's existence as neuroepitheliomal, are their further references.

Therapeutics Development for Ciliary Body Medulloepithelioma

Chemotherapy

  • Systemic Chemotherapy: VEC containing vincristine, etoposide, and carboplatin has been useful for tumor reduction prior to surgery and for the control of metastatic disease.
  • Intra-arterial Chemotherapy: Administering chemotherapy directly through the ophthalmic artery results in greater local concentration of the drug while lowering systemic toxicity and increasing the response to the tumor.

Targeted Therapy

  • Tyrosine Kinase Inhibitors (TKIs): Research is being conducted into the possible inhibition of tumor growth and angiogenesis via TKIs which act on the oncogenic pathways of VEGF or PDGF receptors.
  • Immunotherapy: Immunotherapy techniques such as immune checkpoint inhibitors are increasingly promising by using the body's immune system to target CBM.

Our Services

At Protheragen, we offer customized services tailored to the specific needs of our clients. Our team collaborates with the client to ensure that every step, from constructing a personalized diagnostic panel to designing a targeted therapy and even conducting a preclinical study, meets their unique needs.

Diagnostics Development

  • Karyotype Analysis Service
  • Omics Analysis Service
  • Biomarker Development Service
  • Artificial Intelligence Service

Therapeutic Development

  • Small Molecule Drug
  • Cell Therapy
  • Gene Therapy
  • Therapeutic Antibody
  • Therapeutic Peptide
  • Therapeutic Protein

Preclinical Research

  • Pharmacodynamics Study Services
  • Pharmacokinetics Study Services
  • Drug Safety Evaluation Services

Disease Models

  • Myc-Driven Mouse Models
  • DICER1-Related Mouse Models
  • Human CBME Cell-derived Xenograft Models
  • DICER1 Mutation Zebrafish Models

Protheragen's preclinical research services are designed to accelerate the development of new therapies for CBME. We offer a range of in vitro and in vivo models to study tumor biology, evaluate drug efficacy, and assess safety profiles. If you are interested in our services, please feel free to contact us.

References

  • Coc, Ivana Romac, et al. "Evaluation of Ciliary Body Medulloepitheliomas in candidates for phakic lens implantation." International Ophthalmology 44.1 (2024): 402.
  • Schmalfuss, Tiago Ribeiro, Egidio Picetti, and Helena Messinger Pakter. "Glaucoma due to Ciliary Body Medulloepitheliomas and pseudoplateau iris: a systematic review of the literature." Arquivos Brasileiros de Oftalmologia 81 (2018): 254-261.