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Uveal Melanoma of the choroid is the most common primary intraocular malignant tumor in adults. This tumor originates from the vascular membrane in the eyeball, and the posterior pole of the eye is a common site with high malignancy. It is easy to metastasize to organs such as the liver and lungs through blood circulation, and about half of the metastatic patients have a median survival of less than one year. Its occurrence is related to high-frequency mutations in the GNAQ/11 gene and abnormal regulation of ROS. The clinical manifestations are divided into choroidal type and intraocular diffusion type, among which choroidal type can form mushroom shaped masses. Diagnosis mainly relies on techniques such as fundus examination combined with fluorescein angiography, B-ultrasound, and MRI, and should be distinguished from various fundus diseases. The treatment plan should be developed based on the size of the tumor and the classification of genetic testing. Close range radiotherapy is the mainstream method for eye protection, while enucleation surgery is suitable for late stage patients. Metabolic reprogramming is an important mechanism for liver metastasis of this tumor, and abnormal activation of propionate carboxylase promotes tumor progression by affecting glutamine metabolism.
Figure 1. Uveal Melanoma.
The occurrence and development of uveal Melanoma is a complex process involving multiple factors, stages, accumulation of multiple gene variations, and interactions. Currently, it is widely believed that abnormal expression of oncogenes and tumor suppressor genes leads to cellular malignancy, and these genes typically play a role in regulating cell proliferation, division, and differentiation.
Uveal Melanoma of the choroid can develop inward or outward. If it develops outward, it can cause early spread outside the eyes, and clinically there may be eyeball protrusion, but the changes in the fundus are not significant. Inward development causes a spherical protrusion under the retina, which develops rapidly, has a short course of disease, early visual impairment, and widespread retinal detachment, making it a common clinical condition. There is also a relatively rare type that develops along the choroidal plane, forming diffuse, flat proliferation without forming localized elevations. This type develops slowly and has a long course of illness, making it easy to miss diagnosis. The following are the classifications of each type:
| Clinical Manifestations | Details |
| Choroidal Uveal Melanoma | Due to the external limitation of the sclera and the internal limitation of the vitreous membrane, it expands along the choroidal surface in the early stage. Before the glass membrane is punctured, the tumor appears as a flat oval shape with low elevation, and the retinal changes on the tumor surface are not significant and the development is relatively slow. Once the glass membrane is punctured, the tumor rapidly expands in the subretinal space due to the loss of its original limitations, forming a typical mushroom shaped appearance with a large base, round head, and thin neck. |
| Uveal Melanoma of the ciliary body | There may be different clinical manifestations depending on the location. Moving forward can easily cause an increase in intraocular pressure and detachment of the iris root; Backward development can lead to peripheral retinal detachment, which may only manifest clinically as a phenomenon similar to central retinal disease or papillitis. Without dilated pupil examination, misdiagnosis or missed diagnosis is often prone to occur. A mass that develops from the ciliary body towards the center can compress the lens, causing it to shift or dislocate. For those who spread outward in the early stage, the lump may appear under the anterior conjunctiva. |
| Patients with uveal melanoma spreading within the eye and along the optic nerve | It is relatively rare, but systemic metastasis along the bloodstream is more common. Metastasis is most common in the liver, followed by subcutaneous tissue and lungs, and can also invade the central nervous system. The incidence of metastasis is related to the cell type of the tumor, with epithelioid cell type being the highest and spindle cell type being the lowest. If measured by reticular fibers, the higher the content of reticular fibers, the lower the transfer rate; On the contrary, the lower the content of reticular fibers, the higher the transfer rate. |
In addition to inquiring about medical history and clinical symptoms in detail, conducting detailed clinical examinations, synthesizing all clinical data, analyzing comprehensively, and making cautious judgments. Ophthalmic examination can serve as a diagnostic basis. The entire course of the disease can be roughly divided into four stages: intraocular phase, secondary glaucoma phase, extraocular spread, and systemic metastasis phase, but the evolution of the four stages may not be gradual. It can spread out of the eye or metastasize throughout the body without undergoing glaucoma. Throughout the course of the disease, death can occur due to systemic metastasis of the tumor. The higher the late stage, the higher the systemic metastasis rate.
1. Secondary glaucoma or tumor necrosis can induce uveitis or endophthalmitis.
2. Ocular lesions can also be accompanied by abnormal pupils (the pupils in the corresponding area are unresponsive, difficult to dilate, or not perfectly round).
3. Obstruction of blood flow reflux or local tumor necrosis can cause scleritis.
The prognosis of Uveal Melanoma of the choroid is better than that of Uveal Melanoma of the skin, with a mortality rate of 50% for the former and up to 80% for the latter. Local recurrence or metastasis mostly occurs within 1 year after surgery, and in some cases, it can be prolonged for 5 or more years. There is a close relationship between prognosis and tumor cell types, with spindle cell type having a lower mortality rate and epithelial cell type having a higher mortality rate.
| Clinical Manifestations | Details |
| Radiotherapy | Stallard used radium needles and subsequently developed patches of cobalt (Co), iodine (I), ruthenium (Ru), iridium (Ir), gold (Au), palladium (Pa), etc. for radiation therapy, which damaged the DNA of tumor cells to destroy them, damaged tumor blood vessels, and caused ischemic necrosis of tumor tissue. The current radiation therapies used to treat choroidal melanoma include scleral surface patch radiotherapy, charge particle beam radiotherapy, and gamma knife therapy. After decades of clinical observation, radiation therapy has achieved encouraging results and has become one of the main treatment methods for choroidal melanoma in many countries. |
| Local excision | Since Raubitschek first proposed surgical treatment for uveal Uveal Melanoma in 1914, only a few reports have suggested local tumor resection as an alternative to traditional enucleation. In 1961, Stallard proposed that local surgical resection could be used to preserve the affected eye and save vision in the event of radiation therapy failure for Uveal Melanoma of the choroid in one eye. |
| Immunotherapy | This disease is related to immunity and can be attacked by specific melanoma transfer factors, genetically engineered melanoma monoclonal antibodies and their biological missiles, cytokines (rIL-2, rIFN, rTFN, etc.), TIL, LAK cells, etc; Tumors can also be localized or even eliminated by enhancing the body's immune system. Other immune enhancers, such as BCG, Bacillus subtilis, yeast polysaccharides, Poria cocos, Poria cocos, Ganoderma lucidum polysaccharides, levamisole, etc., all have immune enhancing effects. |
| Cat. No. | Product Name | Host | Isotype | Application | |
| DMAB7005 | Anti-Melanoma monoclonal antibody, clone INC46 | Mouse | IgG1 | IHC-P | Inquiry |
| DMAB5506MH | Anti-Melanoma monoclonal antibody, clone QOM3 | Mouse | IgG1 | IHC | Inquiry |
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