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Retinal symatoma is a rare retinal tumor, most commonly seen in early childhood, usually caused by mutations in the RB1 tumor suppressor gene.
previous studies have shown that patients with this type of patients have an increased risk of developing malignant tumors (SMNs) and are genetically susceptible.
addition, treatment, especially radiotherapy, increases the risk of sarcoma and melanoma.
, however, it is still little known whether the disease increases the risk of onththic secondary malignancies (SMN) and multiple (≥2) SMNs.
in this study, the researchers quantified the risk of SMN events in 1,128 genetic and 924 non-hereditary retinal cell tumor survivors through long-term follow-up and detailed histological information (diagnosed between 1914 and 2006; follow-up through 2016).
compared the risk of cancer in patients with retinal scleoma with that of the general population through standardized rate of occurrence (SIR) analysis.
the associated competitive risks of death by assessing the cumulative incidence.
results showed a statistically significant increase in SMN risk in inherited retinal cytoma survivors (N=239;SIR=11.9), accompanied by SIR with 80 times the number of sarcoma, nasal tumors, and germ cell tumors.
researchers also observed a significant increase in the risk of melanoma and central nervous system, oral and mammary SMNs (SIRs= 3.1-17), but no increase in risk of uterus, kidneys, lungs, bladder, pancreas, and other types of tumors.
The cumulative rate of SMN after diagnosis of retinal syringe tumor researchers further analyzed the cumulative rate of cumulative occurrence in patients with hereditary retinal symatoma, with the cumulative rate of the first SMN being 33.1% and the second SMN being 6.0%.
risk of non-hereditary retinal syringe tumors did not increase.
addition to the increased risk of sarcoma and melanoma after diagnosis in patients with hereditary retinal cytoma, the study confirms that the risk of epithelocytic malignancies is higher than previously reported.
the cumulative rate of the disease revealed the long-term SMN burden in patients with hereditary retinal scleoma.
