Secondary Sinonasal Malignancies After Retinoblastoma.

PURPOSE: Retinoblastoma (RB) is the most common intraocular tumor in childhood. Survivors of RB are at risk of developing secondary malignancies. Prior radiotherapy substantially increases the risk of malignancy in the orbit, brain, and nasal cavities. The authors report 2 new cases of secondary sino-orbital malignancies in RB survivors who underwent radiotherapy during early childhood. A literature review on secondary sinonasal and orbital malignancies in RB is presented.

METHODS A: case series and literature review of relevant publications on the topic was performed, with appropriate data extracted.

RESULTS

Twelve cases of secondary sinonasal and orbital malignancies in RB survivors have been reported, including 5 cases of adenocarcinoma, 2 cases of sinonasal neuroendocrine carcinoma, and 2 cases of olfactory neuroblastoma were also reported. Single cases each of leiomyosarcoma and squamous cell carcinoma were reported. Secondary malignancies are more common in hereditary RB with germline mutations in the RB1 gene. Radiotherapy significantly increases the risk of secondary malignant neoplasms. Secondary sinonasal malignancies are rare in RB survivors.

CONCLUSIONS

Ophthalmologists should be vigilant of the increased risk of developing secondary orbital and sinonasal malignancies in patients with retinoblastoma, especially in germline mutations and postradiotherapy. Life-long follow-up in RB survivors is recommended to detect and effectively manage these rare but fatal secondary malignant neoplasms.