Secondary tumors after retinoblastoma associated with high mortality

Despite aggressive treatment, the prognosis is poor for patients with hereditary retinoblastoma who develop secondary tumors, according to a study by French researchers.

Isabelle Aerts, MD, and colleagues at the Institut Curie, in Paris, conducted the study, which evaluated outcomes of treatment in 25 patients with secondary malignant neoplasms (SMN) after retinoblastoma.

“It is important to provide information to retinoblastoma patients regarding the risk of a second tumor as this may facilitate an early tumor detection,” the authors said.

Twelve of the 25 patients were diagnosed with osteosarcomas, 12 patients with soft tissue sarcomas and one with a malignant oligodendroglioma. The SMN was located inside the radiation field in 21 cases and outside in four cases, according to the study authors.

Preoperative chemotherapy was administered to 23 patients, 16 patients underwent surgical removal and 12 patients received postoperative chemotherapy. Two patients underwent external beam radiotherapy.

Complete remission was achieved in 14 of 24 evaluable patients and partial remission in two patients. Progressive disease remained in eight patients, the authors noted.

After a mean follow-up of 8.8 years, only six patients survived, the authors said.

The study is published in the European Journal of Cancer.