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Retinoblastoma survivors carry increased risk of soft-tissue sarcomas for decades
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Patients who survive hereditary retinoblastoma have an increased risk of developing soft-tissue sarcomas for decades after their initial diagnosis, according to a study published in the Journal of the National Cancer Institute. Patients treated with radiotherapy were particularly at increased risk, the study authors found.
Ruth A. Kleinerman, MPH, an epidemiologist at the National Cancer Institute, and colleagues evaluated the risks for developing individual soft-tissue sarcoma histologic subtypes in 1-year retinoblastoma survivors. The study included 963 patients diagnosed with hereditary retinoblastoma between 1914 and 1984.
The researcher identified 69 soft-tissue sarcomas in 68 patients. Leiomyosarcoma, the most frequent subtype, was found in 23 cases (33%), of which 78% were diagnosed 30 years or more after the initial retinoblastoma diagnosis.
Fibrosarcoma, the next most common subtype, was identified in 13 cases (19%), followed by malignant fibrous histiocytoma in 12 cases (17%), rhabdomyosarcoma in eight cases (12%) and liposarcoma in three (4.3%). Another 10 cases (15%) had nonspecified soft-tissue tumors and sarcomas.
Sixty-six of the 69 soft-tissue sarcomas occurred in patients who had received radiation treatment, the authors noted.
The researchers found that patients treated with radiotherapy had a significantly greater risk for developing soft-tissue sarcomas within the field of radiation. These patients also had an increased risk for such sarcomas outside the field of radiation, particularly for leiomyosarcomas.
At 50 years after radiotherapy, patients with hereditary retinoblastoma had a 13.1% cumulative risk for developing any soft-tissue sarcoma. These patients also had an 8.9% cumulative risk for developing a soft-tissue sarcoma within the radiation field and a 5.1% risk outside the radiation field, according to the study authors.
The researchers found that patients treated without radiation had an increased risk for soft-tissue sarcomas as well. This finding suggests that these patients may have a genetic predisposition to soft-tissue sarcomas independent of radiation treatment, the authors said.
"Given the excellent survival of retinoblastoma patients, it is important that survivors continue to undergo regular medical surveillance for sarcomas in their adult years," they said.