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Survivors of fusion-negative sarcoma at increased risk for second malignancies
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Survivors of sarcoma demonstrated an increased risk for second malignant neoplasms, according to study results published in Cancer.
Survivors of fusion-negative tumors faced a greater risk than those with fusion-positive tumors.
“Individuals with alveolar rhabdomyosarcoma — mostly fusion-positive soft tissue sarcomas — are less likely to have a first-degree relative with cancer or to develop a second malignancy compared with those with embryonal or pleomorphic rhabdomyosarcoma (fusion-negative soft tissue sarcomas),” Philip J. Lupo, PhD, assistant professor of pediatrics–oncology at Baylor College of Medicine in Houston, and colleagues wrote. “Based on this growing evidence, we hypothesized that individuals with fusion-negative sarcomas were more likely to carry germline cancer-predisposing lesions and, therefore, more likely to develop second malignant neoplasms compared with those with fusion-positive sarcomas.”
Lupo and colleagues used the SEER database to identify individuals with no prior cancer history who were diagnosed with sarcoma between birth and 39 years of age (n = 8,785; 76.8% white; 55.3% male).
Fusion-positive malignancies occurred in 54.9% of patients. These patients were more likely than those with fusion-negative malignancies to be female, older at the time of diagnosis and treated with radiotherapy (P < .001).
Patients with fusion-positive malignancies were more likely to survive at least 5 years after diagnosis (79.3% vs. 72%).
Researchers monitored these individuals for second malignant neoplasms.
After disqualifying all tumors diagnosed within 6 months of the initial sarcoma and subsequent malignancies with similar histology, the researchers identified 166 second malignancies.
The median time to diagnosis of second malignancy was 5.6 years (range, 0.5 to 20.3 years).
Patients who survived fusion-negative sarcoma tumors demonstrated a nearly threefold greater risk for second malignancy compared with the reference population (standardized incidence ratio [SIR] = 2.89; 95% CI, 2.3-3.59). Those who survived fusion-positive sarcoma tumors demonstrated nearly twice the risk for second malignancy as the reference population (SIR = 1.86; 95% CI, 1.48-2.3).
“The results of the current study confirm the high second malignancy neoplasm risk experienced by survivors of sarcoma, and reveal a higher incidence of second malignancy neoplasms in survivors of fusion-negative sarcoma compared with survivors of fusion-positive sarcoma,” Lupo and colleagues wrote. “We propose that sarcoma-predisposing germline mutations may contribute to tumor initiation and subsequent second malignancy neoplasm risk in individuals with fusion-negative sarcomas, but not fusion-positive sarcomas. This may inform a growing body of evidence for tailored second malignancy neoplasm surveillance among survivors of sarcoma based on germline mutational profiles.” – by Andy Polhamus
Disclosure: The researchers report no relevant financial disclosures.
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