Systemic sclerosis and malignancy: a review of the literature.

Malignancy is associated with systemic sclerosis in between 3.6 and 10.7% of patients diagnosed with systemic sclerosis. The diagnosis of systemic sclerosis may occur before, concurrent with, or after the diagnosis of malignancy. All published reports of malignancy with systemic sclerosis are reviewed. Lung cancer is the most frequent type of cancer seen in patients with systemic sclerosis, followed by breast cancer. Risk factors for the development of malignancy in patients with systemic sclerosis are female gender, increased age, and diffuse systemic sclerosis. Autoantibodies such as anticentromere and antitopoisomerase I are inconsistent in their risk for developing malignancy. Vigilance is recommended in this group of patients based on their increased risk of developing cancer.