Abstract
BackgroundPrevious reports have shown an increased number of colorectal cancers in patients with cystic fibrosis. We assessed the database of our cystic fibrosis center to identify patients with all kinds of cancer retrospectively. All patients visiting the Cystic Fibrosis Centre Innsbruck between 1995 and 2019 were included.Case presentationAmong 229 patients with cystic fibrosis treated at the Cystic Fibrosis Centre in Innsbruck between 1995 and 2019, 11 subjects were diagnosed with a malignant disease. The median age at diagnosis was 25.2 years (mean 24.3 years). There were four gynecological malignancies (cervical intraepithelial neoplasia and cervical cancer), two hematological malignancies (acute lymphocytic leukemia), one gastrointestinal malignancy (peritoneal mesothelioma), and four malignancies from other origins (malignant melanoma, neuroblastoma, adrenocortical carcinoma, and thyroid cancer). One malignancy occurred after lung transplantation. There was a strong preponderance of females, with 10 of the 11 cases occurring in women. Six deaths were attributed to cancer.ConclusionsMost diagnoses were made below 30 years of age, and half of the subjects died from the malignant disease. Awareness of a possible malignancy is needed in patients with atypical symptoms. Regular screenings for cancer should also be considered, not only for gastrointestinal tumors.
Highlights
ConclusionsMost diagnoses were made below 30 years of age, and half of the subjects died from the malignant disease
Previous reports have shown an increased number of colorectal cancers in patients with cystic fibrosis
Most diagnoses were made below 30 years of age, and half of the subjects died from the malignant disease
Summary
Further studies about cancer risk in patients with CF and a larger patient collective are needed. The data could be obtained from national patient registries. Improvement of life expectancy in patients with CF increases the expected absolute risk of malignancies; it is necessary to integrate screening in the care of patients with CF. Our data suggest an increased risk for gynecological malignancies. We recommend screening for gastrointestinal and gynecological malignancies. Special alertness for malignant diseases is obviously needed in patients after transplantation due to long-term immunosuppression. Screening for other types of malignant tumors should be included in the regular follow-up of adult patients. The role of CFTR in risk of cancer may change considering the effect of new CFTR modulator therapies on CFTR, and this needs to be investigated in further studies
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