Malignancies in patients with cystic fibrosis: a case series

Our plain-English summary of Malignancies in patients with cystic fibrosis: a case series, by D. Appelt, T. Fuchs, G. Steinkamp, et al (Journal of Medical Case Reports, 2022).

The study

• Authors: D. Appelt, T. Fuchs, G. Steinkamp, H. Ellemunter
• Journal: Journal of Medical Case Reports
• Published: 2022;16(1):27
• Type of article: Case report
• Cited by: 16 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Background: Previous 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 Presentation: Among 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.

Conclusions: Most 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.

© 2022. The Author(s).

In plain English

This article was a case series from the Cystic Fibrosis Centre Innsbruck in Austria. The authors went back through their center's patient records for any cancer among the 229 patients with cystic fibrosis treated there between 1995 and 2019. They found 11 people with a malignant disease. Ten were women and one was a man. The median age at diagnosis was 25.2 years (mean 24.3). Four women had cervical cancer or cervical intraepithelial neoplasia. Other cancers included two cases of acute lymphocytic leukemia, malignant melanoma, neuroblastoma, adrenal carcinoma, thyroid cancer and one peritoneal mesothelioma. The mesothelioma patient was a 28-year-old woman with cystic fibrosis liver disease and fluid building up in her abdomen. A biopsy showed malignant deciduoid mesothelioma, and she died six weeks after hospital admission. The article did not mention asbestos or any exposure history for her. Six deaths were attributed to cancer. By the authors' calculation, the cervical cases represented an incidence 49 times that of the regional general population. The authors concluded that their data suggest an increased risk of gynecological cancers. They noted that the study was retrospective and small.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Retrospective case series from one cystic fibrosis center's patient database
• Who was studied: 229 patients with cystic fibrosis treated at the Cystic Fibrosis Centre Innsbruck, Austria, between 1995 and 2019; 11 were diagnosed with cancer (10 women, 1 man)
• Exposure: The study concerned cancer in people with cystic fibrosis. The article reported no asbestos or other occupational or environmental exposure for any patient, including the patient with peritoneal mesothelioma.
• Main results:
  • Of the 11 patients with cancer, 10 were female and 1 was male
  • Median age at cancer diagnosis was 25.2 years (mean 24.3 years) among the 11 patients
  • Four of the ten women had cervical cancer or cervical intraepithelial neoplasia
  • One patient (case 7), a 28-year-old woman with cystic fibrosis liver disease and ascites, was diagnosed by fine-needle biopsy with malignant deciduoid mesothelioma (listed as peritoneal mesothelioma) and died six weeks after hospital admission
  • Six deaths among the 11 patients were attributed to cancer
  • The authors calculated that the 4 cervical cases among 229 patients over 24 years corresponded to 0.073% of patients per year, compared with 0.0015% in the general population of Tyrol for the same age range, which they described as a 49-times-higher incidence
  • Only 1 of the 11 patients had received a lung transplant
• The authors' conclusion: The authors concluded that their data suggest an increased risk of gynecological malignancies in cystic fibrosis. They recommended screening for gastrointestinal and gynecological cancers, and they called for larger studies.
• Limitations the authors state:
  • The study was retrospective, involved a small number of patients, and covered a period during which cystic fibrosis treatment was constantly changing
  • Some cancers, such as neuroblastoma or leukemia, may have occurred by coincidence

Topics

Medical terms in this study, explained in our Lexicon: Fibrosis, Colorectal Cancer, Peritoneal Mesothelioma, Carcinoma, Lung Transplant.

Related studies

• Benign cystic mesothelioma: a case series with one case complicated by pregnancy — O. Akbayir, A. Gedikbasi, et al. (2011)
• A CARE-compliant case report: Lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis — H. Huang, R. Feng, et al. (2017)
• Mesothelioma after lung transplantation — P.N. Chhajed, L. Bubendorf, et al. (2006)
• Malignant peritoneal mesothelioma: a series of 19 cases — T. van Gelder, H.C. Hoogsteden, et al. (1989)
• Multicystic peritoneal mesothelioma: outcomes and patho-biological features in a multi-institutional series treated by cytoreductive surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) — D. Baratti, M. Vaira, et al. (2010)
• Two-Stage Cytoreductive Surgery and Intraperitoneal Chemotherapy for Diffuse Malignant Peritoneal Mesothelioma: Predictors of Overall Survival in an Intention-to-Treat Series — J.C. Leinwand, R.N. Taub, et al. (2020)

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