Low-grade Appendiceal Mucinous Neoplasm (Appendiceal Villous Adenoma) with Cystic Fibrosis: A Case Report

Emrah Doğan,Muge Kuzu Avcı,Korkut Bozkurt,Ozge Oral Tapan,Utku Tapan,Hakan Hakan Avcı

doi:10.15388/lietchirur.2021.20.38

Emrah Doğan, Muge Kuzu Avcı + Show 4 more

Open Access

https://doi.org/10.15388/lietchirur.2021.20.38

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Abstract

Cystic fibrosis (CF) is an autosomal dominant disease characterized by the dysfunction of exocrine secretory glands resulting from a mutation in the transmembrane regulator protein (CFTR) gene. As life expectancy increases in patients with cystic fibrosis secondary to advances in treatment, advanced age malignancies secondary to cystic fibrosis emerge. Especially, the frequency of gastrointestinal system malignancies and colon cancers increases with aging. Appendiceal tumors are a rare entity and constitute less than 1% of gastrointestinal tumors. We presented a villous adenoma encountered in an 18-year-old male patient with CF accompanied by clinical and radiological findings. Our case is the first reported appendiceal tumor that emerged in patients with cystic fibrosis.

Highlights

Cystic fibrosis (CF) is an autosomal dominant disease characterized by the dysfunction of exocrine secretory glands resulting from a mutation in the transmembrane regulator protein (CFTR) gene [1]
We presented an appendiceal tumor encountered in an 18-year-old male patient with CF accompanied by clinical and radiological findings
Our case is the first reported appendiceal villous adenoma that emerged in patients with cystic fibrosis

Summary

Introduction

Cystic fibrosis (CF) is an autosomal dominant disease characterized by the dysfunction of exocrine secretory glands resulting from a mutation in the transmembrane regulator protein (CFTR) gene [1]. Our case is the first reported appendiceal villous adenoma that emerged in patients with cystic fibrosis. Case report An 18-year-old male patient was admitted to our hospital with abdominal pain. In X-ray chest graph, bilaterally tram-track linear opacities were observed [Figure 1]. These signs were interpreted as bronchiectasis due to CF. Low-grade appendiceal mucinous neoplasm (appendiceal villous adenoma) with Cystic Fibrosis: a case report no taken response to compression. He was operated on with a preliminary diagnosis of acute appendicitis. Villiform mucinous epithelial proliferation was observed to replace the normal appendix mucosa [Figure 2]. Villiform mucinous epithelial proliferation appears to be composed of long mucinous epithelial cells with a low degree of nuclear atypia [Figure 4]

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Conclusion