Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review

Our plain-English summary of Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review, by R. Talerico, C. Cardillo, F. De Vito, et al (Frontiers in Immunology, 2020).

The study

• Authors: R. Talerico, C. Cardillo, F. De Vito, F. Schinzari, M. Soldato, M.C. Giustiniani, E. Verrecchia, R. Manna
• Journal: Frontiers in Immunology
• Published: 2020;11:889
• Type of article: Case report
• Cited by: 11 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

A 65-year-old Italian physician affected by Familial Mediterranean fever (FMF) was hospitalized due to progressive abdominal enlargement, which had begun 6 months before admission. Physical examination revealed ascites and bilateral leg edema. Abdominal CT scan showed ascitic fluid and extensive multiple peritoneal implants; peritoneal CT-guided biopsy revealed an epithelial-type malignant mesothelioma. The patient's past medical history revealed recurrent episodes of abdominal pain and fever from the age of 2. Clinical diagnosis of FMF was suspected at the age of 25, while genetic analysis, performed at the age of 50, confirmed homozygosity for the M694I mutation in the MEFV gene. Treatment with the first line FMF drug colchicine was started and stopped several times because of worsened leukopenia. The patient in fact had a history of asymptomatic leukopenia/lymphopenia from an early age; the intake of colchicine aggravated his pre-existing problem until the definitive suspension of the drug. As for second-line drugs, canakinumab was first prescribed, but due to prescription issues, it was not possible to be administered. When he was given anakinra, there was a worsening of leukopenia leading to septic fever. Systematic literature review indicates that, in most cases, recurrent peritoneal inflammation results in benign peritoneal fibrosis or less commonly in encapsulating peritonitis. There are only a few reported cases of recurrent peritoneal inflammation progressing from FMF to peritoneal mesothelioma (MST). In such cases, intolerance to colchicine or its erratic intake may lead to long-term recurrent inflammation, which usually precedes the development of the tumor, while pre-existing leukopenia, as in our patient, could also be a factor promoting or accelerating the tumor progression. In conclusion, we suggest that in the presence of intolerance or resistance to colchicine, interleukin (IL)-1 inhibition could suppress peritoneal inflammation and prevent MSTs.

Copyright © 2020 Talerico, Cardillo, De Vito, Schinzari, Soldato, Giustiniani, Verrecchia and Manna.

In plain English

This was a case report with a review of published cases. It described a 65-year-old Italian physician with familial Mediterranean fever (FMF), a hereditary inflammatory disorder, who was hospitalized in January 2019 with abdominal swelling. A biopsy showed epithelioid malignant mesothelioma of the peritoneum (the abdominal lining), and he died 2 weeks later. No asbestos exposure was evident in his history. He could not keep taking the standard FMF drug colchicine because it worsened his low white-cell counts. The authors said their literature search found nine peritoneal malignant and two benign cystic mesotheliomas in FMF patients, none with asbestos exposure, and that many of these patients used colchicine irregularly. Their table, however, lists ten patients, including one pleural case and one with unknown asbestos exposure. The authors noted that the link between FMF and mesothelioma is controversial. They suggested that, for patients who cannot tolerate colchicine or do not respond to it, drugs blocking interleukin-1 could suppress peritoneal inflammation and prevent mesothelioma.

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: Case report of one patient, combined with a literature review of previously reported FMF patients with mesothelioma
• Who was studied: One 65-year-old Italian physician with familial Mediterranean fever, hospitalized in January 2019 at the authors' center in Rome. The literature review covered previously published FMF patients with mesothelioma.
• Exposure: No asbestos exposure was evident; the patient had always worked in the Emergency Room. The exposure the authors discussed was long-standing recurrent peritoneal inflammation from FMF, poorly controlled because of colchicine intolerance.
• Main results:
  • In this patient, an abdominal CT scan showed multiple extensive peritoneal lesions. A CT-guided biopsy gave a diagnosis of epithelioid malignant mesothelioma.
  • The patient's condition deteriorated rapidly and he died 2 weeks after the diagnosis.
  • Colchicine at 1 mg/day was stopped several times because it worsened his neutropenia (below 1,500 per mm3). When he was given the interleukin-1 blocker anakinra, his leukopenia worsened and led to septic fever.
  • A peritoneal biopsy taken during hernia surgery at age 45 showed mononuclear cell infiltrates with a fibrotic appearance.
  • Genetic testing at age 50 showed the patient was homozygous for the M694I mutation in exon 10 of the MEFV gene.
  • In the literature review, the authors stated they collected nine cases of peritoneal malignant mesothelioma and two of peritoneal benign cystic mesothelioma in FMF patients.
  • The authors stated that none of the reviewed mesothelioma patients with FMF had a history of asbestos exposure. Their table lists asbestos exposure as "Unknown" for one patient.
  • Many of the reviewed cases reported poor compliance with colchicine because of its side effects.
  • This was the second mesothelioma case at the authors' center, which has followed 450 FMF patients since 1997. Neither of the two patients was treated with colchicine.
• The authors' conclusion: The authors said this and similar cases led them to support a relationship between chronic peritoneal inflammation and mesothelioma. They suggested that in patients intolerant of or resistant to colchicine, interleukin-1 inhibition could suppress peritoneal inflammation and prevent mesotheliomas.
• Limitations the authors state:
  • The authors acknowledged that the association between FMF and mesothelioma is controversial.
  • The authors noted that long-term follow-up of the reviewed benign cystic mesothelioma patients has not been described.

Topics

The National Library of Medicine indexes this study under subjects our site covers: Mesothelioma.

Medical terms in this study, explained in our Lexicon: Mesothelioma, Ascites, Bilateral, CT Scan, Biopsy, Systematic Review, Inflammation, Fibrosis.

Related studies

• Pleural mesothelioma in a patient with Familial Mediterranean Fever: A case report — A. Albtoosh, O.W. Alhasan, et al. (2026)
• Flaxseed lignans enriched in secoisolariciresinol diglucoside prevent acute asbestos-induced peritoneal inflammation in mice — R.A. Pietrofesa, A. Velalopoulou, et al. (2016)
• Case Report and Review of Literature: Familial Malignant Pleural Mesothelioma in a 39 Years Old Patient With an Inconclusive 18F-FDG PET/CT Result — M. Idkedek, K.S. Tahayneh, et al. (2022)
• Malignant Peritoneal Mesothelioma in an Infant With Familial ATM Mutations — A. Mijalovsky, D. Halperin, et al. (2018)
• Malignant peritoneal mesothelioma presenting with persistent high fever — L.Y. Chen, L.X. Huang, et al. (2011)
• Synthetic secoisolariciresinol diglucoside (LGM2605) inhibits Libby amphibole fiber-induced acute inflammation in mice — M. Christofidou-Solomidou, R.A. Pietrofesa, et al. (2019)

Browse more research in Frontiers in Immunology, or return to medical research.