Malignant Peritoneal Mesothelioma Arising in Young Adults with Long-standing Indwelling Intra-abdominal Shunt Catheters

Tala Mujahed, Henry D. Tazelaar, William R. Sukov, Kevin C. Halling, Jaime I. Davila, Carolyn Glass, Elizabeth N. Pavlisko, Kyle C. Strickland, Victor Roggli, Monira Haque, Wadad Mneimneh, Elliot Carter, Francoise Galateau-Salle, David Glidden, Richard Garcia-Kennedy, Brandon T. Larsen

Research output: Contribution to journalArticlepeer-review

Abstract

Only 50% to 70% of patients with mesothelioma report asbestos exposure. Other exposures (eg, radiation) play a role in some cases, but some patients have no obvious cause. We describe a series of patients with long-standing indwelling intra-abdominal shunt catheters who developed malignant peritoneal mesothelioma, suggesting a novel association. We identified 7 patients who had shunts and subsequently developed mesothelioma (5 women; median age: 31 y, range: 18 to 45 y). Clinical history and pathology materials were reviewed, and RNA sequencing was performed. Clinical presentations varied; 6 patients had hydrocephalus and a ventriculoperitoneal shunt, and 1 patient had portal hypertension and a portoatrial shunt. The median duration of shunt therapy in 5 cases was 29 years (range: 12 to 35 y); the remaining 2 patients also had shunts for many years, but specific details were unavailable. Two patients had radiotherapy for malignancies in childhood. One had an alleged exposure to asbestos and 1 had prior exposure to talc. The rest had no known risk factors. Histologically, all tumors were purely epithelioid. Treatments included surgical debulking, chemotherapy, and palliative care. All 7 died of disease (median survival: 7 mo, range: 1 to 18 mo). Molecular testing showed loss of NF2 and CDKN2A/B and a BAP1 mutation in 1 case, and no genomic alterations associated with mesothelioma in 2 cases. Peritoneal mesothelioma may represent a complication of long-standing indwelling shunt catheters. The mechanism is unknown, but chronic peritoneal irritation may play a role. Albeit rare, mesothelioma should be considered in patients with a shunt who present with new ascites.

Original languageEnglish (US)
Pages (from-to)255-262
Number of pages8
JournalAmerican Journal of Surgical Pathology
Volume45
Issue number2
DOIs
StatePublished - Feb 2021

Keywords

  • asbestos
  • hydrocephalus
  • mesothelioma
  • ventriculoperitoneal shunt

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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