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

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.

Progression of Peritoneal Mesothelioma In Situ to Invasive Mesothelioma Arising in the Setting of Endometriosis With Germline BAP1 Mutation: A Case Report

“…Risk factors included asbestos exposure (4/16, 25%) and personal (5/15, 33%) and/or family (10/12, 83%) history of malignancy; however, genetic testing data were not available. Malignant peritoneal mesothelioma has been rarely described in other chronic inflammatory diseases involving the peritoneum including Crohn disease, familial Mediterranean fever-associated recurrent peritonitis, recurrent peritonitis secondary to diverticulitis and indwelling peritoneal catheters (13)(14)(15). Additional research is needed, but together these data suggest a possible role for chronic inflammatory peritoneal diseases such as endometriosis in promoting tumor progression in peritoneal mesothelioma.…”

Mesothelioma in the pleura, pericardium and peritoneum: Recommendations from the International Collaboration on Cancer Reporting (ICCR)

“…In line with the ever-expanding literature of clinically relevant molecular changes, the section on ancillary data and recommendation has been expanded, and will probably require expansion into the future. [91][92][93][94][95][96] Possibly most significantly, in-situ mesothelioma is now an accepted diagnostic category and is timely; as more data are collected on the clinical significance of this diagnosis, this will inform any updates of future UICC/AJCC staging manuals and, eventually, clinical trials.…”

Biphasic Peritoneal Mesothelioma: A Lethal Clinical Entity

“…The combination of CRS and HIPEC is found to extend survival to an average of 36 months [3,6,7,9,11]. Other treatments include a variety of chemotherapy and platinum agents and immunotherapy [1][2][3][4][5]8,[13][14][15]. At the time of our patient's radical resection, the diagnosis was unclear.…”