Malignant Peritoneal Mesothelioma in the setting of a Ventriculo-Peritoneal Shunt: A novel clinical presentation

Our plain-English summary of Malignant Peritoneal Mesothelioma in the setting of a Ventriculo-Peritoneal Shunt: A novel clinical presentation, by M. Haque, N. Hameed, C.T. Perry, et al (Clinical Medicine Insights. Oncology, 2018).

The study

• Authors: M. Haque, N. Hameed, C.T. Perry, E. Carter, W.S. Mneimneh
• Journal: Clinical Medicine Insights. Oncology
• Published: 2018;12:1179554918779583
• Type of article: Case report
• Cited by: 2 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

We report a case of malignant peritoneal mesothelioma (MPM) in a 31-year-old male with history of cerebral palsy, hydrocephalus, and ventriculoperitoneal shunt (VPS) placed since infancy. He presented with fever, abdominal pain and distension. Computed tomography scan revealed a thick-walled rim-enhancing fluid collection, interpreted as pseudocyst. Intraoperatively, diffuse nodular peritoneal thickening with adhesions was demonstrated. The resection specimen consisted of multiple membranous fragments displaying firm nodules. Microscopic examination revealed a tumefactive malignant-appearing epithelioid proliferation involving the peritoneum, focally invading the underlying fat. Immunohistochemically, the tumor cells expressed keratin AE1/AE3, CK7, CK5/6, Calretinin, WT1 and D2-40, and were negative for CEA and MOC31. The findings were consistent with MPM, epithelioid type. The patient's condition continued to decline with increasing abdominal distension during the month following the original diagnosis. While atypical mesothelial hyperplasia has been described in association with long standing VPS, well-documented cases of MPM have not been previously reported in such context.

In plain English

This case report described a 31-year-old Caucasian male with cerebral palsy, spina bifida, seizure disorder and hydrocephalus who had had a ventriculoperitoneal shunt since infancy. The patient had fever, abdominal pain and swelling. A CT scan showed a large fluid collection inside the abdomen with the tip of the shunt's catheter embedded in it, which was first read as an infected 'pseudocyst'. At surgery the abdominal lining was thickened with nodules, and only part of the tissue could be removed. Microscope and staining tests showed malignant peritoneal mesothelioma, epithelioid type. No environmental or domestic asbestos exposure was identified, and there was no previous radiation therapy. The patient declined over the following month and was discharged to hospice care without further aggressive therapy. The authors reported this as, to their knowledge, the first well-documented case of this cancer developing with a long-standing shunt and no exposure history. They called chronic irritation from the shunt a conceivable hypothesis, but stated that the cause was not clear. Genetic testing was not performed.

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 (one patient)
• Who was studied: One 31-year-old Caucasian male with cerebral palsy, spina bifida, seizure disorder and hydrocephalus, with a ventriculoperitoneal shunt placed since infancy. The article does not state where or when the patient was treated.
• Exposure: A long-standing ventriculoperitoneal shunt. No history of asbestos exposure (environmental or domestic) or radiation therapy was identified.
• Main results:
  • A CT scan showed a large intra-abdominal fluid collection with the shunt catheter tip embedded in it. It was interpreted as an infected peritoneal pseudocyst.
  • At surgery there was nodular thickening of the peritoneum with adhesions and pseudocyst formation.
  • Only part of the diseased tissue could be removed surgically.
  • The fluid culture grew Pseudomonas stutzeri.
  • Under the microscope there were frequent mitotic figures (dividing cells), without necrosis. The appearance was consistent with a malignant-appearing epithelioid tumor.
  • The tumor cells stained strongly for keratin markers, including pankeratin AE1/AE3 and CK7. They were positive for mesothelial markers (calretinin, WT-1, D2-40) and negative for MOC-31 and monoclonal CEA, among other markers.
  • The diagnosis was malignant peritoneal mesothelioma, epithelioid type.
  • The patient's condition declined with increasing abdominal distension during the month after diagnosis.
  • The patient was discharged to hospice care without further aggressive therapy.
• The authors' conclusion: The authors stated that, to their knowledge, this was the first reported well-documented case of malignant peritoneal mesothelioma developing in association with a long-standing ventriculoperitoneal shunt in the absence of exposure history. They proposed chronic irritation and inflammation from the shunt, with progression from mesothelial hyperplasia to mesothelioma, as a conceivable hypothesis.
• Limitations the authors state:
  • The authors stated that the underlying cause of the mesothelioma in this case was not clear.
  • Mutational or germline genetic testing was not performed.

Topics

Medical terms in this study, explained in our Lexicon: Peritoneal Mesothelioma, Epithelioid, Peritoneum, Calretinin, Podoplanin (D2-40), Atypical Mesothelial Proliferation, Standing.

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