Pediatric Peritoneal Epithelial Malignant Mesothelioma Case Report

Our plain-English summary of Pediatric Peritoneal Epithelial Malignant Mesothelioma Case Report, by E. Bellew, S. Lee, Hiren Patel, et al (Case Reports in Radiology, 2021).

The study

• Authors: E. Bellew, S. Lee, Hiren Patel, C.F. Levy, R. Goldfisher, J. Amodio
• Journal: Case Reports in Radiology
• Published: 2021;2021:5581757
• Type of article: Case report
• Cited by: 3 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

We present a 14-year-old boy with peritoneal epithelial malignant mesothelioma (PEMM). While pathology is required to make this diagnosis, radiology plays a crucial role throughout the clinical course of this disease. The key imaging characteristics of peritoneal mesothelioma have been previously well-described in the adult population, but there are rare reports in the pediatric population. This pediatric report highlights the multidimensional use of imaging in this disease, from the initial evaluation to therapeutic supplementation and subsequent follow-up.

Copyright © 2021 Elizabeth Bellew et al.

In plain English

This case report described one 14-year-old boy with peritoneal epithelial malignant mesothelioma, focusing on how imaging was used. He had chronic abdominal pain and had lost over 20 pounds in 9 months. MR enterography showed a thickened omentum and peritoneum, a moderate to large volume of ascites (fluid in the abdomen) and nodules along the diaphragm. A needle biopsy was too small to give a diagnosis. Laparoscopic biopsies then confirmed mesothelioma, and molecular testing found a CDKN2A deletion. He received 5 cycles of chemotherapy (cisplatin, pemetrexed and Avastin). He then had debulking surgery with heated cisplatin chemotherapy inside the abdomen, a second such heated treatment, and 4 cycles of intraperitoneal chemotherapy. Scans showed the disease shrinking. When a later MRI raised concern for relapse, PET and fluid testing pointed instead to changes caused by surgery and treatment. The authors reported no evidence of disease after one year of therapy. The article did not describe any asbestos exposure for this patient. The authors noted that one case may not be generalizable to others.

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 (single patient), focused on the role of imaging
• Who was studied: One 14-year-old boy with a history of growth hormone deficiency, gastroesophageal reflux disease and asthma. The authors are from Northwell Health, New Hyde Park, NY, USA. The article does not give dates of care.
• Exposure: No asbestos, radiation or other exposure history is reported for this patient
• Main results:
  • The patient presented with chronic abdominal pain and over 20-pound weight loss in a 9-month period
  • Inflammatory markers were elevated at diagnosis: CRP 14.6mg/mL (normal less than 4 mg/mL) and ESR 47 (normal less than 15)
  • MR enterography showed a thickened omentum, diffuse smooth peritoneal enhancement and thickening, and a moderate to large volume of ascites. No mass was seen. Enhancing nodules were present along the diaphragm.
  • The ultrasound-guided omental core biopsy and fluid cytology were not diagnostic because the sample was too small
  • CA-125 was elevated at 171 U/mL (normal less than 38 U/mL). Other tumor markers were normal.
  • Laparoscopic biopsy showed neoplastic mesothelial cells. Stains for calretinin, CK 5/6 and WT-1 were positive, confirming mesothelioma.
  • Molecular testing of the tumor showed CDKN2A homozygous and heterozygous deletion
  • The patient had no germline mutations, including BAP1
  • After 5 cycles of IV chemotherapy (cisplatin, pemetrexed and Avastin), MRI showed decreased peritoneal thickening, resolution of ascites and smaller diaphragmatic nodules. PET/CT had shown decreased FDG avidity after the third cycle.
  • The patient had debulking surgery with HIPEC with cisplatin, then a second HIPEC with cisplatin. No active disease was found at the second HIPEC. He then had 4 cycles of intraperitoneal cisplatin alternating with mitomycin.
  • After intraperitoneal chemotherapy, MRI showed new peritoneal nodularity and large volume ascites, but PET showed no avidity. Paracentesis found no malignant cells, and the changes were judged to be postsurgical and treatment-related.
  • There was no evidence of disease after one year of therapy. Follow-up is MRI every 4 months.
• The authors' conclusion: The authors concluded that peritoneal mesothelioma should be considered when the imaging findings shown in this case are present, and that an interdisciplinary approach is essential for diagnosis and treatment
• Limitations the authors state:
  • This is the report of one individual's disease and treatment, which may vary from patient to patient and may not be generalizable to a cohort
  • Imaging findings cannot be used alone. Pathology and molecular testing are needed for final diagnosis.
  • Next-generation sequencing failed due to inadequate specimen, so tumor mutational burden could not be assessed

Topics

Medical terms in this study, explained in our Lexicon: Mesothelioma, Pathology, Peritoneal Mesothelioma.

Related studies

• Malignant peritoneal mesothelioma in two pediatric patients: MR imaging findings — M. Haliloglu, F.A. Hoffer, et al. (2000)
• STRN-ALK rearranged pediatric malignant peritoneal mesothelioma - Functional testing of 527 cancer drugs in patient-derived cancer cells — A. Murumägi, D. Ungureanu, et al. (2021)
• Treatment of peritoneal mesothelioma in pediatric patients — L.A. Cioffredi, P.A. Jänne, et al. (2009)
• Pediatric Benign Cystic Peritoneal Mesothelioma — J. Durell, H. Dagash, et al. (2016)
• Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) with cisplatin in pediatric patients with peritoneal mesothelioma: a single institution experience and long term follow up — P. Malekzadeh, M. Good, et al. (2021)
• A Review of the Use of Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Malignancy in Pediatric Patients — D.J. Byrwa, C.J. Twist, et al. (2023)

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