Spectrum of peritoneal mesothelioma in childhood: clinical and histopathologic features, including DNA cytometry

Our plain-English summary of Spectrum of peritoneal mesothelioma in childhood: clinical and histopathologic features, including DNA cytometry, by F.K. Niggli, T.J. Gray, F. Raafat, et al (Pediatric Hematology and Oncology, 1994).

The study

• Authors: F.K. Niggli, T.J. Gray, F. Raafat, M.C. Stevens
• Journal: Pediatric Hematology and Oncology
• Published: 1994;11(4):399-408
• Type of article: Case report
• Cited by: 16 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Different types of peritoneal mesothelioma (PM) occur in children and adults. All these share certain histopathologic features but differ in other aspects, such as age of occurrence, site and sex predominance, etiology, and biologic behavior. The article describes four patients, two with cystic PM (one of whom had multiple recurrences) and two with malignant PM (one of whom had pleural metastases). These cases illustrate the variable behavior of this tumor in childhood and highlight the difficulties encountered in diagnosis and treatment. Three different groups of mesothelioma are recognized: a classic, asbestos-related, malignant mesothelioma of adults, typically occurring in the pleural cavity; a multicystic mesothelioma, predominantly affecting the pelvic peritoneum of young women and associated with a good prognosis; and mesotheliomas in children, which are not associated with asbestos exposure and have an unpredictable biologic behavior requiring individual treatment strategies. In the patients studied, DNA index measured by flow cytometry showed a difference between the cystic (aneuploid) and malignant (diploid) tumors. The proliferative rate (S phase) of the tumor was low in all four cases.

In plain English

This article described four children with peritoneal mesothelioma (a tumor of the abdominal lining) treated at a children's hospital in Birmingham, England. A girl diagnosed with malignant disease at 13 died of bowel complications 7 years later, and autopsy found no tumor. A girl diagnosed with malignant disease at 4, whose chest scan was consistent with pleural tumor deposits, remained well without progressive disease 1.5 years later. A boy diagnosed with cystic mesothelioma at 15 was alive and well at 21 but not disease free. A girl diagnosed with cystic mesothelioma at 20 months had only a biopsy and was well 4 years later. DNA content was diploid (normal) in both malignant tumors and hyperdiploid (raised) in both cystic ones. S phase (the share of cells copying DNA) was low in all four, ranging from 5.0% to 7.5%. The article's stated median of 6.4% does not fit these values. The authors said the data were insufficient to tell whether DNA content separates benign from malignant forms. They concluded that these children need individual treatment and that aggressive therapy should be avoided. The text gives no asbestos exposure history for any of the children.

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 series (four case reports) with flow cytometry DNA studies
• Who was studied: Four children with peritoneal mesothelioma treated at the Children's Hospital, Birmingham, England: three girls (aged 13 years, 20 months and 4 years at diagnosis) and one boy (aged 15 years at diagnosis)
• Exposure: Not reported. The article gives no individual asbestos or other exposure histories for these four children. It states only, in general terms, that childhood mesothelioma is rarely associated with asbestos exposure.
• Main results:
  • Case 1 (malignant peritoneal mesothelioma, girl aged 13 at diagnosis) died of bowel complications 7 years after diagnosis. Autopsy revealed no tumor.
  • Case 2 (a boy, aged 15 at diagnosis) was diagnosed with cystic mesothelioma and was alive and well at 21 years of age but not disease free
  • Case 3 (cystic mesothelioma, girl aged 20 months at diagnosis) had no treatment beyond laparoscopy and biopsy and was well 4 years later
  • Case 4 (malignant mesothelioma, girl aged 4 at diagnosis) had a chest CT consistent with pleural tumor deposits. After chemotherapy with doxorubicin and cisplatin, she remained well without evidence of progressive disease 1.5 years after diagnosis.
  • The two malignant tumors (cases 1 and 4) were diploid (DNA index 1.0), with S phase of 7.5% and 5.3%
  • The two benign variants (cases 2 and 3) were hyperdiploid, with DNA index 1.19 (S phase 5.4%) and 1.62 (S phase 5%)
  • The authors described the proliferative rate (S phase) as low in all four cases and gave a median of 6.4%. That figure does not match the four values the article reports (7.5%, 5.3%, 5.4%, 5.0%). The median of those four values, worked out from them here and not stated in the article, is 5.35%.
• The authors' conclusion: The authors concluded that these patients require individual treatment strategies but that an aggressive therapeutic approach should be avoided
• Limitations the authors state:
  • The authors stated there are insufficient data to determine whether DNA index can help distinguish benign from malignant peritoneal mesothelioma

Topics

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

Medical terms in this study, explained in our Lexicon: Peritoneal Mesothelioma, Etiology, Mesothelioma, Pleural Cavity, Multicystic Mesothelioma (MCM), Peritoneum, Prognosis, Asbestos Exposure.

Related studies

• DNA analysis by flow cytometry in malignant pleural mesothelioma: relationship to histology and survival — H. Dazzi, N. Thatcher, et al. (1990)
• Mesothelioma of childhood — A.E. Fraire, S. Cooper, et al. (1988)
• Preoperative Circulating Tumor DNA in Patients with Peritoneal Carcinomatosis is an Independent Predictor of Progression-Free Survival — J.M. Baumgartner, V.M. Raymond, et al. (2018)
• Characterization of DNA hypermethylation in two cases of peritoneal mesothelioma — R. Hama, Y. Watanabe, et al. (2012)
• Primary malignant tumors of peritoneal and retroperitoneal origin: clinical and imaging features — M.G. Lubner, J.L. Hinshaw, et al. (2014)
• Rare thoracic cancers, including peritoneum mesothelioma — S. Siesling, J.M. van der Zwan, et al. (2012)

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