Malignant pleural mesothelioma in a child with ataxia-telangiectasia

Our plain-English summary of Malignant pleural mesothelioma in a child with ataxia-telangiectasia, by C. Rosas-Salazar, S.W. Gunawardena, J.E. Spahr (Pediatric Pulmonology, 2013).

The study

• Authors: C. Rosas-Salazar, S.W. Gunawardena, J.E. Spahr
• Journal: Pediatric Pulmonology
• Published: 2013;48(1):94-7
• Type of article: Case report
• Cited by: 7 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Ataxia-telangiectasia (AT) is a hereditary disorder characterized by progressive neurological dysfunction, oculocutaneous telangiectasia, immunodeficiency, cancer susceptibility, and radiation sensitivity. Pleural neoplasms are extremely rare in the pediatric population, even in patients with AT. We describe the case of a 16-year-old male with AT who developed a malignant pleural mesothelioma (MPM). Benign or infectious lung and pleural diseases are common in those with AT. Hence, delayed diagnosis of respiratory neoplasms can occur in these patients. This report highlights the need of heightened vigilance in patients with AT with recurrent or persistent pleuropulmonary disease. To our knowledge, no other cases of MPM in children with AT have been reported.

Copyright © 2012 Wiley Periodicals, Inc.

In plain English

This article was a case report about one 16-year-old male with ataxia-telangiectasia (AT), an inherited disorder, who developed malignant pleural mesothelioma (MPM), a cancer of the lining around the lungs. Over several months he had cough, a right-sided pneumonia, a collapsed lung that cleared on its own, and fluid around both lungs (bilateral pleural effusions). His findings were first thought to come from chronic aspiration linked to swallowing problems. A chest CT later showed a right pleural mass that had invaded the sac around the heart (the pericardium). A biopsy was consistent with tubulopapillary MPM, and three pathologists separately agreed with the diagnosis. The article reported no history of radiation, asbestos, or prenatal isoniazid exposure. He received five courses of chemotherapy with cisplatin and pemetrexed and died about 8 months after diagnosis. The authors stated that, to their knowledge, no other cases of MPM in children with AT had been described, and only one adult case had been reported. The authors concluded that MPM should be included among the possible diagnoses in patients with AT who do not respond to conventional therapy.

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 16-year-old male with ataxia-telangiectasia (AT) and recurrent lung and pleural disease. The authors are based at Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.
• Exposure: No known asbestos exposure. The article reports no history of radiation, asbestos, or prenatal isoniazid exposure.
• Main results:
  • The patient's early course included a right-sided pneumonia treated with oral antibiotics.
  • A chest radiograph during the early course showed a pneumothorax (collapsed lung) on the same side, which resolved on its own.
  • At presentation to the pulmonology clinic, a chest radiograph showed bilateral pleural effusions.
  • The patient's findings were initially thought to be related to chronic aspiration with dysfunctional swallowing.
  • Chest CT showed a pleural mass in the right chest with invasion of the pericardium.
  • Biopsy pathology was consistent with tubulopapillary malignant pleural mesothelioma.
  • Three pathologists separately evaluated the biopsy and agreed with the diagnosis.
  • The patient received five courses of chemotherapy with cisplatin and pemetrexed.
  • The patient died approximately 8 months after diagnosis.
  • To the authors' knowledge, no other cases of MPM in children with AT had been described.
  • Only one adult case of MPM with AT had been reported.
• The authors' conclusion: The authors concluded that, although benign lung and pleural diseases are common in patients with AT, MPM should be included in the differential diagnosis of patients not responding to conventional therapy.

Topics

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

Medical terms in this study, explained in our Lexicon: Pleural Mesothelioma, Neoplasm.

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