Malignant transformation of well-differentiated papillary mesothelioma 13 years after the diagnosis: a case report

Our plain-English summary of Malignant transformation of well-differentiated papillary mesothelioma 13 years after the diagnosis: a case report, by L. Costanzo, S. Scarlata, G. Perrone, et al (The Clinical Respiratory Journal, 2014).

The study

• Authors: L. Costanzo, S. Scarlata, G. Perrone, L. Rossi, A. Papa, F.M. Di Matteo, G. Tonini, A. Onetti Muda, R. Antonelli-Incalzi, S. Tomao
• Journal: The Clinical Respiratory Journal
• Published: 2014;8(1):124-9
• Type of article: Case report
• Cited by: 13 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Well-differentiated papillary mesothelioma (WDPM) is a rare mesothelial tumour affecting mostly the peritoneum of women in their reproductive age, but it may occur also at other sites, including the pleura. It is considered a specific pathological entity different from diffuse malignant mesothelioma as it displays a characteristic histological pattern and is associated with a slowly progressive clinical course. We report the case of a 79-year-old man with a history of right pleural WDPM at age 64, which was successfully treated with chemotherapy, radiotherapy and talc pleurodesis. Thirteen years after the first diagnosis, he presented with mediastinal lymph nodes metastasis and with an extremely rare pattern of tracheal and bronchial infiltration that was detected at bronchoscopy. Biopsy samples revealed loss of histological differentiation of the neoplastic cells. This case report highlights that WDPM is a tumour of uncertain malignant potential that may undergo transformation over many years. Finally, the role of immunohistochemistry in the diagnosis of WDPM and the possible mechanisms leading to this unique way of metastatisation are discussed.

© 2013 John Wiley & Sons Ltd.

In plain English

This case report described one man with a history of occupational asbestos exposure. The article does not name a fiber type or job. In 1997 a screening chest X-ray found thickening of the right pleura, and a needle biopsy showed well-differentiated papillary mesothelioma (WDPM), a rare mesothelial tumour. He received chemotherapy, which was stopped because of blood-related toxicity, then radiotherapy and talc pleurodesis. The disease did not progress during follow-up. In June 2011, aged 79, he had dry cough, loss of appetite and about 7 kg of weight loss over 8 months. Scans showed right pleural nodules, pleural fluid and enlarged chest lymph nodes. Bronchoscopy found whitish nodules in the airway lining. Biopsies showed cancer cells consistent with malignant mesothelioma that had spread to the trachea, bronchi and mediastinal lymph nodes. The authors concluded that WDPM is a tumour of uncertain malignant potential that may transform many years after diagnosis. They suggested, as a hypothesis, that talc pleurodesis may have influenced how the tumour spread.

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)
• Who was studied: One 79-year-old Caucasian man treated in Italy, first diagnosed with right pleural WDPM in 1997 and admitted to the authors' hospital in June 2011
• Exposure: History of occupational asbestos exposure; fiber type, job and dose not reported
• Main results:
  • In 1997 a chest X-ray done in an occupational safety and health screening program showed right pleural thickening; WDPM was diagnosed by CT-guided core needle biopsy
  • First treatment was chemotherapy, stopped for haematological toxicity, followed by radiotherapy and talc pleurodesis; the patient showed no progression on follow-up and was fairly free of symptoms until October 2010
  • At admission in June 2011 he reported dry cough, anorexia and weight loss of about 7 kg over the past 8 months
  • CT showed diffuse right pleural nodularity, pleural effusions and multiple enlarged mediastinal lymph nodes, including a large subcarinal node; no lesion was found outside the chest
  • At bronchoscopy, the lining of the carina and both bronchi was red and swollen and studded with whitish nodules
  • Biopsies of the carina and left bronchus, and needle aspiration of the subcarinal lymph node, showed malignant cells staining for calretinin and WT1, consistent with malignant mesothelioma spreading to the trachea, bronchi and mediastinal lymph nodes
  • On re-examination, the 1997 specimen showed features of WDPM (no mitoses, no cellular atypias, no stromal invasion). The authors said these findings, together with the slow clinical course, support the original diagnosis
  • The authors decided not to perform genetic analysis in this case
  • The patient was given first-line chemotherapy with pemetrexed and carboplatin and was still under oncology care
  • The authors stated that the few previously reported transformations concerned peritoneal tumours, and that none described such a long interval (13 years) before malignant transformation
• The authors' conclusion: The authors concluded that WDPM is a tumour of uncertain malignant potential that may undergo dedifferentiation, transformation and spread even many years after first diagnosis. They hypothesised that talc pleurodesis may have encouraged spread through subpleural vessels and lymphatics to the mediastinal lymph nodes and then into the airway walls. They said the case shows how treatments such as pleurodesis could influence the spread of the tumour.

Topics

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

Medical terms in this study, explained in our Lexicon: Malignant Transformation, Well-Differentiated Papillary Mesothelioma, Peritoneum, Pleura, Malignant Mesothelioma, Chemotherapy, Talc Pleurodesis, Mediastinal Lymph Nodes.

Related studies

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• Well-Differentiated Papillary Mesothelioma With Two Synchronous Serous Gynaecologic Carcinomas in a 62-Year-Old Woman: Lessons Learned for the Gynaecologic Surgeon — J.M. McGinnis, V. Bloomfield, et al. (2020)
• An incidental diagnosis of well-differentiated papillary mesothelioma in a woman operated on for recurrent endometriosis — R. Mangal, O. Taskin, et al. (1995)
• Well-differentiated papillary mesothelioma with invasive foci — A. Churg, T. Allen, et al. (2014)
• Well-differentiated papillary mesothelioma of the female peritoneum: a clinicopathologic study of 26 cases — A. Malpica, S. Sant'Ambrogio, et al. (2012)
• BAP1 loss is unusual in well-differentiated papillary mesothelioma and may predict development of malignant mesothelioma — H.E. Lee, J.R. Molina, et al. (2018)

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