Progression of Well-Differentiated Papillary Mesothelial Tumour to Mesothelioma in a Patient with Ehlers Danlos Syndrome
Well-differentiated papillary mesothelioma has been renamed well-differentiated papillary mesothelial tumour (WDPMT) in the current WHO classification because all mesotheliomas are now regarded as malignant. WDPMT is now defined as a non-invasive papillary mesothelial proliferation, with retained la...
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| Format: | Article |
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MDPI AG
2021-10-01
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| Series: | Journal of Molecular Pathology |
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| Online Access: | https://www.mdpi.com/2673-5261/2/4/26 |
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| author | Sarita Prabhakaran Matthew Hussey Kenneth J. O’Byrne Sonja Klebe |
| author_facet | Sarita Prabhakaran Matthew Hussey Kenneth J. O’Byrne Sonja Klebe |
| author_sort | Sarita Prabhakaran |
| collection | DOAJ |
| description | Well-differentiated papillary mesothelioma has been renamed well-differentiated papillary mesothelial tumour (WDPMT) in the current WHO classification because all mesotheliomas are now regarded as malignant. WDPMT is now defined as a non-invasive papillary mesothelial proliferation, with retained labelling for BAP1-desirable. The current WHO classification also includes mesothelioma in situ (MIS), which is defined as pre-invasive flat or papillary proliferation of mesothelial cells with a loss of BAP1 or MTAP. WDPMT has been variably defined in the past but was thought to occur more commonly in women and pursue a more indolent course than mesothelioma, but its progression to invasive disease has occasionally been reported. Here, we report a case of a 68-year-old woman with a history of asbestos exposure and an underlying diagnosis of Ehlers Danlos syndrome who was diagnosed with symptomatic WDPMT of the peritoneum that progressed to mesothelioma within two years. On retrospective analysis, the WDPMT showed a loss of BAP1. We suggest that a loss of BAP1 in WDPMT should be reported, since these lesions may show aggressive behaviour, and that they may best be regarded as similar to mesothelioma in situ. |
| first_indexed | 2024-03-10T03:48:45Z |
| format | Article |
| id | doaj.art-63abfb6b47dc4a51b6d7c2072fc68204 |
| institution | Directory Open Access Journal |
| issn | 2673-5261 |
| language | English |
| last_indexed | 2024-03-10T03:48:45Z |
| publishDate | 2021-10-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Molecular Pathology |
| spelling | doaj.art-63abfb6b47dc4a51b6d7c2072fc682042023-11-23T09:01:55ZengMDPI AGJournal of Molecular Pathology2673-52612021-10-012430631110.3390/jmp2040026Progression of Well-Differentiated Papillary Mesothelial Tumour to Mesothelioma in a Patient with Ehlers Danlos SyndromeSarita Prabhakaran0Matthew Hussey1Kenneth J. O’Byrne2Sonja Klebe3Department of Anatomical Pathology, College of Medicine and Public Health, Flinders University, Adelaide, SA 5042, AustraliaDepartment of Surgical Pathology, SA Pathology at Flinders Medical Centre, Adelaide, SA 5042, AustraliaCancer Services, Princess Alexandra Hospital, Brisbane, QLD 4102, AustraliaDepartment of Anatomical Pathology, College of Medicine and Public Health, Flinders University, Adelaide, SA 5042, AustraliaWell-differentiated papillary mesothelioma has been renamed well-differentiated papillary mesothelial tumour (WDPMT) in the current WHO classification because all mesotheliomas are now regarded as malignant. WDPMT is now defined as a non-invasive papillary mesothelial proliferation, with retained labelling for BAP1-desirable. The current WHO classification also includes mesothelioma in situ (MIS), which is defined as pre-invasive flat or papillary proliferation of mesothelial cells with a loss of BAP1 or MTAP. WDPMT has been variably defined in the past but was thought to occur more commonly in women and pursue a more indolent course than mesothelioma, but its progression to invasive disease has occasionally been reported. Here, we report a case of a 68-year-old woman with a history of asbestos exposure and an underlying diagnosis of Ehlers Danlos syndrome who was diagnosed with symptomatic WDPMT of the peritoneum that progressed to mesothelioma within two years. On retrospective analysis, the WDPMT showed a loss of BAP1. We suggest that a loss of BAP1 in WDPMT should be reported, since these lesions may show aggressive behaviour, and that they may best be regarded as similar to mesothelioma in situ.https://www.mdpi.com/2673-5261/2/4/26well-differentiated papillary mesothelial tumourwomenperitoneal mesotheliomaasbestos exposure |
| spellingShingle | Sarita Prabhakaran Matthew Hussey Kenneth J. O’Byrne Sonja Klebe Progression of Well-Differentiated Papillary Mesothelial Tumour to Mesothelioma in a Patient with Ehlers Danlos Syndrome Journal of Molecular Pathology well-differentiated papillary mesothelial tumour women peritoneal mesothelioma asbestos exposure |
| title | Progression of Well-Differentiated Papillary Mesothelial Tumour to Mesothelioma in a Patient with Ehlers Danlos Syndrome |
| title_full | Progression of Well-Differentiated Papillary Mesothelial Tumour to Mesothelioma in a Patient with Ehlers Danlos Syndrome |
| title_fullStr | Progression of Well-Differentiated Papillary Mesothelial Tumour to Mesothelioma in a Patient with Ehlers Danlos Syndrome |
| title_full_unstemmed | Progression of Well-Differentiated Papillary Mesothelial Tumour to Mesothelioma in a Patient with Ehlers Danlos Syndrome |
| title_short | Progression of Well-Differentiated Papillary Mesothelial Tumour to Mesothelioma in a Patient with Ehlers Danlos Syndrome |
| title_sort | progression of well differentiated papillary mesothelial tumour to mesothelioma in a patient with ehlers danlos syndrome |
| topic | well-differentiated papillary mesothelial tumour women peritoneal mesothelioma asbestos exposure |
| url | https://www.mdpi.com/2673-5261/2/4/26 |
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