Nodular glomeruloid pleuroblastoma: a biphasic pleural-based malignant tumor with immature elements

Our plain-English summary of Nodular glomeruloid pleuroblastoma: a biphasic pleural-based malignant tumor with immature elements, by P. Mukonoweshuro, R.L. Attanoos, M.E. Smith (Virchows Archiv, 2006).

The study

• Authors: P. Mukonoweshuro, R.L. Attanoos, M.E. Smith
• Journal: Virchows Archiv
• Published: 2006;449(2):253-7
• Type of article: Case report
• Cited by: 2 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

An unusual diffuse pleural-based tumor arising in an elderly asbestos-exposed male is presented. The tumor presented in a 72-year-old male with a 30-year history of dockyard work and likely significant asbestos exposure. Macroscopically, at post mortem, the pleural tumor diffusely encased the right lung and was composed of an admixture of neoplastic macro-, and by light microscopy, micro-nodules. Histologically, the tumor had a biphasic growth pattern with glomeruloid epithelioid elements and immature blastematous mesenchymal stroma. Immunophenotypically, the tumor had a complex pattern with epithelioid elements expressing cytokeratins, desmin, carcinoembryonic antigen (CEA), Ber EP4 and E-cadherin. The diagnostic problems and medicolegal issues surrounding the diagnosis and differentiation from malignant pleural mesothelioma and other tumors are discussed.

In plain English

This case report described a single patient: a 72-year-old male electrical fitter whose 30 years of dockyard work, the authors wrote, was likely associated with significant occupational asbestos exposure. The article did not name a fiber type. The man presented with breathlessness and a right-sided pleural effusion. Early biopsy and fluid tests found no cancer, but a later chest-wall mass proved to be a malignant biphasic tumor not typical of malignant mesothelioma. Chemotherapy and radiotherapy had limited effect, and he died at 73, 15 months after first presenting. At post mortem, a multi-nodular tumor encased the right lung, and a left pleural plaque was found. No asbestos bodies or pulmonary fibrosis were found in either lung. Under the microscope the tumor showed glomeruloid epithelial structures and immature blastematous stroma. It did not stain for calretinin, and the authors stated that its staining pattern excludes malignant mesothelioma. The authors proposed it as a distinct tumor, which they called nodular glomeruloid pleuroblastoma. They said that calling it asbestos-related would be premature without many further cases.

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 of a single patient, with post mortem, histology and immunohistochemistry. The authors described it as, to their knowledge, the first published report of this tumor type.
• Who was studied: One 72-year-old male electrical fitter with a 30-year history of dockyard work. He presented with breathlessness and a right-sided pleural effusion with nodular pleural thickening. The authors' departments were in Plymouth and Wales, UK; the patient's location is not otherwise stated.
• Exposure: Dockyard work that the authors considered likely to have involved significant occupational asbestos exposure. No fiber type, products or dose are given.
• Main results:
  • In the patient, pleural needle core biopsy and cytology of the effusion did not show malignancy. A later chest-wall mass biopsy showed a malignant biphasic tumor not typical of malignant mesothelioma.
  • Chemotherapy and palliative radiotherapy had limited response. The patient died of the effects of the pleural tumor at age 73, 15 months after initial clinical presentation.
  • At post mortem there was a right-sided multi-nodular pleural tumor; the largest nodule was approximately 60 mm. The right lung with tumor weighed 3,610 g, and the left lung (590 g) showed no tumor.
  • A left pleural plaque was identified at post mortem.
  • No systemic metastases or other primary tumors were found.
  • Asbestos bodies and pulmonary fibrosis were not found in either lung.
  • On microscopy the tumor was biphasic and multi-nodular. Microscopic nodules ranged from 1 to 14 mm, with glomeruloid epithelial structures and blastematous spindle cell stroma.
  • The epithelial component stained strongly for cytokeratins (MNF116) and desmin. It stained focally for cytokeratin 5/6, E-cadherin, Ber-EP4 and CEA. The spindle cell component showed focal WT-1.
  • Calretinin, EMA, p53, CD15, CD31, myogenin and S100 were not detected in either component. The authors stated that this immunophenotype excludes malignant mesothelioma.
• The authors' conclusion: The authors suggested that this is a distinct tumor that pathologists need to be aware of. In asbestos-exposed people, they said, distinguishing it from malignant mesothelioma is important. They considered conclusions that the tumor is asbestos-related premature and said further validation is necessary.
• Limitations the authors state:
  • The authors stated that the occupational history of many further cases will be needed before the tumor's relationship to asbestos exposure can be clarified.

Topics

Medical terms in this study, explained in our Lexicon: Biphasic Mesothelioma, Asbestos Exposure, Epithelioid, Desmin, Carcinoembryonic Antigen (CEA), Pleural Mesothelioma.

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