Epithelioid angiosarcoma of the pleura: a case report

Our plain-English summary of Epithelioid angiosarcoma of the pleura: a case report, by M.S. Roh, J.Y. Seo, S.H. Hong (Journal of Korean Medical Science, 2001).

The study

• Authors: M.S. Roh, J.Y. Seo, S.H. Hong
• Journal: Journal of Korean Medical Science
• Published: 2001;16(6):792-5
• Type of article: Case report
• Cited by: 17 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Angiosarcomas of the pleura are very rare tumors and it is difficult to differentiate them from other common pleural tumors such as mesothelioma and metastasic carcinoma clinically and pathologically. We report a case of a young Korean woman with angiosarcoma arising in the pleura. A 34-yr-old woman presented with dyspnea and chest tightness and pain for several months. A computed tomographic scan of the chest showed diffuse thickening of the left pleura and effusion with passive atelectasis. At thoracotomy the left pleura was thick and indurated. Histologically, the decorticated pleura revealed infiltration of sheets or cords of polygonal and epithelioid tumor cells showing rudimentary vascular differentiation. Immunohistochemically, the tumor cells were strongly positive for CD31, CD34, and vimentin, whereas weakly positive for factor VIII, and negative for cytokeratin, which are characteristic and specific findings of angiosarcoma.

In plain English

This was a case report of a 34-year-old Korean woman with epithelioid angiosarcoma, a rare blood-vessel cancer, in the left pleura, the lining around the lung. She had months of shortness of breath, chest tightness and pain. Four months earlier she had been clinically diagnosed with tuberculosis and treated, without improvement; the authors noted this diagnosis might have been wrong. The authors reported she had no history of smoking or occupational asbestos exposure. A CT scan showed diffuse left pleural thickening with fluid. At surgery, thick tumor tissue encased the lung, and thickened pleural tissue was removed (decortication). Under the microscope, parts looked like spread from breast or kidney cancer. However, stains were strongly positive for CD31, CD34 and vimentin, weakly positive for factor VIII, and negative for cytokeratin. After two cycles of adriamycin and ifosfamide chemotherapy, she was alive at 5 months with no sign of recurrence or spread, but seriously ill with severe chest pain. The authors found no specific cause in her case. They concluded that distinguishing pleural angiosarcoma from mesothelioma and metastatic carcinoma is very important.

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 34-year-old Korean woman with dyspnea, chest tightness and pain for several months, previously diagnosed clinically with tuberculosis; reported from Dong-A University, Busan, Korea
• Exposure: No asbestos exposure: the patient had no history of smoking or occupational exposure to asbestos
• Main results:
  • She had been clinically diagnosed with tuberculosis 4 months before and treated, but her symptoms did not improve
  • Chest CT showed diffuse thickening of the left pleura with effusion
  • At thoracotomy the tumor formed variably thick rinds of tissue encasing the lung
  • She underwent pleural decortication; the resected pleural tissues were leathery and thickened
  • Some areas looked like breast carcinoma or metastatic renal cell carcinoma; unlike mesothelioma, there were sharply delimited intracytoplasmic vacuoles containing red blood cells
  • Tumor cells were strongly positive for CD31, CD34 and vimentin, weakly positive for factor VIII, and negative for cytokeratin, EMA, S100 protein and smooth muscle actin
  • The authors diagnosed epithelioid angiosarcoma rather than epithelioid hemangioendothelioma
  • After surgery she received two cycles of adjuvant chemotherapy with adriamycin and ifosfamide
  • At 5 months there was no evidence of recurrence or metastasis, but she was in serious condition with severe chest pain
  • The authors found no specific cause in this case and noted that her tuberculosis diagnosis might not have been correct
• The authors' conclusion: The authors concluded that pleural angiosarcoma frequently shows epithelioid features and that distinguishing it from mesothelioma and metastatic carcinoma is very important

Topics

Medical terms in this study, explained in our Lexicon: Epithelioid, Pleura, Mesothelioma, Carcinoma, Dyspnea, Atelectasis, Thoracotomy, CD34.

Related studies

• Primary pleural epithelioid angiosarcoma. A case report and review of the literature — E. Dainese, B. Pozzi, et al. (2010)
• Malignant epithelioid vascular tumors of the pleura: report of a series and literature review — P.J. Zhang, V.A. Livolsi, et al. (2000)
• Pseudomesotheliomatous angiosarcoma: a pleuropulmonary lesion simulating malignant pleural mesothelioma — G. Falconieri, R. Bussani, et al. (1997)
• Angiosarcoma of the chest wall — T. Suzuki, S. Suzuki, et al. (1996)
• Frequent expression of conventional endothelial markers in pleural mesothelioma: usefulness of claudin-5 as well as combined traditional markers to distinguish mesothelioma from angiosarcoma — Y. Nakashima, K. Inamura, et al. (2020)
• Primary pleural angiosarcoma associated with pneumoconiosis: An autopsy case — K. Matsuda, T. Yamaryo, et al. (2015)

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