Primary pleural angiosarcoma as a mimicker of mesothelioma: a case report \*\*VS\*\*

Our plain-English summary of Primary pleural angiosarcoma as a mimicker of mesothelioma: a case report **VS**, by Y.C. Kao, J.M. Chow, K.M. Wang, et al (Diagnostic Pathology, 2011).

The study

• Authors: Y.C. Kao, J.M. Chow, K.M. Wang, C.L. Fang, J.S. Chu, C.L. Chen
• Journal: Diagnostic Pathology
• Published: 2011;6:130
• Type of article: Case report
• Cited by: 23 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Primary pleural angiosarcoma is a rare and clinically aggressive tumor. Patients usually present with chest pain, dyspnea, hemoptysis and/or cough. Radiologic studies reveal diffuse pleural thickening and pleural effusion with or without mass lesion. The clinical and radiological features both resemble those of mesothelioma, and its definite diagnosis requires careful histologic examination. However, frequent epithelioid feature and immunoreactivity to cytokeratin in primary pleural angiosarcoma further complicate the pathologic diagnosis. The use of proper immunohistochemical stains is often needed to support endothelial differentiation in the tumor cells and to exclude metastatic carcinoma and mesothelioma. We report the case of a 49-year-old male patient with primary pleural angiosarcoma, who presented with initial hemothorax, followed by a rapid progress to an inoperable status.

In plain English

This case report from Taiwan described one 49-year-old man with primary pleural angiosarcoma. This is a rare cancer of blood-vessel-type cells in the lining of the lung, which the authors said can mimic mesothelioma. He had right chest pain and breathlessness. Scans showed fluid and thickening of the pleura but no mass. The fluid was bloody and showed no cancer cells. He had no history of asbestos exposure or tuberculous infection. One month after surgery to strip the thickened pleura, surgeons found a tumor that could not be removed. The tumor cells stained positive for the blood-vessel markers CD31 and FLI-1. They also stained positive for cytokeratin, a marker that can be misleading in diagnosis. Mesothelioma markers were negative. After radiotherapy and chemotherapy, he was alive with the disease nine months after diagnosis. The authors also reviewed 39 previously published cases and noted that the causes in most cases are unknown. They concluded that the tumor may mimic mesothelioma clinically, radiologically and even pathologically. Diagnosis requires careful pathologic examination aided by immunohistochemical stains.

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), with a review of previously published cases in the discussion
• Who was studied: one 49-year-old man treated at Wan Fang Hospital, Taipei Medical University, Taiwan, with right chest pain for one month
• Exposure: No history of asbestos exposure or tuberculous infection. He was an ex-smoker (half a pack per day for 20 years, quit 10 years earlier).
• Main results:
  • Imaging of the patient showed right-side loculated pleural effusion with pleural thickening but no mass
  • The patient's pleural fluid was bloody: its hematocrit was 39.5%, compared with 38.9% in his blood
  • Cell examination of the patient's pleural fluid was negative for malignancy
  • One month after decortication, open thoracotomy found tumor along the earlier surgical wound and encasing the right pulmonary hilum; curative resection could not be done
  • The patient's tumor stained diffusely positive for CD31, FLI-1, cytokeratin (AE1/AE3) and CK7. It was negative for CD34, factor VIII and the mesothelial markers calretinin, CK5/6, HBME-1 and WT-1. An angiosarcoma was diagnosed.
  • After radiotherapy (3750 cGy in 15 fractions) and several chemotherapy regimens, the patient was alive with disease nine months after diagnosis
  • In the authors' review of the literature, 39 cases of primary pleural angiosarcoma had been previously reported, and the causes in most cases were unknown
• The authors' conclusion: The authors concluded that primary pleural angiosarcoma is a highly malignant disease that may mimic mesothelioma clinically, radiologically and even pathologically, and that accurate diagnosis requires careful pathologic examination aided by immunohistochemical stains.

Topics

Medical terms in this study, explained in our Lexicon: Mesothelioma, Dyspnea, Hemoptysis, Diffuse Pleural Thickening, Pleural Effusion, Epithelioid, Carcinoma, Hemothorax.

Related studies

• Primary pleural epithelioid angiosarcoma. A case report and review of the literature — E. Dainese, B. Pozzi, et al. (2010)
• Primary pleural angiosarcoma associated with pneumoconiosis: An autopsy case — K. Matsuda, T. Yamaryo, et al. (2015)
• Pseudomesotheliomatous angiosarcoma: a pleuropulmonary lesion simulating malignant pleural mesothelioma — G. Falconieri, R. Bussani, et al. (1997)
• 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)
• Pleural epithelioid angiosarcoma with lymphatic differentiation arisen after radiometabolic therapy for thyroid carcinoma: immunohistochemical findings and review of the literature — D. Cabibi, G. Pipitone, et al. (2017)
• Epithelioid angiosarcoma of the pleura: a case report — M.S. Roh, J.Y. Seo, et al. (2001)

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