Primary pleural epithelioid angiosarcoma. A case report and review of the literature

Our plain-English summary of Primary pleural epithelioid angiosarcoma. A case report and review of the literature, by E. Dainese, B. Pozzi, M. Milani, et al (Pathology, Research and Practice, 2010).

The study

• Authors: E. Dainese, B. Pozzi, M. Milani, G. Rossi, M.G. Pezzotta, G. Vertemati, P. Tricomi, F. Sessa
• Journal: Pathology, Research and Practice
• Published: 2010;206(6):415-9
• Type of article: Case report, Review
• Cited by: 35 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Malignant vascular tumors are uncommon sarcomas that arise from endothelial cells of small blood vessels and may affect every organ. Pleural localization is very exceptional, and only 48 cases have been reported in the English literature to date. Even if etiological factors implicated in the development of vascular sarcomas are still unclear, the strongest association with the disease was a history of chronic tuberculous pyothorax, observed only in Japanese patients, while prior radiotherapy and occupational exposure to asbestos have been reported in few Western cases. The mean age at diagnosis was 58 years, and the male to female ratio was 6:1. The overall prognosis was poor, and most of the patients died of disease soon after diagnosis. Histological features and clinical presentation often cause several problems in the differential diagnosis, particularly with mesothelioma and metastasis from poorly differentiated carcinomas. Immunohistochemistry plays an important role in identifying these rare entities, confirming the endothelial origin of the neoplasm with the expression of at least one of the vascular markers CD31, CD34, or factor VIII-related antigen. We report herein a further case of a 62-year-old man who presented with progressive dyspnea and bilateral massive hemothorax. The overall pathological and immunohistochemical features of the pleural specimens supported the diagnosis of epithelioid angiosarcoma.

2009 Elsevier GmbH. All rights reserved.

In plain English

This article was a case report with a review of the published literature. The authors described a 62-year-old Caucasian man, a smoker, with worsening breathlessness and large collections of bloody fluid on both sides of the chest. A CT scan showed no tumor mass. Biopsies of the pleura (the lining around the lungs) showed a tumor of large, highly abnormal cells. Staining tests were strongly positive for the blood-vessel marker CD31 and negative for calretinin, a mesothelioma marker. These results supported a diagnosis of epithelioid angiosarcoma, a cancer of blood-vessel lining cells. The report stated that the patient had no significant occupational history; asbestos exposure was not mentioned for him. The patient died three days after diagnosis. In their review, the authors counted 48 earlier published cases of primary pleural malignant vascular tumors. Occupational asbestos exposure had been described in a few of those earlier reports. Most patients in those earlier reports had died of the disease within 6 months after diagnosis. The authors concluded that these tumors are rare and not clinically well defined, and that further reports are needed to understand their causes.

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 with review of the literature
• Who was studied: One 62-year-old Caucasian man who smoked, first admitted to the emergency unit of another hospital; the authors worked at a hospital in Lecco, Italy. The review covered 48 previously published cases of primary pleural malignant vascular tumors in the English literature.
• Exposure: No asbestos exposure was reported for this patient; the article states he had no significant occupational history. Asbestos appears only in the literature review, as a risk factor reported in earlier cases.
• Main results:
  • In this patient, chest X-rays showed large pleural effusions on both sides, and about 600 mL of bloody fluid was drained from the right side.
  • After a chest tube was placed on the right, a further 1300 mL of bloody fluid was drained from this patient.
  • CT imaging of the patient showed no tumor mass or metastatic nodules.
  • The patient's tumor had a mitotic index (a measure of cell division) of 21 per 10 high-power fields.
  • The patient's tumor cells stained strongly for vimentin, WT1 and the endothelial (blood-vessel lining) marker CD31.
  • Epithelial markers (pan-CK, CK7, CK8.18) were intensely expressed in about 30% of the patient's tumor cells.
  • The patient's tumor was negative for calretinin, MART-1, CK5, CK20, BerEP4, CEA and HHV8 (human herpesvirus 8).
  • The proliferative index (MIB-1) of the patient's tumor was about 40%.
  • The patient died three days after the diagnosis of primary epithelioid angiosarcoma of the pleura.
  • Literature review: 48 earlier cases of primary pleural malignant vascular tumor had been reported in English. Of these, 17 (35%) were described as epithelioid hemangioendothelioma and 31 (65%) as angiosarcoma.
  • Literature review: among the reported cases, the mean age at diagnosis was 58 years (range 22 to 80 years), and the male to female ratio was 6:1.
  • Literature review: earlier reports listed occupational asbestos exposure as a risk factor for pleural vascular sarcomas, with putative latent periods of 17 to 60 years and cumulative exposure of 0.4 to 8 years. Other reported risk factors were prior radiotherapy, cigarette smoking, and, in Japanese patients only, tuberculosis complicated by chronic pyothorax or pleuritis.
  • Literature review: most reported patients died of the disease within 6 months after diagnosis.
• The authors' conclusion: The authors concluded that malignant pleural vascular sarcomas are rare and clinically not well defined, and that further reports are needed to understand the mechanisms behind their development.

Topics

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

Medical terms in this study, explained in our Lexicon: Epithelioid, Sarcoma, Empyema, Occupational Exposure, Prognosis, Differential Diagnosis, Mesothelioma, Metastasis.

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