Primary epithelioid haemangioendothelioma of the peritoneum: an unusual mimic of diffuse malignant mesothelioma

Our plain-English summary of Primary epithelioid haemangioendothelioma of the peritoneum: an unusual mimic of diffuse malignant mesothelioma, by R.L. Attanoos, N.S. Dallimore, A.R. Gibbs (Histopathology, 1997).

The study

• Authors: R.L. Attanoos, N.S. Dallimore, A.R. Gibbs
• Journal: Histopathology
• Published: 1997;30(4):375-7
• Type of article: Case report
• Cited by: 12 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Summary of the article

The authors reported a case of epithelioid haemangioendothelioma, an uncommon low-grade sarcoma of endothelial origin, that arose primarily from the peritoneum and mimicked diffuse malignant mesothelioma both clinically and histologically. A 51-year-old man presented with a short history of increasing abdominal discomfort and distension and had marked ascites. Basic laboratory investigations were normal. Laparotomy showed a confluent multinodular pink tumour covering most of the greater omentum, which was biopsied. Extensive clinical and radiological investigations found no other primary tumour site. The patient deteriorated after surgery, received no specific treatment and died 3 months later. No autopsy was performed. Microscopy showed a broad papillary tumour with a submesothelial proliferation of vacuolated and polygonal epithelioid cells and focal intracytoplasmic lumina containing erythrocytes. Admixed atypical mesothelial cells gave the tumour a biphasic appearance. The mesothelial cells were positive for cytokeratin and focally for thrombomodulin. The underlying tumour was negative for both but strongly positive for von Willebrand factor and QBEnd10 (CD34), which confirmed its endothelial nature. The authors stated that recognising this tumour is important for two reasons. The first is asbestos-related compensation claims: unlike peritoneal mesothelioma, there is no evidence linking epithelioid haemangioendothelioma with asbestos exposure. The second is prognosis. They added that the 3-month survival may indicate a poor outlook for peritoneal tumours with diffuse serosal growth regardless of their histogenesis.

PubMed has no abstract for this study, and the article doesn't print one. We wrote this summary from the full article with the help of AI. It is not the authors' wording.

In plain English

This was a case report by authors at hospitals in Cardiff and Penarth, UK. It described a 51-year-old man who had increasing abdominal discomfort and swelling, with marked fluid in the abdomen (ascites). At surgery, a nodular pink tumour covered most of the greater omentum, a fold of the abdominal lining. Under the microscope the tumour resembled diffuse malignant mesothelioma, including a pattern that looked biphasic. Staining showed that the surface cells were mesothelial. The underlying tumour, however, was strongly positive for von Willebrand factor and QBEnd10, which confirmed that it came from blood-vessel lining (endothelial) cells. Investigations found no other primary tumour, and the diagnosis was primary epithelioid haemangioendothelioma of the peritoneum, a low-grade sarcoma. The man received no specific treatment and died 3 months later. No autopsy was done. The article does not say whether he had been exposed to asbestos. The authors concluded that recognising this mimic matters for asbestos compensation claims and for prognosis. Their reason for the claims point was that, unlike malignant peritoneal mesothelioma, there is no evidence linking this tumour with asbestos exposure.

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
• Who was studied: one 51-year-old man with abdominal discomfort, distension and marked ascites; the authors were based at University Hospital of Wales, Cardiff and Llandough Hospital, Penarth, UK
• Exposure: not reported; the article gives no asbestos exposure history for the patient
• Main results:
  • At laparotomy, a confluent multinodular pink tumour covered most of the greater omentum
  • Extensive clinical and radiological investigations found no other primary tumour site
  • Under the microscope, mesothelial-lined clefts and islands mixed into the tumour gave it an appearance resembling a biphasic pattern
  • The surface mesothelial cells were positive for cytokeratin and focally for thrombomodulin. The underlying tumour was negative for both and strongly positive for von Willebrand factor and QBEnd10, confirming its endothelial nature
  • The diagnosis made was primary epithelioid haemangioendothelioma of the peritoneum
  • No specific treatment was started; the patient died 3 months later and no autopsy was performed
• The authors' conclusion: The authors concluded that awareness of this tumour as a mimic of mesothelioma matters for two reasons. The first is asbestos-related compensation claims: in contrast to malignant peritoneal mesothelioma, they wrote, there is no evidence linking epithelioid haemangioendothelioma with asbestos exposure. The second is prognosis. They added that this patient's 3-month survival may indicate a similarly poor outlook for peritoneal tumours with diffuse serosal growth, whatever their cell of origin.

Topics

The National Library of Medicine indexes this study under subjects our site covers: Mesothelioma, Peritoneal mesothelioma.

Medical terms in this study, explained in our Lexicon: Epithelioid, Peritoneum, Malignant Mesothelioma, Sarcoma, Ascites, Laparotomy, Omentum, Autopsy.

Related studies

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• Spontaneous bilateral haemothorax as presentation of primary pleural epithelioid haemangioendothelioma — L. Ferreiro, E. San-José, et al. (2017)
• 'Mucin-positive' epithelial mesothelioma of the peritoneum: an unusual diagnostic pitfall — D.S. Cook, R.L. Attanoos, et al. (2000)
• Value of mesothelial and epithelial antibodies in distinguishing diffuse peritoneal mesothelioma in females from serous papillary carcinoma of the ovary and peritoneum — R.L. Attanoos, R. Webb, et al. (2002)
• Malignant pleural mesothelioma mimicking the intrapulmonary growth pattern of epithelioid hemangioendothelioma — Y. Muramatsu, K. Isobe, et al. (2014)
• CAMTA1 immunostaining is not useful in differentiating epithelioid hemangioendothelioma from its potential mimickers — Z. Yusıflı, K. Kösemehmetoğlu (2014)

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