Cytologic features, immunocytochemical findings, and DNA ploidy in four rare cases of epithelioid hemangioendothelioma involving effusions

Our plain-English summary of Cytologic features, immunocytochemical findings, and DNA ploidy in four rare cases of epithelioid hemangioendothelioma involving effusions, by Y. Chen, A. Khanna, J.Q. Chen, et al (CytoJournal, 2018).

The study

• Authors: Y. Chen, A. Khanna, J.Q. Chen, H.Z. Zhang, N.P. Caraway, R.L. Katz
• Journal: CytoJournal
• Published: 2018;15:13
• Cited by: 3 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Background: Epithelioid hemangioendothelioma (EHE) involving serous effusion is extremely rare, and the diagnosis can be challenging. DNA ploidy quantitation of EHE in effusion fluids has not been previously described in the English-language literature.

Methods: Specimens of cytological diagnosed with EHE in effusion fluids between 2002 and 2009 were retrieved from the pathology files at MD Anderson Cancer Center. A total of four cases of EHE involving or arising from effusion fluids were found, and we reviewed cytospin, smears, cell block sections, and immunostained slides. DNA image analysis for ploidy and proliferation evaluation was performed on a destained, papanicolaou-stained slide from each case.

Results: The tumor cells were epithelioid with prominent cytoplasmic vacuolization and intracytoplasmic inclusions, which could resemble reactive mesothelial cells, mesothelioma, or adenocarcinoma. The tumor cells were positive for endothelial markers. DNA image analysis in three of the four cases revealed predominantly diploid and tetraploid subpopulations, with few aneuploid cells and fairly low proliferation indices, and these patients had fairly prolonged survival.

Conclusions: DNA image analysis is useful for differentiating EHE from reactive mesothelial cells and high-grade carcinoma. For accurate diagnosis of EHE in effusion fluids, cytologic features should be considered together with clinical history and ancillary studies.

In plain English

This article described four cases of epithelioid hemangioendothelioma (EHE), a rare blood-vessel tumor, in pleural or abdominal fluid at MD Anderson Cancer Center from 2002 to 2009. All four patients were women aged 20 to 65. Three tumors had spread from elsewhere and one began in the peritoneum. The study did not examine asbestos exposure. The tumor cells resembled plasmacytoid or signet ring cells, and two cases were first diagnosed as something else. The cells stained positive for blood-vessel (endothelial) markers. DNA image analysis showed mainly diploid and tetraploid cells. Cells with very high DNA content made up 0.6% to 11.1%. The proliferation index, measurable in three cases, ranged from 1.5% to 7.0%. Counted from each patient's initial diagnosis, one patient was alive at 60 months and was then lost to follow-up, and the others died at 4, 43 and 48 months. The authors concluded that DNA image analysis may help tell EHE apart from reactive mesothelial cells, high-grade sarcomas and carcinomas. They also concluded that accurate diagnosis is possible when cell features are weighed with clinical history and other tests. They added that the link between DNA ploidy and prognosis needs further study.

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: Retrospective case series (four cases) reviewing cytology slides, immunostains and DNA image analysis, with chart review of clinical outcomes
• Who was studied: Four patients with EHE in serous cavity effusions found in MD Anderson Cancer Center pathology files, 2002 to 2009: three metastatic to the serous cavities and one primary peritoneal EHE. All were women aged 20 to 65 years. Two had ascites and two had pleural fluid.
• Exposure: No asbestos or other environmental or occupational exposure was studied. The study examined tumor cell appearance, immunostaining and DNA ploidy in effusion fluid.
• Main results:
  • All four patients were women aged 20 to 65 years.
  • Tumor cells had eccentric nuclei and resembled plasmacytoid or signet ring cells.
  • Case 4's effusion was first diagnosed as metastatic carcinoma. This was later revised to EHE.
  • Case 1's ascitic fluid was first reported as showing no malignant cells (reactive mesothelial cells). Later testing confirmed EHE cells in the fluid.
  • Tumor cells were positive for the endothelial markers CD31, CD34, FLI-1 and factor VIII.
  • Tumor cells were negative for keratin 7, calretinin and CD68.
  • In case 4, tumor cells showed weak-to-moderate focal pancytokeratin staining.
  • Tumor cells in case 1's peritoneal biopsy were positive for mesothelial markers including podoplanin/D2-40. They were negative for calretinin and CK5/6.
  • In all four cases, DNA image analysis showed mainly diploid and tetraploid or near-tetraploid cells. The degree of hyperploidy (cells with DNA content above 5c) ranged from 0.6% to 11.1%.
  • The proliferation index was evaluable in three cases. It ranged from 1.5% to 7.0%, with a mean of 4.01%.
  • The DNA index of the aneuploid subpopulations ranged from 1.5 to 2.1.
  • The patient alive at 60 months (case 2) had the lowest proliferation index, 1.5%.
  • Case 2 was alive 60 months after the initial diagnosis and was then lost to follow-up. Her effusion appeared 40 months after the first diagnosis.
  • Case 1 died 43 months after her initial diagnosis.
  • Case 3 died of the disease 48 months after the initial diagnosis.
  • Case 4 died 4 months after the initial diagnosis of EHE. The article is inconsistent about the cause of this death.
• The authors' conclusion: The authors concluded that DNA image analysis may be useful for differentiating EHE from reactive mesothelial cells, high-grade sarcomas and carcinomas. They also concluded that accurate cytologic diagnosis of EHE in effusions is possible when cytologic features are considered together with clinical history and ancillary studies.
• Limitations the authors state:
  • The relationship between DNA ploidy and prognosis of EHE was not established and needs further study.
  • Cytological material was limited in some cases, so only selected immunohistochemical markers were used.
  • The proliferation index could not be evaluated in case 3.

Topics

Medical terms in this study, explained in our Lexicon: Epithelioid, Pathology, MD Anderson Cancer Center, Cell Block, Reactive Mesothelial Hyperplasia, Mesothelioma, Adenocarcinoma, Carcinoma.

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