Objectives: Epithelioid hemangioendothelioma (EHE) and epithelioid angiosarcoma (EAS) are rare vascular neoplasms that share many morphological characteristics on histology but demonstrate different clinical behavior. Given the many reported clinical and morphological features shared between EAS and EHE, we examined all cases of EAS and EHE diagnosed primarily on fine needle aspiration (FNA) at our three institutions that were confirmed by a tissue diagnosis. Study Design: A total of 29 cases from 25 patients were identified: 15 EHE from 11 patients and 14 EAS from 14 patients. Results: Many cytomorphological features existed on a spectrum that overlapped considerably between EAS and EHE cases. Common features between the two entities include epithelioid morphology and eccentrically placed nucleus. Intracytoplasmic lumens (ICL), a morphological feature that may suggest vascular origin, can be found in both entities but are not always present. Conclusions: Given the general absence of vascular cytomorphological features, such as ICL, the proper classification of these tumors depends on the successful use of immunoperoxidase markers such as factor VIII-related antigen, ERG, or CD31 as well as a high index of suspicion. The distinction between EAS and EHE on FNA alone is treacherous at best.

1.
Mentzel T, Beham A, Calonje E, Katenkamp D, Fletcher CD: Epithelioid hemangioendothelioma of skin and soft tissues: clinicopathologic and immunohistochemical study of 30 cases. Am J Surg Pathol 1997;21:363-374.
2.
Weiss SW, Enzinger FM: Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 1982;50:970-981.
3.
Tsuneyoshi M, Dorfman HD, Bauer TW: Epithelioid hemangioendothelioma of bone. A clinicopathologic, ultrastructural, and immunohistochemical study. Am J Surg Pathol 1986;10:754-764.
4.
Deshpande V, Rosenberg AE, O'Connell JX, Nielsen GP: Epithelioid angiosarcoma of the bone: a series of 10 cases. Am J Surg Pathol 2003;27:709-716.
5.
Meis-Kindblom JM, Kindblom LG: Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol 1998;22:683-697.
6.
Hart J, Mandavilli S: Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis. Arch Pathol Lab Med 2011;135:268-272.
7.
Fletcher CD, Beham A, Bekir S, Clarke AM, Marley NJ: Epithelioid angiosarcoma of deep soft tissue: a distinctive tumor readily mistaken for an epithelial neoplasm. Am J Surg Pathol 1991;15:915-924.
8.
Miettinen M, Wang ZF, Paetau A, Tan SH, Dobi A, Srivastava S, Sesterhenn I: ERG transcription factor as an immunohistochemical marker for vascular endothelial tumors and prostatic carcinoma. Am J Surg Pathol 2011;35:432-441.
9.
Miettinen M, Wang Z, Sarlomo-Rikala M, Abdullaev Z, Pack SD, Fetsch JF: ERG expression in epithelioid sarcoma: a diagnostic pitfall. Am J Surg Pathol 2013;37:1580-1585.
10.
Boucher LD, Swanson PE, Stanley MW, Silverman JF, Raab SS, Geisinger KR: Cytology of angiosarcoma. Findings in fourteen fine-needle aspiration biopsy specimens and one pleural fluid specimen. Am J Clin Pathol 2000;114:210-219.
11.
Klijanienko J, Caillaud JM, Lagace R, Vielh P: Cytohistologic correlations in angiosarcoma including classic and epithelioid variants: Institut Curie's experience. Diagn Cytopathol 2003;29:140-145.
12.
Gagner JP, Yim JH, Yang GC: Fine-needle aspiration cytology of epithelioid angiosarcoma: a diagnostic dilemma. Diagn Cytopathol 2005;33:429-433.
13.
Gill R, O'Donnell RJ, Horvai A: Utility of immunohistochemistry for endothelial markers in distinguishing epithelioid hemangioendothelioma from carcinoma metastatic to bone. Arch Pathol Lab Med 2009;133:967-972.
14.
Folpe AL, Chand EM, Goldblum JR, Weiss SW: Expression of Fli-1, a nuclear transcription factor, distinguishes vascular neoplasms from potential mimics. Am J Surg Pathol 2001;25:1061-1066.
15.
Pettinato G, Insabato L, De Chiara A, Forestieri P, Manco A: Epithelioid hemangioendothelioma of soft tissue. Fine needle aspiration cytology, histology, electron microscopy and immunohistochemistry of a case. Acta Cytol 1986;30:194-200.
16.
Kilpatrick SE, Koplyay PD, Ward WG, Richards F 2nd: Epithelioid hemangioendothelioma of bone and soft tissue: a fine-needle aspiration biopsy study with histologic and immunohistochemical confirmation. Diagn Cytopathol 1998;19:38-43.
17.
Mukherjee S, Mallick J, Pal PC, Chattopadhyay S: Hemangioendothelioma of soft tissue: cytological dilemma in two cases at unusual sites. J Cytol 2012;29:89-91.
18.
Vesoulis Z, Cunliffe C: Fine-needle aspiration biopsy of postradiation epithelioid angiosarcoma of breast. Diagn Cytopathol 2000;22:172-175.
19.
Lin O, Gerhard R, Coelho Siqueira SA, de Castro IV: Cytologic findings of epithelioid angiosarcoma of the thyroid. A case report. Acta Cytol 2002;46:767-771.
20.
Tong GX, Hamele-Bena D, Borczuk A, Monaco S, Khosh MM, Greenebaum E: Fine needle aspiration biopsy of epithelioid hemangioendothelioma of the oral cavity: report of one case and review of literature. Diagn Cytopathol 2006;34:218-223.
21.
Carretero A, Elmberger PG, Skold CM, Collins BT: Pulmonary epithelioid hemangioendothelioma: report of a case with fine needle aspiration biopsy. Acta Cytol 2006;50:455-459.
22.
Jeon YK, Kim HW, Choi HJ, Park IA: Fine needle aspiration cytology of epithelioid angiosarcoma. Report of a case with nuclear grooves and indentations. Acta Cytol 2004;48:223-228.
23.
Saqi A, Ramdall RB, Hamele-Bena D: Metastatic epithelioid angiosarcoma of the heart in peritoneal fluid. Diagn Cytopathol 2004;30:350-352.
24.
Park JW, Serafica-Karen C, Das K: Fine-needle aspiration of metastatic radiation-induced cutaneous epithelioid angiosarcoma of the breast to the liver: a diagnostic dilemma. Diagn Cytopathol 2010;38:768-771.
25.
Ryu HS, Lee SS, Choi HS, Baek H, Koh JS: A case of pulmonary malignant epithelioid hemangioendothelioma misdiagnosed as adenocarcinoma by fine needle aspiration cytology. Diagn Cytopathol 2011;39:801-807.
26.
Murali R, Zarka MA, Ocal IT, Tazelaar HD: Cytologic features of epithelioid hemangioendothelioma. Am J Clin Pathol 2011;136:739-746.
27.
Liu K, Layfield LJ: Cytomorphologic features of angiosarcoma on fine needle aspiration biopsy. Acta Cytol 1999;43:407-415.
28.
Wakely PE Jr, Frable WJ, Kneisl JS: Aspiration cytopathology of epithelioid angiosarcoma. Cancer 2000;90:245-251.
29.
Siddaraju N, Soundararaghavan J, Bundele MM, Roy SK: Fine needle aspiration cytology of epithelioid angiosarcoma: a case report. Acta Cytol 2008;52:109-113.
30.
Fulciniti F, Di Mattia D, Bove P, Mastro AA, De Chiara A, Botti G, Petrillo A, Apice G: Fine needle aspiration of metastatic epithelioid angiosarcoma: a report of 2 cases. Acta Cytol 2008;52:612-618.
31.
Olson MT, Novak A, Boonyaarunnate T, Shahid H, Kirby J, Ali SZ: Cytotechnologist-attended on-site evaluation of adequacy for fine-needle aspiration of bone and soft tissue neoplasms. J Am Soc Cytopathol 2014;3:60-66.
32.
Olson MT, Novak A, Kirby J, Shahid H, Boonyaarunnate T, Ali SZ: Cytotechnologist-attended on-site evaluation of adequacy for metastatic disease involving bone and soft tissue. Acta Cytol 2013;57:550-556.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.