Print this page Email this page
Users Online: 228
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 6  |  Issue : 1  |  Page : 60-62

Epithelioid hemangioendothelioma of the femur


Department of Onco Surgery, Cancer Research Institute, Swami Rama Himalayan University, Dehradun, Uttarakhand, India

Date of Web Publication28-Jul-2016

Correspondence Address:
Dr. Neha Jindal
Department of Onco Surgery, Cancer Research Institute, Swami Rama Himalayan University, Jollygrant, PO - Doiwala, Dehradun - 248 140, Uttarakhand
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.187195

Rights and Permissions
  Abstract 

Epithelioid hemangioendothelioma (EHE) of the bone is extremely uncommon and only 0.01% of the cancer population has it. It is characterized by epithelioid endothelial cells and has variable biological behavior. Because the behavior of these tumors is intermediate between angiosarcoma and hemangioma, it is important to not misdiagnose EHE as an angiosarcoma. EHE typically occurs in patients of 20-40 years of age with no sex predilection. Approximately, half of EHE is present with multifocal disease. Here, we describe the case of a 60-year-old female who presented with pain and swelling of the right thigh. Contrast Enhanced Computed Tomography (CECT) of the right thigh revealed multiple lytic lesions in the right femur with cortical destruction with multiple peripherally enhancing lesions in the muscular plane along the shaft of the right femur. Histopathology report from bone tissue showed connective tissue tumor with CK, Vimentin, CD31, and Fil-1 positivity on immunohistochemistry (IHC). In view of multiple lytic lesions of bone, right hip disarticulation was done instead of salvage surgery of the limb. The tumor is locally aggressive and has high rates of local recurrence. Thereby, necessitating aggressive local treatment which is mostly surgical. These tumors are not generally chemo and radiosensitive, hence these modalities cannot be used as adjuvant to incomplete surgery or local recurrence. IHC plays a valuable role in characterizing these tumors due to its variable biological behavior.

Keywords: Connective tissue tumor, epithelioid hemangioendothelioma (EHE), Immunohistochemistry (IHC)


How to cite this article:
Jain N, Jindal N, Pattanayak M, Saini S. Epithelioid hemangioendothelioma of the femur. Arch Int Surg 2016;6:60-2

How to cite this URL:
Jain N, Jindal N, Pattanayak M, Saini S. Epithelioid hemangioendothelioma of the femur. Arch Int Surg [serial online] 2016 [cited 2021 Sep 16];6:60-2. Available from: https://www.archintsurg.org/text.asp?2016/6/1/60/187195


  Introduction Top


Epithelioid hemangioendothelioma (EHE) is a rare, well-differentiated endothelial tumor with a wide spectrum of behavior forming less than 1% of primary bone tumors.[1] The term hemangioendothelioma is used for a group of vascular tumors with intermediate malignancy showing a histopathological appearance between that of a hemangioma and angiosarcoma.[2] EHE is the most aggressive member of this group of tumors in terms of frequent local recurrences and metastatic potential.[3] EHE growing in the bone is usually multifocal or multicentric in contrast to soft tissue tumors.[3],[4] The surgical strategy differs, depending on the primary localization of the tumor: Excision, large resection with bone reconstruction, or amputation.[1]


  Case Report Top


A 60-year-old lady was admitted with a painful right thigh mass for 2 years. She had trauma 3 months back, following which she developed midshaft fracture of the right femur as suggested on X-ray. She underwent right femoral intramedullary nailing along with bone curettage that was sent as biopsy. Histopathology report from bone tissue showed connective tissue tumor [Figure 1] with cytokeratin (CK), Vimentin, cluster of differentiation 31 (CD31), and Fil-1 positivity on immunohistochemistry (IHC) suggesting EHE. Contrast Enhanced Computed Tomography (CECT) of the right thigh showed multiple lytic lesions in the right femur with cortical destruction and multiple peripherally enhancing lesions in the muscular plane along the shaft of the right femur [Figure 2]. Metastatic work-up showed no distant metastasis. The patient was scheduled for elective surgical resection. In view of multiple lytic lesions of bone and pathological fracture, she was planned for a right hip disarticulation [Figure 3] instead of limb salvage surgery. Intraoperatively, the tumor was found to have direct continuity with the bone.
Figure 1: Image suggesting connective tissue tumor

Click here to view
Figure 2: CECT suggestive of multiple lytic lesions in the right femur with cortical destruction with multiple peripherally enhancing lesions in the muscular plane along the shaft of the right femur

Click here to view
Figure 3: Right lower limb followed by hip disarticulation

Click here to view



  Discussion Top


EHE was described for the first time in 1982 by Weiss and Enzinger as a subgroup of the hemangioendotheliomas having an important endothelial or histiocytic component.[3] Besides EHE, the group of hemangioendotheliomas includes the kaposiform Dabska tumor, spindle-cell, hobnail hemangioendothelioma, and composite.[5] EHE occurs in both sex groups during the second or third decade of life, 60-80% involving the femur, tibia, fibula, or metatarsals.[6] Multiple lesions may be present at the level of one single bone. Additionally, in the bone, EHE is found in the metaphysis or epiphysis. In 55% cases of EHE is multicentric and in 40% cases, several bones are involved.[4],[5] Clinically, EHE is accompanied by pain and swelling. On radiological examination, EHE is an expansive, osteolytic, and poorly demarcated lesion. Pathological fractures may occur as well.[7] Taking into consideration the rarity of the malignant character of bone EHE, the primary bone sarcoma would be differential diagnosis.

The histopathological examination of EHE shows strands or solid nests of epithelioid endothelial cells usually growing centrifugally around a central vein in a stroma varying from myxoid to hyalinized. The cells form primitive vascular channels, occasionally containing erythrocytes. The epithelioid endothelial cells typically have abundant eosinophilic cytoplasm with vacuoles. The nucleus is usually oval shaped and concentrically placed.[2],[4] The EHE cells show immunoreactivity for CD31, CD34, factor VIII, and vimentin.[8] Positive reaction for cytokeratin and epithelial membrane antigen (EMA) have been noted in one-fourth of the cases but the expression is weak and focal.[2],[4]

Because of the high incidence of the multifocal lesions in EHE, it is necessary to perform bone scanning and design the treatment plan according to the results. Radical surgery may be performed if the tumor is suitable for resection.

Survival to local recurrence was 76% at 10 years, significantly higher after wide resection than after intralesional surgery.[9] Amputation above the elbow was adopted in the case reported by Lai et al.[10] of a Kasabach-Merritt syndrome, characterized by a malignant hemangioendothelioma. The case was reported by Duncan as an expandable tumor of the distal third of the pathologically fractured radius which, after resection, required a radius reconstruction.[5]

The role of radiotherapy remains controversial. In the series of Kleer et al.[4] four out of 10 patients who underwent radiotherapy for EHE had a favorable evolution. However, other studies conclude that due to risk of radiation-induced sarcomas, radiation therapy should be reserved to those cases not amenable to wide surgery or when lesions are seated locations difficult to treat.[9] The role of adjuvant chemotherapy, on the other hand, in the treatment of EHE, has not been established so far.[4]

A study on 46 patients reported primary recurrence rate in 13% and local-regional lymph node metastases in 31% during a 48-month follow-up period.[3] On the same series, the mortality rate was 13%.[5]


  Conclusion Top


EHEs are a select subgroup of vascular tumors that do not lend itself well to characterization both clinically and histologically. Variously known as low degree anaplastic angiosarcoma, cellular hemangioma, histiocytoid hemangioma, or hemangioendothelioma, are locally aggressive and have high rates of recurrence, thereby necessitating aggressive local treatment which is mostly surgical. These tumors are not generally chemo and radiosensitive, hence, these modalities cannot be used as adjuvant to incomplete surgery or local recurrence. Treatment plans should, therefore, be tailored to the individual patient in correlation with the extension and location of the disease. IHC plays a valuable role in characterizing these tumors.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Gherman CD, Fodor D. Epithelioid hemangioendothelioma of the forearm with radius involvement. Case report. Diagn Pathol 2011;6:120.  Back to cited text no. 1
    
2.
Weiss SW, Goldblum JR. Hemangioendothelioma: Vascular tumors of intermediate malignancy. In: Weiss SW, Goldblum JR, editors. Enzinger and Weiss's Soft Tissue Tumors. 4th ed. St. Louis: Mosby Yearbook; 2001. p. 891-915.  Back to cited text no. 2
    
3.
Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: A vascular tumor often mistaken for a carcinoma. Cancer 1982;50:970-81.  Back to cited text no. 3
[PUBMED]    
4.
Kleer CG, Unni KK, McLeod RA. Epithelioid hemangioendothelioma of bone. Am J Surg Pathol 1996;20:1301-11.  Back to cited text no. 4
    
5.
Duncan FM, Krochmal DJ, Craft RO, Merritt MV, Smith AA. Epithelioid Hemangioendothelioma of the distal radius: A case report. RCR 2007;2.  Back to cited text no. 5
    
6.
Tsuneyoshi M, Dorfman HD, Bauer TW. Epithelioid hemangioendothelioma of bone. A clinicopathologic, ultrastructural, and immunohistochemical study. Am J SurgPathol 1986;10:754-64.   Back to cited text no. 6
    
7.
Available from: http://www.bonetumor.org/unknowntumor-soft-tissue/epithelioid-hemangioendothelioma [Last accessed on 2015 Nov 13].  Back to cited text no. 7
    
8.
Kabukcuoglu F, Kabukçuoğlu Y, Livaoğlu A, Ozağari A, Armağan R, Kuzgun U. Epithelioid hemangioendothelioma of bone. Acta Orthop Traumatol Turc 2006;40:324-8.  Back to cited text no. 8
    
9.
Angelini A, Mavrogenis AF, Gambarotti M, Merlino B, Picci P, Ruggieri P. Surgical treatment and results of 62 patients with epithelioid hemangioendothelioma of bone. J Surg Oncol 2014;109:791-7.  Back to cited text no. 9
    
10.
Lai FM, Allen PW, Yuen PM, Leung PC. Locally metastasizing vascular tumor. Spindle cell, epithelioid, or unclassified hemangioendothelioma? Am J Clin Pathol 1991;96:660-3.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed2050    
    Printed54    
    Emailed0    
    PDF Downloaded138    
    Comments [Add]    

Recommend this journal