|Year : 2016 | Volume
| Issue : 1 | Page : 60-62
Epithelioid hemangioendothelioma of the femur
Navneet Jain, Neha Jindal, Manisa Pattanayak, Sunil Saini
Department of Onco Surgery, Cancer Research Institute, Swami Rama Himalayan University, Dehradun, Uttarakhand, India
|Date of Web Publication||28-Jul-2016|
Dr. Neha Jindal
Department of Onco Surgery, Cancer Research Institute, Swami Rama Himalayan University, Jollygrant, PO - Doiwala, Dehradun - 248 140, Uttarakhand
Source of Support: None, Conflict of Interest: None
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
| Introduction|| |
Epithelioid hemangioendothelioma (EHE) is a rare, well-differentiated endothelial tumor with a wide spectrum of behavior forming less than 1% of primary bone tumors. 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. EHE is the most aggressive member of this group of tumors in terms of frequent local recurrences and metastatic potential. EHE growing in the bone is usually multifocal or multicentric in contrast to soft tissue tumors., The surgical strategy differs, depending on the primary localization of the tumor: Excision, large resection with bone reconstruction, or amputation.
| Case Report|| |
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 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|
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| Discussion|| |
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. Besides EHE, the group of hemangioendotheliomas includes the kaposiform Dabska tumor, spindle-cell, hobnail hemangioendothelioma, and composite. EHE occurs in both sex groups during the second or third decade of life, 60-80% involving the femur, tibia, fibula, or metatarsals. 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., 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. 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., The EHE cells show immunoreactivity for CD31, CD34, factor VIII, and vimentin. 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.,
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. Amputation above the elbow was adopted in the case reported by Lai et al. 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.
The role of radiotherapy remains controversial. In the series of Kleer et al. 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. The role of adjuvant chemotherapy, on the other hand, in the treatment of EHE, has not been established so far.
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. On the same series, the mortality rate was 13%.
| Conclusion|| |
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]