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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 811-813
Primary epithelioid angiosarcoma of bone: A case report with immunohistochemical study


1 Department of Pathology, Kasturba Medical College, Manipal University, Manipal, India
2 Department of Orthopaedics, Kasturba Medical College, Manipal University, Manipal, India

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Date of Web Publication27-Oct-2010
 

   Abstract 

Primary malignant vascular tumors of the bone are exceedingly rare and represent <1% of primary malignant bone tumors. Angiosarcoma is a malignant mesenchymal neoplasm in which the neoplastic cells demonstrate endothelial differentiation. Epithelioid angiosarcoma (EA) is a rare variant of angiosarcoma that is characterized by large cells with an epithelioid morphology. EA is an aggressive tumor with poor prognosis. Here, we present a case of a 62-year-old man who had primary EA of the left tibia. He was treated with amputation and chemotherapy. After 1 month of chemotherapy, he developed pleural effusion and died.

Keywords: Bone, epithelioid angiosarcoma, tumor, CD34

How to cite this article:
Kudva R, Perveen S, Janardhana A. Primary epithelioid angiosarcoma of bone: A case report with immunohistochemical study. Indian J Pathol Microbiol 2010;53:811-3

How to cite this URL:
Kudva R, Perveen S, Janardhana A. Primary epithelioid angiosarcoma of bone: A case report with immunohistochemical study. Indian J Pathol Microbiol [serial online] 2010 [cited 2020 Nov 28];53:811-3. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/811/72103



   Introduction Top


Angiosarcoma is an uncommon neoplasm characterized by rapidly proliferating, extensively infiltrating anaplastic cells derived from blood vessels and lining irregular blood-filled spaces. Angiosarcomas are aggressive and tend to recur locally, spread widely and have a high rate of lymph node and systemic metastasis. [1] Skeletal angiosarcoma generally affects young adults and elderly individuals. [2] The bones of the lower limb, particularly the femur and the tibia, are most commonly involved, followed by pelvis, vertebral column and bones of upper limbs. [2]

The term "epithelioid angiosarcoma" epithelioid has been used to designate a morphological variant of angiosarcoma characterized by poorly differentiated epithelial-like cells arranged in a carcinoma-like pattern, but which still forms identifiable vascular channels. This has been described infrequently in a variety of sites, including the skin, thyroid, adrenal gland, gall bladder, maxilla and deep soft tissue. [3]


   Case Report Top


A 62-year-old man presented with history of pain and swelling in the left leg of 20 days duration. Plain radiograph and magnetic resonance imaging revealed a multifocal osteolytic lesion in the proximal metaphysis of the left tibia, which destroyed the cortical bone [Figure 1] and [Figure 2]. A trucut biopsy of the bone lesion revealed a tumor composed of anastomosing vascular channels and cystic spaces lined by malignant cells with pleomorphic nuclei, prominent nucleoli and brisk mitotic activity. Focal solid pattern with epithelioid appearance of cells was seen [Figure 3], [Figure 4], [Figure 5]. Immunohistochemical analysis showed CD34 positivity [Figure 6], which confirmed the vascular origin of the tumor, and a diagnosis of primary EA of bone was made. Lower limb amputation (transfemoral) was performed. Specimen of the below-knee amputated leg showed a hemorrhagic tumor mass invading the upper end of the tibia with erosion of cortex, soft tissue extension and extension into medullary cavity up to the mid shaft of the tibia [Figure 7]. Histopathological examination of the resected tumor confirmed the initial diagnosis of EA. Postoperative chemotherapy was given, but the patient discontinued the course. He was on follow-up, and 1 month after surgery, he developed hemorrhagic pleural effusion and succumbed to the disease.
Figure 1: Plain radiograph showing an osteolytic mass with surrounding sclerosis in the proximal aspect of the left tibia - posterior view

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Figure 2: Magnetic resonance imaging showing break in the cortex and lesion extending posteriorly into the popliteal fossa

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Figure 3: Epithelioid appearance of tumor cells (H and E, ×200)

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Figure 4: Angiosarcoma showing irregular anastomosing vascular channels lined by atypical endothelial cells. Intraluminal erythrocytes are visible (H and E, ×200)

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Figure 5: Tumor with entrapped bony trabeculae seen (H and E, ×100)

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Figure 6: CD34 positivity of neoplastic cells by immunohistochemistry (IHC, ×200)

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Figure 7: Cut-surface revealed a hemorrhagic tumor mass measuring 10 cm × 6.5 cm, involving the upper end of the tibia with erosion of the cortex and extending into the soft tissue

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   Discussion Top


Angiosarcoma arises from the vascular endothelium and commonly occurs in soft tissue and skin. [1] Primary angiosarcoma of the bone is very rare and accounts for <1% of malignant bone tumors. [1] It occurs more commonly in middle age and later life, with a male predominance in the ratio of 2:1. [2] Angiosarcoma of bone has a tendency to involve the long tubular bones, and multifocal involvement is common. [4]

EA refers to a rare variant of angiosarcoma composed of neoplastic cells that have an epithelioid morphology. These tumors can be multifocal, affecting elderly male patients, and are often diagnosed radiologically as metastatic carcinoma. [5],[6] Histologically, EA is characterized by the proliferation of cells with a remarkable epithelioid morphology, exhibiting a predominantly sheet-like growth pattern and, occasionally, a pseudoglandular or alveolar arrangement. The cells display significant pleomorphism and cytologic atypia with irregular nuclear membrane and prominent nucleoli. Cystically dilated spaces filled with blood, cytoplasmic vacuoles with entrapped erythrocytes, abundant and abnormal mitosis and necrosis are frequent findings. [6]

The differential diagnosis of EA of bone includes other epithelioid vascular tumors such as epithelioid hemangioma (EH), epithelioid hemangioendothelioma (EHE) and metastatic carcinoma. EH occurs in adult patients, ranging in age from the second to the eighth decades of life, usually solitary, involving the long tubular bones. [5],[6] Lobular growth pattern is an important diagnostic feature of this tumor. The cells are bland and have lobated nuclei with occasional grooves, fine chromatin and small nucleoli, mitosis <5/10 HPF. Cytoplasm is abundant and eosinophilic with vacuoles that may be empty or have fragments of red cells in them. Sheets and cords of cells may also be seen. An inflammatory component rich in eosinophils and plasma cells is often present in the stroma. [5]

EHE of bone occurs primarily in adults, with a slight male predominance. They tend to develop in the long tubular bones and multifocality is present in up to 50% of the cases. Radiographically, they are predominantly lytic with bone expansion or peripheral sclerosis. The histologic appearance is characterized by cords and nests of epithelioid cells in a myxohyaline and chondroid background. The cells are epithelioid, with intracytoplasmic lumina showing entrapped erythrocytes. Spindle cells may predominate. Cellular pleomorphism is minimal. [5],[6]

EA can be distinguished from EHE by the pronounced cellular atypia, less-frequent cytoplasmic vacuoles, lack of chondromyxoid matrix and scarcity of cord-like growth of endothelial cells. EH can be distinguished from EA by the presence of well-formed blood vessels, rare or no nuclear atypia and the presence of an inflammatory component rich in eosinophils and plasma cells. [5]

Metastatic carcinoma may be difficult to distinguish from EA because both tumors can involve multiple bones, affect older individuals and are composed of sheets of epithelioid tumor cells. [7] Features favoring diagnosis of EA are presence of well-formed vascular channels, cytoplasmic vacuoles that are mucin negative with intact or fragments of red blood cells, an intratumoral neutrophilic infiltrate and expression of vascular markers on immunohistochemistry. [5]

The presence of cells with pleomorphic nuclei, prominent nucleoli and increased mitotic activity helped in differentiating the present case from EH and EHE. The focal presence of anastamosing vascular channels lined by malignant cells helped distinguish this tumor from metastatic carcinoma. EA also has overlapping histologic features with epithelioid sarcoma, including CD 34 positivity. [3] However, EA sarcoma commonly exhibits central necrosis, minimal nuclear pleomorphism and inflammatory infiltrate at the periphery of the tumor.

Distinguishing EA from EH, EHE and metastatic carcinoma is important because they differ significantly in their clinical behavior and hence treatment and prognosis. EA is an extremely aggressive tumor with a poor prognosis. Despite wide resection and adjuvant radiation and chemotherapy, most of these patients die within 1-2 years of diagnosis. [4],[8] In contrast, EH is usually treated with curettage and has an excellent prognosis. [5] The treatment of EHE is varied and includes curettage, en bloc resection and radiotherapy. [5] It has a better prognosis than EA. The treatment of metastatic carcinoma is different from that of angoisarcoma of bone, which is not disseminated. [4] When an angiosarcoma of bone is identified, a skeletal survey is recommended to evaluate whether the patient has multicentric disease. [2]


   Conclusion Top


EA of bone is a rare but distinct neoplasm with prominent epithelioid morphology. Awareness of the existence of this tumor is essential to avoid misdiagnosis of metastatic carcinoma due to the epithelioid appearance of the tumor cells and because of differences in their management and clinical course.

 
   References Top

1.Volpe R, Mazabraud A. Hemangioendothelioma (Angiosarcoma) of Bone- A Distinct Pathologic entity with an unpredic table course. Cancer 1982;49;727-36.  Back to cited text no. 1
    
2.Saglik Y, Yildiz Y, Atalar H, Basarir K. Primary angiosarcoma of the fibula: A case report. Acta Orthop Belg 2007;73:799-803.  Back to cited text no. 2
[PUBMED]    
3.Hasegawa T, Fujii Y, Seki K, Yang P, Hirose T, Matsuzaki K, et al. Epithelioid angiosarcoma of bone. Hum Pathol 1997;28:985-88.  Back to cited text no. 3
[PUBMED]    
4.Verbeke SLJ, Bovee JVMG. Bone: Angiosarcoma. Atlas Genet Cytogenet Oncol Haematol 2008;1-4.  Back to cited text no. 4
    
5.Deshpande V, Rosenberg AE, Connell JXO, Nielsen GP. Epithelioid Angiosarcoma of the bone: A series of 10 cases. Am J Surg Pathol 2003;27:709-16.  Back to cited text no. 5
    
6.Deyrup AT, Montag AG. Epithelioid and Epithelial Neoplasms of Bone. Arch Pathol Lab Med 2007;131:205-16.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  
7.Weiss SW, Enzinger FM. Epithelioid Hemangioendothelioma. A vascular tumor often mistaken for a carcinoma. Cancer 1982;50;970-81.  Back to cited text no. 7
    
8.Oliveira CR, Camargo OPD, Filho TEPB,Oliveira RP, Croci AT, Baptista AM. Epithelioid angiosarcoma of bone: Report of a case with immunohistochemical study. Acta orthop Bras 2001;9:1-7.  Back to cited text no. 8
    

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Correspondence Address:
Ranjini Kudva
Professor, Department of Pathology, Kasturba Medical College, Manipal University, Manipal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.72103

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]

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