Journal of Current Oncology

: 2020  |  Volume : 3  |  Issue : 2  |  Page : 86--89

Epithelioid hemangioma of clavicle: A case report and review of literature

Thomas Sumi, Surendran Divya, Augustine Joy 
 Department of Pathology, Amala Institute of Medical Sciences, AmalaNagar, Thrissur, Kerala, India

Correspondence Address:
Dr. Thomas Sumi
Department of Pathology, Amala Institute of Medical Sciences, AmalaNagar, Thrissur 680555, Kerala


Epithelioid hemangioma (EH) of the bone is classified within benign vascular tumors. They are uncommon locally aggressive neoplasms. Tumors usually involve long tubular bones. We report a case of EH of clavicle in a 12-year-old girl treated with tumor resection and fibular grafting.

How to cite this article:
Sumi T, Divya S, Joy A. Epithelioid hemangioma of clavicle: A case report and review of literature.J Curr Oncol 2020;3:86-89

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Sumi T, Divya S, Joy A. Epithelioid hemangioma of clavicle: A case report and review of literature. J Curr Oncol [serial online] 2020 [cited 2021 May 17 ];3:86-89
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Full Text


EH is an uncommon locally aggressive neoplasm.[1] Their exact incidence is unknown. Tumors usually involve long tubular bones (40%), distal lower extremities (18%), flat bones (18%), vertebrae (16%), and small bones of the hands (8%).[2] They are classified within benign vascular tumors. However, they are commonly misdiagnosed as low-grade angiosarcoma.[3] We report a case of EH of clavicle.

 Case Presentation

A 12-year-old girl presented with left shoulder pain of a one-year duration. There was no history of fever/ trauma/ weight loss. On examination, an ill-defined globular swelling was identified over left clavicle measuring 4 × 3 × 3 cm. It was fixed to the clavicle. Tenderness and local rise of temperature were present. The patient had no restriction of movements. X-ray showed a lytic expansile lesion in left clavicle.

A subsequent CT picture showed a heterogeneously enhancing well-circumscribed expansile lesion arising from the mid-third of the diaphysis of the left clavicle with multiple septations within [Figure 1]. No obvious cortical breech was noted. Perilesional soft tissue edema was present. CT-guided biopsy from the lesion was done. Microscopy showed tiny vascular spaces lined by endothelial cells, and a diagnosis of hemangioma was given [Figure 2] and [Figure 3]. Later, the patient was lost for follow-up. After five months, she presented with pain at the same site. The X-ray revealed a slight increase in size of the swelling [Figure 4]. CT angiography showed a few small draining veins from the lower margin of lesion draining to brachiocephalic and subclavian veins. A few small arterial feeders were noted to arise from the proximal thyrocervical trunk and thoraco-acromial arteries. Radiological diagnosis was consistent with hemangioma, with the previous biopsy correlation. Magnetic resonance imaging showed a similar picture of a well-defined eccentric mass lesion with septations [Figure 5] and [Figure 6].{Figure 1} {Figure 2} {Figure 3} {Figure 4} {Figure 5} {Figure 6}

Tumor resection of left clavicle and fibular grafting was done [Figure 6]. The specimen was sent to the pathology lab for histopathological examination.

Grossly, a nodular mass was identified as arising from the clavicle measuring 5 × 4.5 × 4 cm. The cut section showed tiny cystic spaces with hemorrhagic areas. The lesion extended to one of the resected margins [Figure 7]. Microscopically, the fairly circumscribed lesion included vascular channels lined by endothelial cells having plump ovoid vesicular nuclei. No atypia or mitosis was noted. Surrounding stroma showed inflammatory cells such as lymphocytes, plasma cells, and eosinophils. One of the resected margins was involved by the tumor [Figure 8][Figure 9][Figure 10].{Figure 7} {Figure 8} {Figure 9} {Figure 10}


EH, previously designated as angiolymphoid hyperplasia with eosinophilia and histiocytoid hemangioma, is a well-recognized distinct clinicopathologic entity. It has been most extensively documented in the skin and subcutis, and it has also been described in bone, colon, heart, breast, eye, penis, and tongue.[4],[5],[6],[7],[8],[9],[10],[11]

The EH of bone usually does not involve medium- or large-sized blood vessels, especially arteries, and it shows a greater degree of histologic variability in comparison to EH of skin and soft tissue. The histologic and cytologic features of EH have been well delineated, and intraosseous tumors have been described as a locally aggressive neoplasm in the World Health Organization (WHO) classification of bone tumors.[2]

Detection of FBJ murine osteosarcoma viral oncogene rearrangement may be a useful diagnostic tool to assist in the often difficult differential diagnosis of vascular tumors of bone. Fusion events lead to truncation of FOS in EH of bone. FISH karyotyping can identify a balanced t(3;14) translocation.[12]

Although EH is most commonly a solitary lesion, approximately 18–25% of EH cases demonstrate multifocality.[13],[14] Zhou et al. published two cases of EH where initial radiological diagnosis of both were metastatic tumors. The EH should be included in the differential diagnosis of a radiographic osteolytic lesion with an aggressive appearance.[5]

Other vascular tumors that can demonstrate epithelioid features and should be considered in the differential diagnosis of EH of bone include epithelioid hemangioendothelioma and epithelioid angiosarcoma. Epithelioid hemangioendothelioma can be distinguished from EH, because the epithelioid cells are arranged in cords, show a greater degree of atypia, and, importantly, unlike EH, are enmeshed in a hyalinized or chondroid matrix. Epithelioid angiosarcoma, in contrast to EH, exhibits overt cytologic features of malignancy with significant cytologic atypia and increased mitotic activity, including atypical forms.[15]

The EH of bone is usually treated with curettage or marginal en bloc excision when appropriate, and it has an excellent prognosis.[16] Tsikopoulos et al. reported a rare case of scapular epithelioid hemangioma that was initially treated by using a combination of chemoembolization and microwave ablation. This combination has not been previously reported in the literature regarding the management of this tumor. Results reported in this case study demonstrated that a combination of chemoembolization and microwave ablation is a feasible, safe, and effective technique in the treatment of bone epithelioid hemangioma. Even if the tumor is still present afterward, a substantially smaller surgical excision will be needed.[17]

Local recurrence or lymph node involvement is a rare complication after incomplete resection of the tumor, despite the fact that long-term follow-up of patients after surgical resection of epithelioid hemangiomas has invariably failed to substantiate the existence of any malignant behavior.[16],[18] Our patient was treated with tumor resection and fibular graft fixation with a Recon plate. Physiotherapy and supportive measures were given. After one year, implant removal was done. The patient is now doing well.


The EH is a neoplasm with distinctive vascular formative features that occasionally show local–regional aggressive growth. Distinguishing EH of the bone from other malignant vascular tumors of the bone is important; their management and clinical outcome have major differences.

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Conflicts of interest

There are no conflicts of interest.


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