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Table of Contents
Year : 2020  |  Volume : 3  |  Issue : 2  |  Page : 86-89

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

Department of Pathology, Amala Institute of Medical Sciences, AmalaNagar, Thrissur, Kerala, India

Date of Submission25-Sep-2020
Date of Acceptance03-Dec-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Dr. Thomas Sumi
Department of Pathology, Amala Institute of Medical Sciences, AmalaNagar, Thrissur 680555, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jco.jco_32_20

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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.

Keywords: Epithelioid hemangioma, tubular bones, vascular tumors

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;3:86-9

How to cite this URL:
Sumi T, Divya S, Joy A. Epithelioid hemangioma of clavicle: A case report and review of literature. J Curr Oncol [serial online] 2020 [cited 2022 Sep 30];3:86-9. Available from: http://www.https://journalofcurrentoncology.org//text.asp?2020/3/2/86/305852

  Introduction Top

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 Top

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: X-ray with lytic expansile lesion

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Figure 2: Tiny vascular spaces lined by endothelial cells (H&E ×40)

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Figure 3: Similar morphology as above (H&E ×400)

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Figure 4: X-ray picture with increase in size of the swelling

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Figure 5: Magnetic resonance imaging showing well defined eccentric mass lesion with septations

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Figure 6: Postop X-ray showing clavicle with Recon plate and screws

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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: Gross specimen with cut section showing tiny cystic spaces with haemorrhagic areas

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Figure 8: Epithelioid hemangioma formed of well-formed blood vessels lined by prominent epithelioid endothelial cells (H&E ×40)

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Figure 9: Note the prominent intracytoplasmic vacuoles of some of the cells (H&E ×400)

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Figure 10: Epithelioid hemangioma with prominent inflammatory infiltrate containing numerous eosinophils, scattered lymphocytes and plasma cells (H&E ×400)

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  Discussion Top

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.

  Conclusion Top

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.

  References Top

Wenger DE, Wold LE Benign vascular lesions of bone: Radiologic and pathologic features. Skeletal Radiol 2000;29:63-74.  Back to cited text no. 1
Fletcher C, Unni K, Mertens F World Health Organization Classification of Tumours. Pathology & Genetics. Tumours of Soft Tissue and Bone. Lyon: IARC Press; 2020.  Back to cited text no. 2
O’Connell JX, Kattapuram SV, Mankin HJ, Bhan AK, Rosenberg AE Epithelioid hemangioma of bone. A tumor often mistaken for low-grade angiosarcoma or malignant hemangioendothelioma. Am J Surg Pathol 1993;17:610-7.  Back to cited text no. 3
Ling S, Rafii M, Klein M Epithelioid hemangioma of bone. Skeletal Radiol 2001;30:226-9.  Back to cited text no. 4
Zhou Q, Lu L, Fu Y, Xiang K, Xu L, et al. Epithelioid hemangioma of bone: A report of two special cases and a literature review. Skeletal Radiol2016;45:1723-7.  Back to cited text no. 5
Berney DM, Griffiths MP, Brown CL Angiolymphoid hyperplasia with eosinophilia in the colon: A novel cause of rectal bleeding. J Clin Pathol 1997;50:611-3.  Back to cited text no. 6
de Nictolis M, Brancorsini D, Goteri G, Prat J Epithelioid haemangioma of the heart. Virchows Arch 1996;428:119-23.  Back to cited text no. 7
Nair M, Aron M, Sharma MC Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the breast: Report of a case. Surg Today 2000;30:747-9.  Back to cited text no. 8
Mariatos G, Gorgoulis VG, Laskaris G, Kittas C Epithelioid haemangioma (angiolymphoid hyperplasia with eosinophilia) in the inner canthus. J Eur Acad Dermatol Venereol 2001;15:90-1.  Back to cited text no. 9
Fetsch JF, Sesterhenn IA, Miettinen M, Davis CJ Jr. Epithelioid hemangioma of the penis: A clinicopathologic and immunohistochemical analysis of 19 cases, with special reference to exuberant examples often confused with epithelioid hemangioendothelioma and epithelioid angiosarcoma. Am J Surg Pathol 2004;28:523-33.  Back to cited text no. 10
Shimoyama T, Horie N, Ide F Epithelioid hemangioma of the tongue mimicking a malignancy. J Oral Maxillofac Surg 2000;58:1317-9.  Back to cited text no. 11
IJzendoorn, DG, de Jong D, Romagosa C, Picci P, Benassi MS, Gambarotti M, et al. Fusion events lead to truncation of FOS in epithelioid hemangioma of bone. Genes, Chromosomes Cancer 2015;54:565-74  Back to cited text no. 12
Floris G, Deraedt K, Samson I, Brys P, Sciot R Epithelioid hemangioma of bone: A potentially metastasizing tumor? Int J Surg Pathol 2006;14:9-15; discussion 16-20.  Back to cited text no. 13
O’Connell JX, Nielsen GP, Rosenberg AE Epithelioid vascular tumors of bone: A review and proposal of a classification scheme. Adv Anat Pathol 2001;8:74-82.  Back to cited text no. 14
Deshpande V, Rosenberg AE, O’Connell JX, Nielsen GP Epithelioid angiosarcoma of the bone: A series of 10 cases. Am J Surg Pathol 2003;27:709-16.  Back to cited text no. 15
Nielsen GP, Srivastava A, Kattapuram S, Deshpande V, O’Connell JX, Mangham CD, et al. Epithelioid hemangioma of bone revisited: A study of 50 cases. Am J Surg Pathol 2009;33:270-7.  Back to cited text no. 16
Tsikopoulos K, Perdikakis E, Georgiannos D, Bisbinas I Epithelioid hemangioma of the scapula treated with chemoembolization and microwave ablation: A case report. Acta Orthop Traumatol Turc 2018;52:157-61.  Back to cited text no. 17
Errani C, Zhang L, Panicek DM, Healey JH, Antonescu CR. Epithelioid hemangioma of bone and soft tissue: A reappraisal of a controversial entity. Clin Orthop Relat Res 2012;470:1498-506.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]


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