J Korean Med Sci.  2004 Jun;19(3):484-486. 10.3346/jkms.2004.19.3.484.

A Case of Hypertrophic Osteoarthropathy Associated with Epithelioid Hemangioendothelioma

• Kim JH
• Lee EB
• Kim S
• Kang HW
• Suh JW
• Yoon WJ
• Kim SH
• Kang EH
• Im CH
• Song YW
• Lee HS

• KMID: 1786835
• DOI: http://doi.org/10.3346/jkms.2004.19.3.484

Abstract

Epithelioid hemangioendothelioma is a rare vascular tumor, which occurs in the lung, liver, bone, and soft tissue. Hypertrophic osteoarthropathy is a syndrome characterized by subperiosteal new bone formation, joint effusion and clubbing, and may be associated with cyanotic heart disease, chronic pulmonary disease, liver disease, and other miscellaneous diseases. The activation of endothelium and platelets has been suggested to be involved in the development of hypertrophic osteoarthropathy. We report a rare case of hypertrophic osteoarthropathy, which developed in association with hepatic epithelioid hemangioendothelioma with pulmonary metastasis. We also discuss the role of vascular endothelial growth factor in its pathogenesis.

Keyword

Hemangioendothelioma, Epithelioid; Osteoarthropathy, Secondary Hypertrophic; Vascular Endothelial Growth Factor

MeSH Terms

Adult
Biopsy
Blood Platelets/metabolism
Endothelium/metabolism
Femur/radiography
Hemangioendothelioma, Epithelioid/*complications/*pathology
Human
Lung/pathology
Lung Neoplasms/*complications/pathology
Male
Osteoarthropathy, Secondary Hypertrophic/*complications/*pathology
Vascular Endothelial Growth Factor A/metabolism
Vascular Neoplasms/*diagnosis/pathology

Figure

• 

  Fig. 1 Bone scan showing symmetric, diffuse cortical uptake around the diaphysis in both tibias and femurs. The uptake of the right ribs is attributed to tumor invasion.

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  Fig. 2 Plain radiograph of femur showing periosteal reaction on the medial and lateral aspects (arrow heads).

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  Fig. 3 Specimen from a lung biopsy. (A) Pleomorphic polygonal cells with intracellular vascular lumina containing red blood cells (H&E, ×400). (B) Tumor cells are strongly immunostained (brown color) with antibody to vascular endothelial growth factor (Hematoxylin basal stain, ×400).

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