Soft tissue hemangioma of the right upper extremity with intraosseous extension and secondary intravascular papillary endothelial hyperplasia

Abstract

Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson’s tumor, is an uncommon exuberant form of organizing thrombus that may occur within a vessel, vascular tumor, or hematoma and may change the imaging appearance to mimic an aggressive process. It must be distinguished pathologically from angiosarcoma. They have been most commonly reported within superficial soft tissue tumors, and rapid growth and effect on bone are rarely described. We present a case of a patient with a soft tissue hemangioma with IPEH with intraosseous extension that presented with a pathologic fracture of her right humerus with an aggressive appearing osseous lesion. CT and MRI demonstrated a multifocal ill-defined soft tissue mass throughout the right upper extremity with underlying cortical tunneling and scalloping of the proximal humerus. Similar imaging findings were also present in the distal humerus and ipsilateral scapula and evolved during her hospitalization. Following percutaneous biopsy revealing hemangioma with features of papillary endothelial hyperplasia with intraosseous extension, the patient died in the ICU secondary to unrelated septic shock. Diagnosis was confirmed at autopsy. Primary and secondary IPEH have been generally characterized as well-defined solitary masses, most often in the superficial soft tissues. This case of a deep soft tissue hemangioma with type II IPEH, intraosseous extension, and imaging findings of regional multicompartmental involvement is very unusual. Reporting of this case in the literature should be beneficial for pathologic correlation with similar confounding masses as well as propose a possible mechanism for intraosseous extension of soft tissue hemangiomas.