Disseminated Epitheloid Hemangioendothelioma Mimicking Symptoms of Systemic VasculitisHaap M. · Kötter I. · Horger M. · Thamer C. · Wehrmann M. · Häring H-U. · Kanz L. · Hartmann J.
aDepartment of Endocrinology, Metabolism, Clinical Chemistry and Vascular Medicine, bDepartment of Hematology, Oncology, Immunology, and Rheumatology, Pneumology, cDepartment of Radiology, dDepartment of Pathology, eInterdisciplinary Center for Sarcomas, University of Tübingen, Germany
Background: Epitheloid hemangioendothelioma is a rare malignant tumor which can involve bones, liver, lungs, kidneys, deep soft tissue, muscles, dermis, and central nervous system. Multifocal disease occurs in 10% of the cases. The clinical presentation results from occlusion of small blood vessels due to the disease itself or as a paraneoplastic syndrome. Case Report: We present a patient with symptoms suggesting systemic vasculitis (ESR and CRP elevated, weight loss, arthralgia, livedoid rash, and skin ulcerations) who finally was diagnosed having a disseminated epitheloid hemangioendothelioma when PET scan revealed hypermetabolic multifocal skeletal and soft tissue lesions. Discussion: Diseases mimicking systemic vasculitis and the value of PET in this setting are discussed.
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