Angiomatous Neoplasms of the Skeletal System
• For clinical classification purposes it suffices to recognize a benign (hemangioma) and malignant (angiosarcoma) side of the spectrum with hemangioendothelioma as a malignant intermediate form.
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Angiomatous Neoplasms of the Skeletal System Agustinus S. Suhardja, Sophie Verbeke and Johan L. Bloem
Contents
K ey P oi n t s 19.1
Introduction 365
19.2
Classifications 366
••
19.3
Biological and Clinical Behavior 366 19.3.1 Hemangiomas 366 19.3.2 Angiosarcomas 367 19.3.3 Hemangioendotheliomas 367 19.4
Radiological and Pathological Manifestation 367 19.4.1 Hemangiomas 367 19.4.2 Angiosarcomas 370 19.4.3 Hemangioendotheliomas 371 19.5
•• •• ••
Conclusion 372 References 372
•• •• ••
A. S. Suhardja, MD, MSc Department of Radiology, C2S, Leiden University Medical Center, Albinusdreef 2, P.O. Box 9600, 2300 RC Leiden, The Netherlands S. Verbeke, MD Department of Pathology, C2S, Leiden University Medical Center, Albinusdreef 2, P.O. Box 9600, 2300 RC Leiden, The Netherlands J. L. Bloem, MD Department of Radiology, C2S, Leiden University Medical Center, Albinusdreef 2, P.O. Box 9600, 2300 RC Leiden, The Netherlands
For clinical classification purposes it suffices to recognize a benign (hemangioma) and malignant (angiosarcoma) side of the spectrum with hemangioendothelioma as a malignant intermediate form. A radiological diagnosis of angiosarcoma should be considered when multiple lesions are seen in one region. Histologically angiosarcoma looks like metastasis. A radiological suspicion for angiosarcoma should trigger the use of specific vascular markers by the pathologist. Angiosarcoma typically presents as an aggressive-looking osteolytic lesion, or lesions without periosteal reaction. Angiosarcma may present as a well-defined lesion with geographic destruction of bone, resembling fibrous dysplasia. Vertebral hemangiomas with a marked vascular component, as opposed to a marked fatty component, may cause severe symptoms.
19.1
Introduction The diversity of the radiological and pathological manifestations of skeletal angiomatous neoplasms has challenged radiologists and pathologists for decades to provide prognosis-based classifications, to understand their biological behavior, as well as to develop a simple but reliable method in establishing diagnosis of these protean lesions. These neoplasms, which range from the incidentally prevalent hemangiomas to the elusive but
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A. S. Suhardja, S. Verbeke and J. L. Bloem
clinically significant angiosarcomas, can have a seemingly innocuous appearance; however, their exclusion from differential diagnosis could potentially lead to deleterious or even catastrophic consequences for the patient. Although our understanding of the biological and clinical behavior of these neoplasms is still rudimentary, recent advancement in the classifications of these neoplasms can provide a better understanding of the radiological and pathological manifestations of these tumors which can lead to better diagnosis and treatment. This chapter summarizes our current understanding of the classifications, biological and clinical behavior, as well as radiological and pathological manifestations of these neoplasms.
19.2
Classifications While classification of
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