AJR 2003; 181:1432-1433
© American Roentgen Ray Society
MRI of Angiosarcoma of the Calvaria
Paul Thananopavarn,
J. Keith Smith and
Mauricio Castillo
University of North Carolina School of Medicine Chapel Hill, NC
27599-7510
A 92-year-old woman with a history of a cerebrovascular accident and a
seizure disorder presented with altered mental status. Physical examination
revealed that the patient had left-sided weakness and a large subcutaneous
mass on the right side of her forehead. The mass had developed in less than 4
months.
Conventional radiographs (not shown) showed a large expansile lytic frontal
lesion. CT revealed a large hyperdense left-sided frontal mass that involved
the calvaria and extended into the brain, with effacement of the frontal horn
of the left lateral ventricle. Subsequent MRI showed a calvarial mass that
expanded the diploetic space and extended both extracranially and
intracranially. Intracranially, there was compression of the adjacent frontal
lobe with significant mass effect and moderate midline shift. On T1-weighted
images, the affected calvaria showed decreased signal intensity, whereas the
intra- and extracalvarial lesions were isointense relative to the gray matter
(Fig. 3A). T2-weighted images
revealed a well-defined heterogeneous mass with surrounding vasogenic edema
(Fig 3B). With administration
of contrast material, the lesion showed prominent heterogeneous enhancement
(Fig. 3C).
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Fig. 3A. —92-year-old woman with poorly differentiated angiosarcoma of
frontal bone of skull that involves calvaria and extends into brain. Sagittal
T1-weighted image shows mass expanding diploetic space, extending into
extracalvarial soft tissue, and compressing frontal lobe of brain.
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Fig. 3B. —92-year-old woman with poorly differentiated angiosarcoma of
frontal bone of skull that involves calvaria and extends into brain. Axial
T2-weighted image reveals extensive vasogenic edema surrounding heterogeneous
mass and significant midline shift.
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Fig. 3C. —92-year-old woman with poorly differentiated angiosarcoma of
frontal bone of skull that involves calvaria and extends into brain. Sagittal
contrast-enhanced MRI shows well-defined heterogeneously enhancing mass.
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A core needle biopsy revealed a highly cellular neoplasm with many atypical
cells containing abundant eosinophilic or clear cytoplasm. Large areas of
necrosis and mitoses were present. Immunocytochemical staining showed positive
results for vimentin and CD31 and factor VIII antigens. These findings are
consistent with the diagnosis of a poorly differentiated epithelioid
angiosarcoma. At the request of the patient's family, only supportive care was
given, and the patient died shortly after diagnosis.
The term angiosarcoma refers to a high-grade form of hemangioendothelioma;
angiosarcoma is a rare vascular endothelial tumor of bone that occasionally
arises in the skull. These tumors are more common in men; of the 11 reported
cases of angiosarcoma in the skull, only two of the patients were women
[1]. Histologically, poorly
differentiated angiosarcomas have pleomorphic collections of atypical
epithelioid cells whose vascular origin is elucidated through the
endothelial-specific immunohistochemical staining for CD31- and factor
VIII–associated antigens
[2]. High-grade angiosarcomas
may have scattered areas of low-grade tumor and areas of necrosis, resulting
in a heterogeneous pattern of contrast-enhancement on MRI
[3]. Because of their highly
aggressive nature, malignant angiosarcomas are more likely than low-grade
hemangioendotheliomas to expand through the inner and outer tables of the
calvaria or metastasize to other bone sites or to the lung
[4].
These tumors are treated in the same manner as osteosarcomas; however, some
success has been reported in treating high-grade angiosarcomas with ablative
surgery and adjuvant radiation and chemotherapy
[4,
5]. The prognosis of
angiosarcoma is extremely poor: the 5-year survival rate is between
10–35% [2,
6]. To the best of our
knowledge, ours is the first published description of the MRI characteristics
of a poorly differentiated angiosarcoma.
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