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Angiosarcoma of the Spleen: Imaging Characteristics in 12 Patients¹

¹ From the Department of Radiology, Duke University Medical Center, Erwin Rd, Box 3808, Durham, NC 27710 (W.M.T.); Departments of Radiologic Pathology (A.D.L.) and Hematopathology (N.S.A.), Armed Forces Institute of Pathology, Washington, DC; Department of Radiology and Nuclear Medicine, Uniformed Services University of the Health Sciences, Bethesda, Md (A.D.L., R.M.A); Department of Radiology, La Paz University Hospital, Madrid, Spain (L.G.); and Department of Radiology, University of Maryland Medical Center, Baltimore, Md (R.M.A.). From the 2003 RSNA Annual Meeting. Received February 18, 2004; revision requested April 26; revision received May 25; accepted June 28. Address correspondence to W.M.T. (e-mail: thomp132@mc.duke.edu).

View larger version (129K): [in a new window]   Figure 1a. Images in a 72-year-old woman with epigastric pain. (a) Resected specimen shows multiple tumor nodules in the periphery of the rim (white arrows) and a central area of tumor with necrosis and fibrosis (black arrows). (b) Transverse US image of the spleen shows a solitary mass (5 x 6 cm) with an echogenic rim (arrow) and a hypoechoic center (arrowhead). Doppler results showed this rim to be hypervascular. (c) Transverse contrast-enhanced early portal venous phase CT scan shows a solitary 6-cm mass with contrast enhancement in the rim (white arrow) and decreased attenuation in the center (arrowhead). Two enhancing masses (black arrows) are seen in the liver.

View larger version (159K): [in a new window]   Figure 1b. Images in a 72-year-old woman with epigastric pain. (a) Resected specimen shows multiple tumor nodules in the periphery of the rim (white arrows) and a central area of tumor with necrosis and fibrosis (black arrows). (b) Transverse US image of the spleen shows a solitary mass (5 x 6 cm) with an echogenic rim (arrow) and a hypoechoic center (arrowhead). Doppler results showed this rim to be hypervascular. (c) Transverse contrast-enhanced early portal venous phase CT scan shows a solitary 6-cm mass with contrast enhancement in the rim (white arrow) and decreased attenuation in the center (arrowhead). Two enhancing masses (black arrows) are seen in the liver.

View larger version (137K): [in a new window]   Figure 1c. Images in a 72-year-old woman with epigastric pain. (a) Resected specimen shows multiple tumor nodules in the periphery of the rim (white arrows) and a central area of tumor with necrosis and fibrosis (black arrows). (b) Transverse US image of the spleen shows a solitary mass (5 x 6 cm) with an echogenic rim (arrow) and a hypoechoic center (arrowhead). Doppler results showed this rim to be hypervascular. (c) Transverse contrast-enhanced early portal venous phase CT scan shows a solitary 6-cm mass with contrast enhancement in the rim (white arrow) and decreased attenuation in the center (arrowhead). Two enhancing masses (black arrows) are seen in the liver.

View larger version (121K): [in a new window]   Figure 2a. Images in a 68-year-old woman with several weeks of back pain who was proved at percutaneous biopsy to have metastases in the fourth lumbar vertebral body. (a) Resected specimen shows the tumor has grossly enlarged and completely replaced the spleen. Multiple areas of fibrosis (arrows) and necrosis are present. (b) On transverse contrast-enhanced early portal venous phase CT scan, the massively enlarged spleen (arrows) is completely replaced by tumor. Multiple areas of low attenuation ranging in size from a few millimeters to 3-4 cm are seen throughout the spleen.

View larger version (140K): [in a new window]   Figure 2b. Images in a 68-year-old woman with several weeks of back pain who was proved at percutaneous biopsy to have metastases in the fourth lumbar vertebral body. (a) Resected specimen shows the tumor has grossly enlarged and completely replaced the spleen. Multiple areas of fibrosis (arrows) and necrosis are present. (b) On transverse contrast-enhanced early portal venous phase CT scan, the massively enlarged spleen (arrows) is completely replaced by tumor. Multiple areas of low attenuation ranging in size from a few millimeters to 3-4 cm are seen throughout the spleen.

View larger version (117K): [in a new window]   Figure 3a. Images in a 62-year-old woman with upper abdominal pain. (a) Cut surface of the resected specimen shows dark areas of infarction and hemorrhage (arrows) in the periphery and central areas of a vascular tumor (arrowheads). (b) Transverse contrast-enhanced early portal venous phase CT scan shows enhancing masses (arrows) in the center of a large spleen. The periphery of the spleen shows low attenuation, suggesting infarction and necrosis. The liver contains multiple hypervascular metastases (arrowheads) ranging in size from a few millimeters to 1-2 cm. (c) On transverse precontrast T1-weighted gradient-echo MR image (repetition time msec/echo time msec, 8.1/4.2), mass replaces spleen with patchy irregular areas of high signal intensity. The liver has diffuse areas of high signal intensity similar to the spleen. (d) Transverse postcontrast T1-weighted gradient-echo MR image (8.1/4.2) shows intense contrast enhancement of the center (arrows) of the lesion and of multiple metastatic nodules in the liver.

View larger version (129K): [in a new window]   Figure 3b. Images in a 62-year-old woman with upper abdominal pain. (a) Cut surface of the resected specimen shows dark areas of infarction and hemorrhage (arrows) in the periphery and central areas of a vascular tumor (arrowheads). (b) Transverse contrast-enhanced early portal venous phase CT scan shows enhancing masses (arrows) in the center of a large spleen. The periphery of the spleen shows low attenuation, suggesting infarction and necrosis. The liver contains multiple hypervascular metastases (arrowheads) ranging in size from a few millimeters to 1-2 cm. (c) On transverse precontrast T1-weighted gradient-echo MR image (repetition time msec/echo time msec, 8.1/4.2), mass replaces spleen with patchy irregular areas of high signal intensity. The liver has diffuse areas of high signal intensity similar to the spleen. (d) Transverse postcontrast T1-weighted gradient-echo MR image (8.1/4.2) shows intense contrast enhancement of the center (arrows) of the lesion and of multiple metastatic nodules in the liver.

View larger version (132K): [in a new window]   Figure 3c. Images in a 62-year-old woman with upper abdominal pain. (a) Cut surface of the resected specimen shows dark areas of infarction and hemorrhage (arrows) in the periphery and central areas of a vascular tumor (arrowheads). (b) Transverse contrast-enhanced early portal venous phase CT scan shows enhancing masses (arrows) in the center of a large spleen. The periphery of the spleen shows low attenuation, suggesting infarction and necrosis. The liver contains multiple hypervascular metastases (arrowheads) ranging in size from a few millimeters to 1-2 cm. (c) On transverse precontrast T1-weighted gradient-echo MR image (repetition time msec/echo time msec, 8.1/4.2), mass replaces spleen with patchy irregular areas of high signal intensity. The liver has diffuse areas of high signal intensity similar to the spleen. (d) Transverse postcontrast T1-weighted gradient-echo MR image (8.1/4.2) shows intense contrast enhancement of the center (arrows) of the lesion and of multiple metastatic nodules in the liver.

View larger version (141K): [in a new window]   Figure 3d. Images in a 62-year-old woman with upper abdominal pain. (a) Cut surface of the resected specimen shows dark areas of infarction and hemorrhage (arrows) in the periphery and central areas of a vascular tumor (arrowheads). (b) Transverse contrast-enhanced early portal venous phase CT scan shows enhancing masses (arrows) in the center of a large spleen. The periphery of the spleen shows low attenuation, suggesting infarction and necrosis. The liver contains multiple hypervascular metastases (arrowheads) ranging in size from a few millimeters to 1-2 cm. (c) On transverse precontrast T1-weighted gradient-echo MR image (repetition time msec/echo time msec, 8.1/4.2), mass replaces spleen with patchy irregular areas of high signal intensity. The liver has diffuse areas of high signal intensity similar to the spleen. (d) Transverse postcontrast T1-weighted gradient-echo MR image (8.1/4.2) shows intense contrast enhancement of the center (arrows) of the lesion and of multiple metastatic nodules in the liver.

View larger version (171K): [in a new window]   Figure 4a. Transverse CT scans in a 58-year-old man with faintness and loss of consciousness. (a) Precontrast scan shows high attenuation (arrowhead) within the spleen that was later confirmed to be blood. There is free fluid around the liver because of intraperitoneal hemorrhage (black arrows). An accessory spleen (white arrow) is demonstrated medial to the spleen. (b) Postcontrast scan in the late portal venous phase shows minimal contrast enhancement of the splenic mass. There are areas of decreased enhancement due to bleeding and/or necrosis (black arrow) and a small amount of hemorrhage (white arrow) adjacent to the spleen.

View larger version (166K): [in a new window]   Figure 4b. Transverse CT scans in a 58-year-old man with faintness and loss of consciousness. (a) Precontrast scan shows high attenuation (arrowhead) within the spleen that was later confirmed to be blood. There is free fluid around the liver because of intraperitoneal hemorrhage (black arrows). An accessory spleen (white arrow) is demonstrated medial to the spleen. (b) Postcontrast scan in the late portal venous phase shows minimal contrast enhancement of the splenic mass. There are areas of decreased enhancement due to bleeding and/or necrosis (black arrow) and a small amount of hemorrhage (white arrow) adjacent to the spleen.

View larger version (123K): [in a new window]   Figure 5. Transverse unenhanced CT scan in a 36-year-old man with a 3-month history of abdominal discomfort demonstrates a vague mass (arrow) in the posterior-medial aspect of the spleen, with multiple small calcifications.

View larger version (153K): [in a new window]   Figure 6a. (a, b) Transverse postcontrast early portal venous phase CT scans in a 69-year-old man with back pain and a pathologic fracture of the 12th thoracic vertebral body. Scans demonstrate multiple hypoattenuating splenic masses (arrows), ranging in size from 1-2 mm to 3-4 cm, in a normal-sized spleen. Attempted CT-guided biopsy of the spleen was complicated with severe splenic hemorrhage, which required emergency splenectomy.

View larger version (150K): [in a new window]   Figure 6b. (a, b) Transverse postcontrast early portal venous phase CT scans in a 69-year-old man with back pain and a pathologic fracture of the 12th thoracic vertebral body. Scans demonstrate multiple hypoattenuating splenic masses (arrows), ranging in size from 1-2 mm to 3-4 cm, in a normal-sized spleen. Attempted CT-guided biopsy of the spleen was complicated with severe splenic hemorrhage, which required emergency splenectomy.

View larger version (121K): [in a new window]   Figure 7a. Transverse MR images in a 53-year-old man with upper abdominal pain. (a) T1-weighted gradient-recalled-echo image (225/4.2, 90° flip angle) demonstrates a 6-cm mass in the spleen, with low signal intensity in the periphery (arrows) of the lesion and high signal intensity in the center. There are multiple lesions in the liver with similar characteristics to that in the spleen. (b) T2-weighted fast spin-echo image (2729/183) shows high signal intensity in the periphery and most of the center of the lesions (compared with signal intensity of the normal spleen). There is some inhomogeneity in the center of the lesion. Similar findings are seen in the multiple liver lesions. (c) Precontrast and (d) postcontrast gradient-recalled-echo images (220/1.3, 90° flip angle) obtained at a slightly different level than a and b. The contrast enhancement is in the portal venous phase, and marked enhancement of the periphery of the splenic lesion and the two hepatic lesions is seen. The low signal intensity in the center of the lesions is caused by necrosis (proved at pathologic examination).

View larger version (159K): [in a new window]   Figure 7b. Transverse MR images in a 53-year-old man with upper abdominal pain. (a) T1-weighted gradient-recalled-echo image (225/4.2, 90° flip angle) demonstrates a 6-cm mass in the spleen, with low signal intensity in the periphery (arrows) of the lesion and high signal intensity in the center. There are multiple lesions in the liver with similar characteristics to that in the spleen. (b) T2-weighted fast spin-echo image (2729/183) shows high signal intensity in the periphery and most of the center of the lesions (compared with signal intensity of the normal spleen). There is some inhomogeneity in the center of the lesion. Similar findings are seen in the multiple liver lesions. (c) Precontrast and (d) postcontrast gradient-recalled-echo images (220/1.3, 90° flip angle) obtained at a slightly different level than a and b. The contrast enhancement is in the portal venous phase, and marked enhancement of the periphery of the splenic lesion and the two hepatic lesions is seen. The low signal intensity in the center of the lesions is caused by necrosis (proved at pathologic examination).

View larger version (132K): [in a new window]   Figure 7c. Transverse MR images in a 53-year-old man with upper abdominal pain. (a) T1-weighted gradient-recalled-echo image (225/4.2, 90° flip angle) demonstrates a 6-cm mass in the spleen, with low signal intensity in the periphery (arrows) of the lesion and high signal intensity in the center. There are multiple lesions in the liver with similar characteristics to that in the spleen. (b) T2-weighted fast spin-echo image (2729/183) shows high signal intensity in the periphery and most of the center of the lesions (compared with signal intensity of the normal spleen). There is some inhomogeneity in the center of the lesion. Similar findings are seen in the multiple liver lesions. (c) Precontrast and (d) postcontrast gradient-recalled-echo images (220/1.3, 90° flip angle) obtained at a slightly different level than a and b. The contrast enhancement is in the portal venous phase, and marked enhancement of the periphery of the splenic lesion and the two hepatic lesions is seen. The low signal intensity in the center of the lesions is caused by necrosis (proved at pathologic examination).

View larger version (131K): [in a new window]   Figure 7d. Transverse MR images in a 53-year-old man with upper abdominal pain. (a) T1-weighted gradient-recalled-echo image (225/4.2, 90° flip angle) demonstrates a 6-cm mass in the spleen, with low signal intensity in the periphery (arrows) of the lesion and high signal intensity in the center. There are multiple lesions in the liver with similar characteristics to that in the spleen. (b) T2-weighted fast spin-echo image (2729/183) shows high signal intensity in the periphery and most of the center of the lesions (compared with signal intensity of the normal spleen). There is some inhomogeneity in the center of the lesion. Similar findings are seen in the multiple liver lesions. (c) Precontrast and (d) postcontrast gradient-recalled-echo images (220/1.3, 90° flip angle) obtained at a slightly different level than a and b. The contrast enhancement is in the portal venous phase, and marked enhancement of the periphery of the splenic lesion and the two hepatic lesions is seen. The low signal intensity in the center of the lesions is caused by necrosis (proved at pathologic examination).