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DOI: 10.1148/rg.244045006
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Right arrow Gastrointestinal Radiology

From the Archives of the AFIP

Primary Vascular Neoplasms of the Spleen: Radiologic-Pathologic Correlation1

Robert M. Abbott, MD, Angela D. Levy, LTC, MC, USA, Nadine S. Aguilera, MD, Luis Gorospe, MD and William M. Thompson, MD

1 The opinions and assertions contained herein are the private views of the authors and are not to be construed as official nor as reflecting the views of the Departments of the Army or Defense.



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Figure 1.  Normal spleen. Drawing depicts normal splenic architecture. A branching network of trabeculae (black arrows) extends from the inner aspect of the capsule (arrowheads). Primary and secondary lymphoid follicles of the white pulp (white arrow) are scattered throughout communicating compartments of red pulp.

 


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Figure 2.  Normal spleen. Photomicrograph (original magnification, x10; hematoxylin-eosin [H-E] stain) shows a trabecular branch (arrow) arising from the capsule (*) and extending into normal splenic red pulp.

 


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Figure 3a.  Normal splenic red pulp. (a) Photomicrograph (original magnification, x20; H-E stain) shows normal red pulp (*) and T cells in the periarteriolar lymphoid sheath (arrows). (b) Photomicrograph (original magnification, x40; H-E stain) shows splenic sinuses (S) lined by tapered endothelial cells. The splenic cords (C) may contain erythrocytes, macrophages, and lymphoid cells.

 


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Figure 3b.  Normal splenic red pulp. (a) Photomicrograph (original magnification, x20; H-E stain) shows normal red pulp (*) and T cells in the periarteriolar lymphoid sheath (arrows). (b) Photomicrograph (original magnification, x40; H-E stain) shows splenic sinuses (S) lined by tapered endothelial cells. The splenic cords (C) may contain erythrocytes, macrophages, and lymphoid cells.

 


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Figure 4.  Normal splenic white pulp. Photomicrograph (original magnification, x40; H-E stain) shows a secondary follicle from splenic white pulp. The germinal center (*) is surrounded by B lymphocytes in the mantle zone (mz) and marginal zone (mrz).

 


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Figure 5.  Transverse sonogram of the normal spleen shows a diffusely homogeneous mid-level echotexture throughout the spleen.

 


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Figure 6a.  Contrast-enhanced CT images of the normal spleen show a mottled pattern of enhancement in the early portal venous phase (a), homogeneous enhancement during the portal venous phase (b), and washout of contrast material during the equilibrium phase of contrast enhancement (c).

 


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Figure 6b.  Contrast-enhanced CT images of the normal spleen show a mottled pattern of enhancement in the early portal venous phase (a), homogeneous enhancement during the portal venous phase (b), and washout of contrast material during the equilibrium phase of contrast enhancement (c).

 


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Figure 6c.  Contrast-enhanced CT images of the normal spleen show a mottled pattern of enhancement in the early portal venous phase (a), homogeneous enhancement during the portal venous phase (b), and washout of contrast material during the equilibrium phase of contrast enhancement (c).

 


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Figure 7a.  MR imaging appearance of the normal spleen. (a) Gadolinium-enhanced fast spoiled gradient-echo image obtained during the arterial phase shows a mottled pattern of enhancement throughout the spleen. (b) Gadolinium-enhanced T1-weighted image obtained during the portal venous phase shows uniform high signal intensity throughout the spleen. (c) Fast spin-echo T2-weighted image shows high signal intensity throughout the spleen.

 


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Figure 7b.  MR imaging appearance of the normal spleen. (a) Gadolinium-enhanced fast spoiled gradient-echo image obtained during the arterial phase shows a mottled pattern of enhancement throughout the spleen. (b) Gadolinium-enhanced T1-weighted image obtained during the portal venous phase shows uniform high signal intensity throughout the spleen. (c) Fast spin-echo T2-weighted image shows high signal intensity throughout the spleen.

 


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Figure 7c.  MR imaging appearance of the normal spleen. (a) Gadolinium-enhanced fast spoiled gradient-echo image obtained during the arterial phase shows a mottled pattern of enhancement throughout the spleen. (b) Gadolinium-enhanced T1-weighted image obtained during the portal venous phase shows uniform high signal intensity throughout the spleen. (c) Fast spin-echo T2-weighted image shows high signal intensity throughout the spleen.

 


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Figure 8a.  Splenic hemangioma. (a) Photomicrograph (original magnification, x4; H-E stain) shows a solitary, nonencapsulated splenic hemangioma (arrows) that is well demarcated from the adjacent normal spleen. The hemangioma is composed of multiple blood-filled spaces. (b) Photomicrograph (original magnification, x40; H-E stain) shows the blood-filled spaces of the hemangioma (H) lined by flat endothelial cells (arrows).

 


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Figure 8b.  Splenic hemangioma. (a) Photomicrograph (original magnification, x4; H-E stain) shows a solitary, nonencapsulated splenic hemangioma (arrows) that is well demarcated from the adjacent normal spleen. The hemangioma is composed of multiple blood-filled spaces. (b) Photomicrograph (original magnification, x40; H-E stain) shows the blood-filled spaces of the hemangioma (H) lined by flat endothelial cells (arrows).

 


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Figure 9a.  Splenic hemangiomatosis in a 9-year-old boy with Klippel-Trénaunay syndrome and worsening left upper quadrant pain. (a) Longitudinal sonogram of the spleen shows splenomegaly and multiple echogenic masses (arrows). (b) Axial T1-weighted MR image shows multiple subtle hypointense masses (arrow) throughout the spleen. (c) Axial T2-weighted MR image shows several high-signal-intensity masses (arrow). (d) Photograph of the cut surface of the resected spleen shows multiple spongy masses (arrow). Central fibrosis (arrowhead) is present in several of the hemangiomas.

 


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Figure 9b.  Splenic hemangiomatosis in a 9-year-old boy with Klippel-Trénaunay syndrome and worsening left upper quadrant pain. (a) Longitudinal sonogram of the spleen shows splenomegaly and multiple echogenic masses (arrows). (b) Axial T1-weighted MR image shows multiple subtle hypointense masses (arrow) throughout the spleen. (c) Axial T2-weighted MR image shows several high-signal-intensity masses (arrow). (d) Photograph of the cut surface of the resected spleen shows multiple spongy masses (arrow). Central fibrosis (arrowhead) is present in several of the hemangiomas.

 


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Figure 9c.  Splenic hemangiomatosis in a 9-year-old boy with Klippel-Trénaunay syndrome and worsening left upper quadrant pain. (a) Longitudinal sonogram of the spleen shows splenomegaly and multiple echogenic masses (arrows). (b) Axial T1-weighted MR image shows multiple subtle hypointense masses (arrow) throughout the spleen. (c) Axial T2-weighted MR image shows several high-signal-intensity masses (arrow). (d) Photograph of the cut surface of the resected spleen shows multiple spongy masses (arrow). Central fibrosis (arrowhead) is present in several of the hemangiomas.

 


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Figure 9d.  Splenic hemangiomatosis in a 9-year-old boy with Klippel-Trénaunay syndrome and worsening left upper quadrant pain. (a) Longitudinal sonogram of the spleen shows splenomegaly and multiple echogenic masses (arrows). (b) Axial T1-weighted MR image shows multiple subtle hypointense masses (arrow) throughout the spleen. (c) Axial T2-weighted MR image shows several high-signal-intensity masses (arrow). (d) Photograph of the cut surface of the resected spleen shows multiple spongy masses (arrow). Central fibrosis (arrowhead) is present in several of the hemangiomas.

 


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Figure 10a.  Splenic hemangioma in a 61-year-old woman who complained of nausea, vomiting, and epigastric pain. (a) Longitudinal sonogram of the spleen shows a well-defined hypoechoic mass containing a central, shadowing echogenic focus (arrow). (b) Contrast-enhanced CT scan obtained during the late portal venous phase shows a nearly isoattenuating mass in the splenic hilus with a central calcification (arrow). (c) Photograph of the cut surface of the resected spleen shows a heterogeneous mass with fibrous septa and a focus of central fibrosis (arrow).

 


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Figure 10b.  Splenic hemangioma in a 61-year-old woman who complained of nausea, vomiting, and epigastric pain. (a) Longitudinal sonogram of the spleen shows a well-defined hypoechoic mass containing a central, shadowing echogenic focus (arrow). (b) Contrast-enhanced CT scan obtained during the late portal venous phase shows a nearly isoattenuating mass in the splenic hilus with a central calcification (arrow). (c) Photograph of the cut surface of the resected spleen shows a heterogeneous mass with fibrous septa and a focus of central fibrosis (arrow).

 


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Figure 10c.  Splenic hemangioma in a 61-year-old woman who complained of nausea, vomiting, and epigastric pain. (a) Longitudinal sonogram of the spleen shows a well-defined hypoechoic mass containing a central, shadowing echogenic focus (arrow). (b) Contrast-enhanced CT scan obtained during the late portal venous phase shows a nearly isoattenuating mass in the splenic hilus with a central calcification (arrow). (c) Photograph of the cut surface of the resected spleen shows a heterogeneous mass with fibrous septa and a focus of central fibrosis (arrow).

 


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Figure 11a.  Multiple splenic hemangiomas in a 74-year-old asymptomatic man with a history of prostate cancer. (a) Contrast-enhanced CT scan obtained during the early portal venous phase shows multiple, densely enhancing splenic masses (arrows). (b) On a CT scan obtained during the equilibrium phase of enhancement, the hemangiomas (arrows) are isoattenuating compared with the adjacent normal spleen.

 


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Figure 11b.  Multiple splenic hemangiomas in a 74-year-old asymptomatic man with a history of prostate cancer. (a) Contrast-enhanced CT scan obtained during the early portal venous phase shows multiple, densely enhancing splenic masses (arrows). (b) On a CT scan obtained during the equilibrium phase of enhancement, the hemangiomas (arrows) are isoattenuating compared with the adjacent normal spleen.

 


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Figure 12a.  Cavernous hemangioma in a 29-year-old man with left upper quadrant pain and a palpable mass. (a) Contrast-enhanced CT scan shows a well-defined intrasplenic mass with peripheral enhancement surrounding areas of nonenhancement (*). (b) Photograph of the cut surface of the resected spleen shows a well-demarcated cavernous hemangioma with central fibrosis (*) that corresponds to the areas of CT nonenhancement.

 


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Figure 12b.  Cavernous hemangioma in a 29-year-old man with left upper quadrant pain and a palpable mass. (a) Contrast-enhanced CT scan shows a well-defined intrasplenic mass with peripheral enhancement surrounding areas of nonenhancement (*). (b) Photograph of the cut surface of the resected spleen shows a well-demarcated cavernous hemangioma with central fibrosis (*) that corresponds to the areas of CT nonenhancement.

 


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Figure 13a.  Splenic hemangioma in a 69-year-old woman who complained of left upper quadrant pain. (a) Axial T1-weighted MR image shows a large hypointense mass completely replacing the spleen. The mass contains several foci of high signal intensity (arrow) that may represent hemorrhage. (b) Coronal T2-weighted MR image shows that the mass is medium in signal intensity with linear areas of low signal intensity and a focus of high-signal-intensity hemorrhage (arrow).

 


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Figure 13b.  Splenic hemangioma in a 69-year-old woman who complained of left upper quadrant pain. (a) Axial T1-weighted MR image shows a large hypointense mass completely replacing the spleen. The mass contains several foci of high signal intensity (arrow) that may represent hemorrhage. (b) Coronal T2-weighted MR image shows that the mass is medium in signal intensity with linear areas of low signal intensity and a focus of high-signal-intensity hemorrhage (arrow).

 


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Figure 14a.  Splenic hamartoma. (a) Photomicrograph (original magnification, x10; H-E stain) shows disorganized sinuses and splenic red pulp elements (*) compared with the organized sinuses in the normal spleen (upper portion of the image). (b) Photomicrograph (original magnification, x40; H-E stain) shows an irregular, disorganized splenic sinus containing a megakaryocyte and nucleated red cells. (c) Photomicrograph (original magnification, x40; CD8 stain) shows CD8 positivity of the sinus lining cells (brown staining).

 


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Figure 14b.  Splenic hamartoma. (a) Photomicrograph (original magnification, x10; H-E stain) shows disorganized sinuses and splenic red pulp elements (*) compared with the organized sinuses in the normal spleen (upper portion of the image). (b) Photomicrograph (original magnification, x40; H-E stain) shows an irregular, disorganized splenic sinus containing a megakaryocyte and nucleated red cells. (c) Photomicrograph (original magnification, x40; CD8 stain) shows CD8 positivity of the sinus lining cells (brown staining).

 


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Figure 14c.  Splenic hamartoma. (a) Photomicrograph (original magnification, x10; H-E stain) shows disorganized sinuses and splenic red pulp elements (*) compared with the organized sinuses in the normal spleen (upper portion of the image). (b) Photomicrograph (original magnification, x40; H-E stain) shows an irregular, disorganized splenic sinus containing a megakaryocyte and nucleated red cells. (c) Photomicrograph (original magnification, x40; CD8 stain) shows CD8 positivity of the sinus lining cells (brown staining).

 


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Figure 15a.  Splenic hamartoma in an asymptomatic 4-year-old girl who was being evaluated for a urinary tract infection. (a) Transverse sonogram of the spleen shows a rounded, well-defined mixed echotexture mass. (b) Color Doppler sonogram shows hypervascularity within the mass.

 


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Figure 15b.  Splenic hamartoma in an asymptomatic 4-year-old girl who was being evaluated for a urinary tract infection. (a) Transverse sonogram of the spleen shows a rounded, well-defined mixed echotexture mass. (b) Color Doppler sonogram shows hypervascularity within the mass.

 


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Figure 16a.  Incidentally discovered splenic hamartoma in a 67-year-old man. (a) Longitudinal sonogram of the spleen shows a mass with poorly defined margins (white arrow) that bulges the splenic hilum (black arrow). (b) Contrast-enhanced CT scan shows heterogeneous enhancement of the mass (arrows).

 


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Figure 16b.  Incidentally discovered splenic hamartoma in a 67-year-old man. (a) Longitudinal sonogram of the spleen shows a mass with poorly defined margins (white arrow) that bulges the splenic hilum (black arrow). (b) Contrast-enhanced CT scan shows heterogeneous enhancement of the mass (arrows).

 


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Figure 17a.  Splenic hamartoma in a 67-year-old man with transient upper abdominal pain. (a-c) MR images show the mass to be isointense with T1-weighted pulse sequences (a), slightly hyperintense relative to the normal splenic parenchyma with T2-weighted pulse sequences (b), and homogeneously enhancing after intravenous gadolinium administration (c). (d) Photograph of the cut surface of the resected surgical specimen shows a smoothly marginated, predominantly homogeneous mass.

 


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Figure 17b.  Splenic hamartoma in a 67-year-old man with transient upper abdominal pain. (a-c) MR images show the mass to be isointense with T1-weighted pulse sequences (a), slightly hyperintense relative to the normal splenic parenchyma with T2-weighted pulse sequences (b), and homogeneously enhancing after intravenous gadolinium administration (c). (d) Photograph of the cut surface of the resected surgical specimen shows a smoothly marginated, predominantly homogeneous mass.

 


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Figure 17c.  Splenic hamartoma in a 67-year-old man with transient upper abdominal pain. (a-c) MR images show the mass to be isointense with T1-weighted pulse sequences (a), slightly hyperintense relative to the normal splenic parenchyma with T2-weighted pulse sequences (b), and homogeneously enhancing after intravenous gadolinium administration (c). (d) Photograph of the cut surface of the resected surgical specimen shows a smoothly marginated, predominantly homogeneous mass.

 


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Figure 17d.  Splenic hamartoma in a 67-year-old man with transient upper abdominal pain. (a-c) MR images show the mass to be isointense with T1-weighted pulse sequences (a), slightly hyperintense relative to the normal splenic parenchyma with T2-weighted pulse sequences (b), and homogeneously enhancing after intravenous gadolinium administration (c). (d) Photograph of the cut surface of the resected surgical specimen shows a smoothly marginated, predominantly homogeneous mass.

 


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Figure 18.  Photomicrograph (original magnification, x40; H-E stain) of a splenic lymphangioma shows thin-walled cystic spaces filled with proteinaceous fluid (*). Attenuated endothelial cells (arrow) line the cysts.

 


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Figure 19a.  (a) Splenic lymphangiomas in a 41-year-old woman who was being evaluated for ovarian torsion. Axial CT scan shows multifocal, subcapsular hypoattenuating masses. (b) Splenic lymphangiomas in a 34-year-old woman with splenic lymphangiomatosis. Axial CT scan shows innumerable hypoattenuating masses in an enlarged spleen. (c, d) Capillary lymphangioma in a 9-year-old girl with abdominal pain. (c) CT scan shows a septated cystic mass (arrows) bulging the splenic hilum. (d) Photograph of the cut surface of the resected spleen shows a solid-appearing mass.

 


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Figure 19b.  (a) Splenic lymphangiomas in a 41-year-old woman who was being evaluated for ovarian torsion. Axial CT scan shows multifocal, subcapsular hypoattenuating masses. (b) Splenic lymphangiomas in a 34-year-old woman with splenic lymphangiomatosis. Axial CT scan shows innumerable hypoattenuating masses in an enlarged spleen. (c, d) Capillary lymphangioma in a 9-year-old girl with abdominal pain. (c) CT scan shows a septated cystic mass (arrows) bulging the splenic hilum. (d) Photograph of the cut surface of the resected spleen shows a solid-appearing mass.

 


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Figure 19c.  (a) Splenic lymphangiomas in a 41-year-old woman who was being evaluated for ovarian torsion. Axial CT scan shows multifocal, subcapsular hypoattenuating masses. (b) Splenic lymphangiomas in a 34-year-old woman with splenic lymphangiomatosis. Axial CT scan shows innumerable hypoattenuating masses in an enlarged spleen. (c, d) Capillary lymphangioma in a 9-year-old girl with abdominal pain. (c) CT scan shows a septated cystic mass (arrows) bulging the splenic hilum. (d) Photograph of the cut surface of the resected spleen shows a solid-appearing mass.

 


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Figure 19d.  (a) Splenic lymphangiomas in a 41-year-old woman who was being evaluated for ovarian torsion. Axial CT scan shows multifocal, subcapsular hypoattenuating masses. (b) Splenic lymphangiomas in a 34-year-old woman with splenic lymphangiomatosis. Axial CT scan shows innumerable hypoattenuating masses in an enlarged spleen. (c, d) Capillary lymphangioma in a 9-year-old girl with abdominal pain. (c) CT scan shows a septated cystic mass (arrows) bulging the splenic hilum. (d) Photograph of the cut surface of the resected spleen shows a solid-appearing mass.

 


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Figure 20a.  Splenic lymphangioma in 70-year-old man with asymptomatic splenomegaly. (a) Contrast-enhanced CT scan obtained during the delayed phase shows multiple, circumscribed low-attenuation lesions throughout the spleen. There is calcification present (arrow). (b) Photograph of the cut surface of the resected spleen shows multiple, thin-walled cystic spaces replacing normal splenic parenchyma.

 


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Figure 20b.  Splenic lymphangioma in 70-year-old man with asymptomatic splenomegaly. (a) Contrast-enhanced CT scan obtained during the delayed phase shows multiple, circumscribed low-attenuation lesions throughout the spleen. There is calcification present (arrow). (b) Photograph of the cut surface of the resected spleen shows multiple, thin-walled cystic spaces replacing normal splenic parenchyma.

 


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Figure 21a.  Littoral cell angioma. (a) Photomicrograph (original magnification, x10; H-E stain) shows multiple blood-filled spaces (*) within a focus of littoral cell angioma. (b) Photomicrograph (original magnification, x20; H-E stain) shows papillary projections (arrow) extending into channels containing exfoliated cells. (c) Photomicrograph (original magnification, x40; H-E stain) shows hemosiderin pigment (arrow) within littoral cell angioma.

 


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Figure 21b.  Littoral cell angioma. (a) Photomicrograph (original magnification, x10; H-E stain) shows multiple blood-filled spaces (*) within a focus of littoral cell angioma. (b) Photomicrograph (original magnification, x20; H-E stain) shows papillary projections (arrow) extending into channels containing exfoliated cells. (c) Photomicrograph (original magnification, x40; H-E stain) shows hemosiderin pigment (arrow) within littoral cell angioma.

 


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Figure 21c.  Littoral cell angioma. (a) Photomicrograph (original magnification, x10; H-E stain) shows multiple blood-filled spaces (*) within a focus of littoral cell angioma. (b) Photomicrograph (original magnification, x20; H-E stain) shows papillary projections (arrow) extending into channels containing exfoliated cells. (c) Photomicrograph (original magnification, x40; H-E stain) shows hemosiderin pigment (arrow) within littoral cell angioma.

 


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Figure 22a.  Sonographic features of littoral cell angioma. (a) Longitudinal sonogram of the spleen in a 50-year-old woman with asymptomatic splenomegaly shows a heterogeneous splenic echotexture with multiple hyperechoic masses. (b) Transverse sonogram of the spleen in a 75-year-old man with asymptomatic splenomegaly shows a focal hyperechoic mass (arrow).

 


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Figure 22b.  Sonographic features of littoral cell angioma. (a) Longitudinal sonogram of the spleen in a 50-year-old woman with asymptomatic splenomegaly shows a heterogeneous splenic echotexture with multiple hyperechoic masses. (b) Transverse sonogram of the spleen in a 75-year-old man with asymptomatic splenomegaly shows a focal hyperechoic mass (arrow).

 


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Figure 23a.  Littoral cell angioma in a 55-year-old woman who was found to have splenomegaly when she was evaluated for leg swelling. (a) Contrast-enhanced CT scan obtained during the early portal venous phase shows multiple, partially confluent hypoattenuating masses in the spleen. (b) Contrast-enhanced CT scan obtained during the late portal venous phase shows that the masses homogeneously enhance and become imperceptible.

 


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Figure 23b.  Littoral cell angioma in a 55-year-old woman who was found to have splenomegaly when she was evaluated for leg swelling. (a) Contrast-enhanced CT scan obtained during the early portal venous phase shows multiple, partially confluent hypoattenuating masses in the spleen. (b) Contrast-enhanced CT scan obtained during the late portal venous phase shows that the masses homogeneously enhance and become imperceptible.

 


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Figure 24a.  Littoral cell angioma in a 39-year-old woman who was found to have splenomegaly when she was being evaluated for peptic ulcer disease. (a) Contrast-enhanced CT scan shows the enlarged spleen, which contains innumerable hypoattenuating masses. (b) Photograph of the cut surface of the resected spleen shows multiple blood-filled spaces (arrow).

 


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Figure 24b.  Littoral cell angioma in a 39-year-old woman who was found to have splenomegaly when she was being evaluated for peptic ulcer disease. (a) Contrast-enhanced CT scan shows the enlarged spleen, which contains innumerable hypoattenuating masses. (b) Photograph of the cut surface of the resected spleen shows multiple blood-filled spaces (arrow).

 


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Figure 25a.  Littoral cell angioma in a 41-year-old man who was found to have thrombocytopenia before blood donation. (a) T1-weighted MR image shows splenomegaly and multiple subtle hypointense splenic masses (arrow). (b) On a T2-weighted MR image, the masses remain hypointense (arrow), adjacent to the normally bright splenic parenchyma. (c) Photograph of the cut surface of the resected spleen shows multiple well-demarcated masses (arrows).

 


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Figure 25b.  Littoral cell angioma in a 41-year-old man who was found to have thrombocytopenia before blood donation. (a) T1-weighted MR image shows splenomegaly and multiple subtle hypointense splenic masses (arrow). (b) On a T2-weighted MR image, the masses remain hypointense (arrow), adjacent to the normally bright splenic parenchyma. (c) Photograph of the cut surface of the resected spleen shows multiple well-demarcated masses (arrows).

 


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Figure 25c.  Littoral cell angioma in a 41-year-old man who was found to have thrombocytopenia before blood donation. (a) T1-weighted MR image shows splenomegaly and multiple subtle hypointense splenic masses (arrow). (b) On a T2-weighted MR image, the masses remain hypointense (arrow), adjacent to the normally bright splenic parenchyma. (c) Photograph of the cut surface of the resected spleen shows multiple well-demarcated masses (arrows).

 


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Figure 26a.  Splenic peliosis. (a) Photomicrograph (original magnification, x4; H-E stain) shows multiple blood-filled lakes (arrows) within the splenic parenchyma. (b) Photomicrograph (original magnification, x40; H-E stain) shows the absence of an endothelial lining in peliosis.

 


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Figure 26b.  Splenic peliosis. (a) Photomicrograph (original magnification, x4; H-E stain) shows multiple blood-filled lakes (arrows) within the splenic parenchyma. (b) Photomicrograph (original magnification, x40; H-E stain) shows the absence of an endothelial lining in peliosis.

 


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Figure 27a.  Splenic peliosis in a 9-year-old boy with Down syndrome and AIDS. (a) Contrast-enhanced CT scan shows multiple small hypoattenuating lesions within the spleen (arrow). (b) Photograph of the cut surface of the resected gross specimen shows dark, blood-filled lakes (arrows) within the splenic parenchyma.

 


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Figure 27b.  Splenic peliosis in a 9-year-old boy with Down syndrome and AIDS. (a) Contrast-enhanced CT scan shows multiple small hypoattenuating lesions within the spleen (arrow). (b) Photograph of the cut surface of the resected gross specimen shows dark, blood-filled lakes (arrows) within the splenic parenchyma.

 


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Figure 28a.  Splenic angiosarcoma. (a) Photograph of the cut surface of a splenectomy specimen shows a diffusely infiltrating hemorrhagic mass replacing the normal splenic parenchyma. (b) Photomicrograph (original magnification, x2; H-E stain) shows diffusely infiltrating purple masses (arrowheads) within the splenic parenchyma. There are areas of focal hemorrhage (*). (c) Photomicrograph (original magnification, x20; H-E stain) shows focal hemorrhage (*). (d) Photomicrograph (original magnification, x40; H-E stain) shows irregular, hyperchromatic nuclear pleomorphism.

 


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Figure 28b.  Splenic angiosarcoma. (a) Photograph of the cut surface of a splenectomy specimen shows a diffusely infiltrating hemorrhagic mass replacing the normal splenic parenchyma. (b) Photomicrograph (original magnification, x2; H-E stain) shows diffusely infiltrating purple masses (arrowheads) within the splenic parenchyma. There are areas of focal hemorrhage (*). (c) Photomicrograph (original magnification, x20; H-E stain) shows focal hemorrhage (*). (d) Photomicrograph (original magnification, x40; H-E stain) shows irregular, hyperchromatic nuclear pleomorphism.

 


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Figure 28c.  Splenic angiosarcoma. (a) Photograph of the cut surface of a splenectomy specimen shows a diffusely infiltrating hemorrhagic mass replacing the normal splenic parenchyma. (b) Photomicrograph (original magnification, x2; H-E stain) shows diffusely infiltrating purple masses (arrowheads) within the splenic parenchyma. There are areas of focal hemorrhage (*). (c) Photomicrograph (original magnification, x20; H-E stain) shows focal hemorrhage (*). (d) Photomicrograph (original magnification, x40; H-E stain) shows irregular, hyperchromatic nuclear pleomorphism.

 


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Figure 28d.  Splenic angiosarcoma. (a) Photograph of the cut surface of a splenectomy specimen shows a diffusely infiltrating hemorrhagic mass replacing the normal splenic parenchyma. (b) Photomicrograph (original magnification, x2; H-E stain) shows diffusely infiltrating purple masses (arrowheads) within the splenic parenchyma. There are areas of focal hemorrhage (*). (c) Photomicrograph (original magnification, x20; H-E stain) shows focal hemorrhage (*). (d) Photomicrograph (original magnification, x40; H-E stain) shows irregular, hyperchromatic nuclear pleomorphism.

 


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Figure 29a.  Sonographic features of splenic angiosarcoma. (a) Transverse sonogram of the spleen in a 72-year-old woman who complained of left upper quadrant pain shows a well-defined mass (arrowheads) of heterogeneous echotexture. (b) Contrast-enhanced CT scan shows a rim-enhancing splenic mass (arrows) and multiple enhancing liver metastases (arrowheads). (c, d) Longitudinal gray-scale (c) and color Doppler (d) sonograms in a 62-year-old woman who presented with Kasabach-Merritt syndrome show splenomegaly with multiple hypoechoic masses (arrows in c) throughout the spleen. Color Doppler US shows increased vascularity (arrow in d) within the masses.

 


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Figure 29b.  Sonographic features of splenic angiosarcoma. (a) Transverse sonogram of the spleen in a 72-year-old woman who complained of left upper quadrant pain shows a well-defined mass (arrowheads) of heterogeneous echotexture. (b) Contrast-enhanced CT scan shows a rim-enhancing splenic mass (arrows) and multiple enhancing liver metastases (arrowheads). (c, d) Longitudinal gray-scale (c) and color Doppler (d) sonograms in a 62-year-old woman who presented with Kasabach-Merritt syndrome show splenomegaly with multiple hypoechoic masses (arrows in c) throughout the spleen. Color Doppler US shows increased vascularity (arrow in d) within the masses.

 


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Figure 29c.  Sonographic features of splenic angiosarcoma. (a) Transverse sonogram of the spleen in a 72-year-old woman who complained of left upper quadrant pain shows a well-defined mass (arrowheads) of heterogeneous echotexture. (b) Contrast-enhanced CT scan shows a rim-enhancing splenic mass (arrows) and multiple enhancing liver metastases (arrowheads). (c, d) Longitudinal gray-scale (c) and color Doppler (d) sonograms in a 62-year-old woman who presented with Kasabach-Merritt syndrome show splenomegaly with multiple hypoechoic masses (arrows in c) throughout the spleen. Color Doppler US shows increased vascularity (arrow in d) within the masses.

 


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Figure 29d.  Sonographic features of splenic angiosarcoma. (a) Transverse sonogram of the spleen in a 72-year-old woman who complained of left upper quadrant pain shows a well-defined mass (arrowheads) of heterogeneous echotexture. (b) Contrast-enhanced CT scan shows a rim-enhancing splenic mass (arrows) and multiple enhancing liver metastases (arrowheads). (c, d) Longitudinal gray-scale (c) and color Doppler (d) sonograms in a 62-year-old woman who presented with Kasabach-Merritt syndrome show splenomegaly with multiple hypoechoic masses (arrows in c) throughout the spleen. Color Doppler US shows increased vascularity (arrow in d) within the masses.

 


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Figure 30a.  Spontaneous hemorrhage within a splenic angiosarcoma in a 58-year-old man who presented with syncope. (a) Unenhanced CT scan shows focal areas of high-attenuation hemorrhage (*) within a splenic mass. (b) Contrast-enhanced CT scan shows a heterogeneously enhancing mass within the spleen.

 


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Figure 30b.  Spontaneous hemorrhage within a splenic angiosarcoma in a 58-year-old man who presented with syncope. (a) Unenhanced CT scan shows focal areas of high-attenuation hemorrhage (*) within a splenic mass. (b) Contrast-enhanced CT scan shows a heterogeneously enhancing mass within the spleen.

 


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Figure 31a.  Splenic angiosarcoma in a 36-year-old man who complained of recurrent fever, fatigue, and left upper quadrant pain. (a) Unenhanced CT scan shows focal calcifications (arrows) in an ill-defined splenic mass. (b) Contrast-enhanced CT scan shows heterogeneous enhancement of the mass containing calcification.

 


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Figure 31b.  Splenic angiosarcoma in a 36-year-old man who complained of recurrent fever, fatigue, and left upper quadrant pain. (a) Unenhanced CT scan shows focal calcifications (arrows) in an ill-defined splenic mass. (b) Contrast-enhanced CT scan shows heterogeneous enhancement of the mass containing calcification.

 


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Figure 32a.  Splenic angiosarcoma with liver metastasis in a 53-year-old man who complained of epigastric and left shoulder pain. (a) T1-weighted MR image shows a dominant splenic mass with a central area of high signal intensity, a finding consistent with hemorrhage. The liver metastases also demonstrate high-signal-intensity hemorrhage. (b) T2-weighted MR image shows mixed high signal intensity in the splenic mass and liver metastases.

 


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Figure 32b.  Splenic angiosarcoma with liver metastasis in a 53-year-old man who complained of epigastric and left shoulder pain. (a) T1-weighted MR image shows a dominant splenic mass with a central area of high signal intensity, a finding consistent with hemorrhage. The liver metastases also demonstrate high-signal-intensity hemorrhage. (b) T2-weighted MR image shows mixed high signal intensity in the splenic mass and liver metastases.

 





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