A 75-year-old woman with a medical history of gastroesophageal reflux disease and type II diabetes presented to the hospital with a 3-month history of gradually worsening headaches, vague upper abdominal pain, and lower back pain. The patient denied fevers, night sweats, contact with sick individuals, occupational exposure to infection, bleeding, immunodeficiency, intravenous drug use, alcohol or tobacco abuse, history of malignancy, family history of genetic disorders, and international travel. Physical examination revealed a skin-colored mass protruding from the right side of her forehead, but there were no other notable abnormalities. Her diabetes was managed with diet, and the only prescription medication she was taking was esomeprazole. She was not taking anticoagulants. Initial laboratory work-up revealed anemia and profound thrombocytopenia (hemoglobin level, 9.4 g/dL; platelet count, 16 × 109/L); these were refractory to aggressive treatment, including plasmapheresis, immunosuppression with prednisolone, and numerous transfusions. Contrast material–enhanced magnetic resonance (MR) imaging of the head was performed at admission to further evaluate the patient’s headache and the mass on the patient’s forehead. Ultrasonography (US) of the abdomen was performed to evaluate the cause of abdominal pain. The discovery of liver lesions at US led us to perform contrast-enhanced computed tomography (CT) of the chest, abdomen, and pelvis. Contrast-enhanced MR imaging of the abdomen was performed to narrow the diagnostic considerations for the lesions identified at CT. Bone marrow biopsy revealed no evidence of infectious or neoplastic processes. Endoscopy and colonoscopy were performed; however, they revealed no abnormalities. Further laboratory work-up included extensive testing for parasites, fungi, bacteria, and viruses, including the human immunodeficiency virus (HIV). All of the results were negative. On the 17th day of admission, the patient became acutely unresponsive, her condition deteriorated rapidly, and she died. Unenhanced head CT was performed at the time of the patient’s acute decompensation.
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Digital Object Identifier (DOI)
Case 213, published in Radiology, v. 272, no. 3, p. 911-913, DOI: http://dx.doi.org/10.1148/radiol.14110885
Also available in UKnowledge: http://uknowledge.uky.edu/radiology_facpub/3/
Krol, John J.; Krol, Vera V.; Dawkins, Adrian; and Ganesh, Halemane S., "Case 213: Primary Spenic Angiosarcoma" (2015). Radiology Faculty Publications. 4.
Figure 1: Axial T1-weighted (repetition time msec/echo time msec, 400/17; 5-mm section thickness) contrast-enhanced MR image of the brain (10 mL of gadopentetate dimeglumine administered intravenously over 15 seconds, Magnevist; Bayer Healthcare Pharmaceuticals, Leverkusen, Germany) shows a peripherally enhancing soft-tissue lesion extending through the diploic space into the parenchyma of the right frontal lobe.
Figure 2.ppt-2015.12.21_184.108.40.206.ppt (81 kB)
Figure 2: Transverse gray-scale US image of the upper abdomen shows ill-defined heterogeneous hypoechoic lesions. Some of the lesions contain internal echoes, and some have relatively hyperechoic rims. The visualized hepatic parenchyma demonstrates diffuse heterogeneity.
Figure 3.ppt-2015.12.21_220.127.116.113.ppt (91 kB)
Figure 3: Axial contrast-enhanced CT image of the chest acquired during the arterial phase (80 mL of iohexol 300 administered at a rate of 4 mL/sec, Omnipaque 300; GE Healthcare, Milwaukee, Wis). There is no lung or breast mass.
Figure 4a.ppt-2015.12.21_18.104.22.1684.ppt (117 kB)
Figure 4a: Axial contrast-enhanced CT images of the liver during the arterial phase (80 mL of iohexol 300 administered at a rate of 4 mL/sec) show (a) a large complex low-attenuation lesion in the spleen and multiple complex low-attenuation lesions throughout the liver and (b) fatty atrophy of the pancreas, without focal mass, and no renal mass.
Figure 4b.ppt-2015.12.21_13.38.03.939.ppt (134 kB)
Figure 4b: Axial contrast-enhanced CT images of the liver during the arterial phase (80 mL of iohexol 300 administered at a rate of 4 mL/sec) show (a) a large complex low-attenuation lesion in the spleen and multiple complex low-attenuation lesions throughout the liver and (b) fatty atrophy of the pancreas, without focal mass, and no renal mass.
Figure 5.ppt-2015.12.21_22.214.171.1240.ppt (99 kB)
Figure 5: Representative axial FDG (13.9 mCi, infused at a blood glucose level of 113 mg/dL and incubated for 62 minutes) PET/CT image through the upper abdomen shows low-grade uptake of the radiotracer in the periphery of the cystic necrotic lesions within the spleen and liver.
Figure 6.ppt-2015.12.21_126.96.36.1993.ppt (105 kB)
Figure 6: Coronal T2-weighted (900/78, 6-mm section thickness) ultrafast spin-echo MR image of the abdomen (Symphony; Siemens Medical Systems, Erlangen, Germany) shows a large complex T2 heterogeneous hyperintense lesion in the spleen and multiple heterogeneous T2 hyperintense lesions throughout the liver.
Figure 7a.ppt-2015.12.21_188.8.131.529.ppt (94 kB)
Figure 7a: Axial T1-weighted (4.3/2, 2-mm section thickness) dynamic contrast-enhanced ultrafast gradient-echo MR images of the abdomen (10 mL of gadopentetate dimeglumine) obtained during the (a) early and (b) late arterial phases. Images show areas of heterogeneous enhancement of the large splenic lesion and several of the hepatic lesions (a) and an additional peripherally enhancing mass in the L1 vertebral body (b).
Figure 7b.ppt-2015.12.21_13.40.03.607.ppt (90 kB)
Figure 7b: Axial T1-weighted (4.3/2, 2-mm section thickness) dynamic contrast-enhanced ultrafast gradient-echo MR images of the abdomen (10 mL of gadopentetate dimeglumine) obtained during the (a) early and (b) late arterial phases. Images show areas of heterogeneous enhancement of the large splenic lesion and several of the hepatic lesions (a) and an additional peripherally enhancing mass in the L1 vertebral body (b).
Figure 8.ppt-2015.12.21_184.108.40.2064.ppt (124 kB)
Figure 8: Unenhanced axial CT image of the head at the time of acute mental status changes and clinical deterioiration shows acute subdural and epidural hemorrhage causing mass effect in the brain and subfalcine herniation.
Figure 9.ppt-2015.12.21_220.127.116.111.ppt (198 kB)
Figure 9: Gross pathologic image of the liver shows parenchymal involvement by ill-defined hemorrhagic and necrotic foci of angiosarcoma.
Figure 10.ppt-2015.12.21_18.104.22.168.ppt (213 kB)
Figure 10: Gross pathologic image of the spleen shows a markedly distorted organ with one dominant lesion and numerous surrounding cystic, necrotic, and hemorrhagic lesions.
Figure 11.ppt-2015.12.21_22.214.171.1248.ppt (252 kB)
Figure 11: Hematoxylin-eosin stained slice of the spleen (original magnification, ×20) shows an area of haphazard anastomosis of poorly formed vascular channels adjacent to an area of hemorrhage and necrosis. The vascular channels are lined by highly pleomorphic epithelioid cells of high nuclear grade.