case_images: PMC5019921_01
case_images: CT scan shows marked enlargement of the spleen and heterogeneous composition and demonstrates low attenuation masses.
case_images: CRIS2016-4173060.001.jpg
case_images: PMC5019921_01_CRIS2016-4173060.001.jpg
case_images: fig1
case_images: A computed tomography with contrast of the abdomen and pelvis demonstrated marked splenomegaly that is heterogeneous with multiple low attenuation masses (Figure 1).
case_images: PET scan demonstrating an enlarged spleen with hypermetabolism.
case_images: CRIS2016-4173060.002.jpg
case_images: PMC5019921_01_CRIS2016-4173060.002.jpg
case_images: fig2
case_images: In addition the patient underwent a positron emission tomography scan which showed splenic hypermetabolism (Figure 2).
case_images: (a) Splenectomy with distal pancreatectomy and (b) enlarged dark brown, soft, and rubbery spleen measuring 19.0 x 13.0 x 10.5 cm.
case_images: CRIS2016-4173060.003.jpg
case_images: PMC5019921_01_CRIS2016-4173060.003.jpg
case_images: fig3
case_images: Once the spleen was removed the total weight measured 1,124 grams (Figure 3).
case_images: (a) Low power magnification (4x), showing effaced splenic architecture with large areas of hemorrhage. (b) Higher power magnification (20x) showing numerous large atypical cells (arrows).
case_images: CRIS2016-4173060.004.jpg
case_images: PMC5019921_01_CRIS2016-4173060.004.jpg
case_images: fig4
case_images: The organ was sent for pathological analysis, with routine sections showing sheets and clusters of large atypical cells with vesicular chromatin and prominent nucleoli in a background of extensive necrosis, lymphodepletion, and abundant macrophages (Figure 4).
case_images: Immunohistochemistry shows the large tumor cells are positive for vascular markers (a) CD34, (b) CD31, and (c) vWF. Only small residual lymphocytes are positive for (d) CD45, and the large atypical cells are negative.
case_images: CRIS2016-4173060.005.jpg
case_images: PMC5019921_01_CRIS2016-4173060.005.jpg
case_images: fig5
case_images: Ki-67 showed a proliferation index of 5-10% (Figure 5).