PMC6620850_01
PET scan, initial coronal section at presentation, showing marked splenomegaly. The spleen measured approximately 20 cm in length with relatively focal areas of increased radiotracer uptake in the splenic parenchyma with max SUV 4.4, compared to background max SUV throughout the spleen ranging 2.5-3.0. Background radiotracer uptake throughout the liver was very slightly greater than of the spleen, 2.7-3.2. There was also a focus of asymmetric increased radiotracer uptake in the left parapharyngeal soft tissues with associated soft tissue thickening on CT images and maximum SUV 3.5.
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PET scan showed increased splenic and pharyngeal lymph node uptake (Figure 1).
H&E staining of the liver core biopsy shows abnormal diffuse sinusoidal lymphoid infiltration. The lymphocytes are small to intermediate in size with clear cytoplasm and devoid of azurophilic granules, 20x.
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Her liver biopsy showed infiltration by abnormal lymphocytes (Figures 2 and 3) as did her bone marrow biopsy.
Immunohistochemistery for CD3 shows the abnormal lymphocytes are negative for CD3. Rare scattered T-cells are highlighted in the background, 40x.
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Her liver biopsy showed infiltration by abnormal lymphocytes (Figures 2 and 3) as did her bone marrow biopsy.
Pet scan 4 weeks after 6 cycles of DA-EPOCH showed massive splenomegaly. The spleen measures up to 27.7 cm in length with pronounced hypermetabolic activity throughout the spleen with a maximal SUV of up to 9.8. There are new peripheral wedge-shaped areas of photopenia consistent with infarcts. There is a pronounced mass effect on the stomach and left kidney from the massive splenomegaly. There is ongoing hepatomegaly as well, with the right hepatic lobe measuring 23.1 cm in length compared to 22.0 previously. Hepatic radiotracer uptake is homogenous but decreased from what would normally be expected (the average maximum SUV is approximately 1.9). This diffusely reduced radiotracer uptake could be secondary to a sequestration of radiotracer in the spleen. The previously described area of low-grade uptake in the left parapharyngeal soft tissues now demonstrates a maximal SUV of 2.0, previously 3.5.
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Imaging also showed a new splenic infarct with worsening splenomegaly (Figure 4).
Patient underwent open splenectomy. Pathology reported a hepatosplenic T-cell lymphoma diffusedly involving a markedly enlarged spleen, 4025 grams, 29.5 x 22.0 x 10.9 cm. The capsule is tan, remarkable for adhesions and multiple defects ranging in size from 3.7 x 1.0 cm to 16.5 x 11.5 cm. The specimen is remarkable for three possible hilar nodes ranging in size from 1.2 x 0.7 x 0.5 cm to 4.5 x 2.1 x 2.0 cm. Sectioning through the spleen reveals gray-white to yellow hemorrhagic lobular cut surfaces. No definitive grossly unremarkable splenic parenchyma can be identified.
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Given her symptoms and cytopenias thought to be worsened by her splenomegaly, she underwent an open splenectomy (~9 lbs), as shown in Figure 5.