case_images: PMC5078781_01
case_images: (a) Sagittal view of the sinus on computerized tomography (CT) showing the complete opacification of the right sphenoid sinus which was proven to be ALCL involvement by biopsy. (b) Axial view of CT C/A/P obtained at the time of diagnosis showing the diffuse osseous metastatic spread of disease involving vertebral bodies, pelvis, and humerus. (c) Representative lung field section of the CT C/A/P in (b) showing the numerous subcentimeter nodular densities within the lung parenchyma and along the margins of the fissures and pleural margins. Patient did not have significant lymphadenopathy (not shown). (d) Representative CT lung field performed after the first dose of hyperCVAD without BV showing interval progression of lung disease as well as development of new right hilar adenopathy (not shown). (e) Representative lung field of PET-CT (level approximates that of image (d)) showing resolution on the reticulonodular opacities and decrease in size of the largest subpleural nodule in the L lung. No PET avidity noted. Not shown are persistent diffuse lytic lesions that are not PET avid.
case_images: CRIONM2016-2596423.001.jpg
case_images: PMC5078781_01_CRIONM2016-2596423.001.jpg
case_images: fig1
case_images: On initial computerized tomography imaging (CT), she was found to have a large sphenoid mass, multiple pulmonary nodules, and multiple lytic bone lesions (Figure 1). CT showed confirmed progression of her pulmonary disease (Figure 1).
case_images: PMC5078781_02
case_images: (a) Hypercellular bone marrow core biopsy demonstrating interstitial proliferation of large lymphoma cells. Hematoxylin and eosin stained bone marrow core biopsy (x100). (b) CD30 immunohistochemical stain of the bone marrow core biopsy. A subset of the large lymphoma cells are CD30 positive. Ber-H2 antibody (x200).
case_images: CRIONM2016-2596423.002.jpg
case_images: PMC5078781_02_CRIONM2016-2596423.002.jpg
case_images: fig2
case_images: CD5+ monoclonal B-cell population with lambda light chain restriction. CT of his chest, abdomen, and pelvis showed diffuse lymphadenopathy (Figure 2(a)). The bone marrow core biopsy showed a hypercellular marrow (95%) (Figure 2): 50% of the cells were large lymphoid cells positive for CD5, CD20, and dimly BCL 6 positive. Reanalysis of the bone marrow specimen revealed that approximately 10-15% of the lymphoma cells expressed CD30 by immunohistochemistry (Figure 2).
case_images: Staging NM-PET imaging after chemotherapy. (a) PET staging after 2 cycles of R-CHOP: partial anatomic response to therapy. Interval decrease in diffuse lymphadenopathy in comparison to initial CT. (b) Staging PET-CT after 5 cycles of R-CHOP, 1 cycle of R-DHAP, and IT methotrexate and cytarabine treatments. Widespread diffuse adenopathy with overall progression. (c) Staging after 2 cycles of brentuximab and topotecan. Significant decreases in size and metabolic activity of the diffuse adenopathy were observed.
case_images: CRIONM2016-2596423.003.jpg
case_images: PMC5078781_02_CRIONM2016-2596423.003.jpg
case_images: fig3
case_images: Further treatment with R-DHAP salvage chemotherapy was stopped due to continued, progressive disease (Figure 3(b)). A staging PET/CT performed after completion of the 2nd cycle of TTR showed significant improvement in adenopathy (Figure 3(c)), but he developed Grade 2 diarrhea, Grade 3 neutropenia, Grade 3 anemia, Grade 2 thrombocytopenia, and Grade 2 vertigo, with a subsequent decrease in his ECOG performance status from 1 to 2.