case_images: PMC7346343_01
case_images: Histology photomicrograph of the excised right thigh soft tissue mass showing a malignant mesenchymal tumor with markedly pleomorphic spindle to bizarre cells exhibiting marked nuclear pleomorphism, coarse chromatin and abundant eosinophilic cytoplasm (H&E, 200x)
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case_images: PMC7346343_01_13569_2020_133_Fig1_HTML.jpg
case_images: Fig1
case_images: She underwent excision biopsy of the primary lesion at a local hospital and histopathology was suggestive of undifferentiated pleomorphic sarcoma, with 14-15 mitoses per high power field, no necrosis and FNCLCC grade II (Fig. 1).
case_images: Axial (a) and coronal (b) fused positron emission tomography:computed tomography (PET-CT) images showing 18-fluorodeoxyglucose (18-FDG) avid soft tissue nodule in the left lung upper lobe (green arrows) after completion of gemcitabine-docetaxel chemotherapy. CT scan images (c, d) in lung window showing progression of nodules in left lung upper lobe (red arrows) after 3 months of watchful waiting. 18-FDG PET-CT scan images showing reduction in size with resolution of metabolic activity of lung nodules after 3 months (e, f) (blue arrows) and 9 months (g, h) (white arrows) of treatment with pembrolizumab + pazopanib
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case_images: PMC7346343_01_13569_2020_133_Fig2_HTML.jpg
case_images: Fig2
case_images: She presented to our center at this point for further management and in view of metastatic disease, was advised doxorubicin-based chemotherapy. After discussion of the encouraging results from the phase 2 trial conducted by Tap et al. with the patient, the platelet derived growth factor receptor alpha antibody olaratumab was also added. However, response assessment done after 4 cycles of doxorubicin and olaratumab showed progressive disease and she was then switched to gemcitabine and docetaxel regimen. She received 8 cycles of gemcitabine/docetaxel and had reduction in number and size of lung nodules, suggestive of partial response (Fig. 2a, b). A CT scan of the chest was done, which revealed disease progression (Fig. 2c, d). There was significant improvement in her cough within one month of therapy and response evaluation done after 3 months of therapy showed regression of lung metastases (Fig. 2e, f). Same combination was continued with no unmanageable adverse effects, and a repeat evaluation done after 9 months of treatment showed further disease regression (Fig. 2 g, h).
case_images: Immunohistochemistry for anti-programmed death ligand-1 antibody showing membranous positivity in tumor cells (Ventana SP263 assay)
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case_images: PMC7346343_01_13569_2020_133_Fig3_HTML.jpg
case_images: Fig3
case_images: Biopsy blocks were reviewed for expression of programmed death ligand-1 (PD-L1) by IHC using the Ventana PD-L1 assay (SP263), which was positive, with a tumor cell score of 25 percent (Fig. 3).