case_images: PMC8437221_01
case_images: (A) MR abdomen showing liver mass at diagnosis. (B) CT chest showing lung nodules after third cycle of chemotherapy. (C) MR abdomen showing new splenic lesions. (D) CT liver showing new liver nodules. (E, F) The gross and microscopic appearance of the primary tumor. (E) Grossly, the mass is well circumscribed, compressing the surrounding liver parenchyma. The mass contains both solid and cystic components. Areas of hemorrhage and necrosis are present. (F) Microscopically, the viable tumor is characterized by spindle- or stellate-shaped cells with hyperchromatic nuclei, inconspicuous nucleoli, and ill-defined cells borders, embedded in a fibrotic matrix. Note the presence of small groups of residual benign hepatocytes (arrow). (G) The lung nodule demonstrates cellular interstitial and organizing pneumonia with no evidence of a neoplasm. CT, computed tomography; MR, magnetic resonance.
case_images: po-5-po.21.00102-g001.jpg
case_images: PMC8437221_01_po-5-po.21.00102-g001.jpg
case_images: fig1
case_images: Imaging revealed a 9-cm lesion in the right lobe of her liver, which was biopsied to reveal an UESL (Fig 1A). Restaging revealed mild radiographic improvement, so she underwent surgical removal of the mass, which was notable for negative margins and 40% tumor necrosis (Figs 1E and 1F). Extensive infectious workup revealed multiple bibasilar ground glass pulmonary opacities with a halo sign, concerning for hemorrhage and/or invasive fungal infection (Fig 1B). A biopsy of the lesion revealed a cellular interstitial and organizing pneumonia, with no evidence of microbes or granulomas (Fig 1G). Chemotherapy was continued with another two cycles of IE, which revealed a decrease in size of the suspicious lung lesions except for a single right middle lobe lesion, which had increased in size along with enlarging splenic lesions (Fig 1C). The patient resumed antifungal therapy for 10 months with generally stable findings until a new liver lesion arose just outside of the surgical field (Fig 1D).
case_images: Treatment course and longitudinal TP53 ddPCR findings from cfDNA. cfDNA, cell-free DNA; ddPCR, droplet digital polymerase chain reaction; IE, ifosfamide and etoposide; VDCy, vincristine, doxorubicin, and cyclophosphamide.
case_images: po-5-po.21.00102-g002.jpg
case_images: PMC8437221_01_po-5-po.21.00102-g002.jpg
case_images: fig2
case_images: As shown in Figure 2, although detectable at diagnosis and even before surgery, subsequent samples did not reveal any detectable mutant TP53. Despite the numerous worrisome lesions, interrogation of longitudinal plasma-derived cfDNA samples for the tumor-specific TP53 deletion has remained negative, supporting that these lesions were not indicative of sarcoma recurrence now more than 18 months off therapy (Fig 2).