case_images: PMC11750646_01
case_images: The imaging data of this patient's disease progression. (A) In August 2022, chest CT showed a 2.8cmx2.1cm mass in the upper lobe of the left lung. (B) In August 2022, on chest CT, the red arrow refers to the left fourth rib lesion. (C) In May 2023, PET/CT showed multiple metastases throughout the body. (D) In May 2023, Axial T1 MRI contrast of the pelvis shows metastases in and around the left iliac bone, measuring 9.6 cmx6.4 cm, with a visible fluid-fluid level within it (arrow). (E) In May 2023, coronal T1 MRI enhancement showed a subcutaneous nodule on the right buttock, approximately 1.1 cmx1.0cm in size (arrow). (F) In October 2023, brain CT showed large patches of hyperdense opacities in the right basal ganglia and frontoparial lobe, with the maximum level ranging from about 6.2cmx5.0cm (arrow). (G) In October 2023, chest CT showed a soft tissue nodular opacity at the base of the left pleura, about 1.6 cmx2.6cm in size (arrow).
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case_images: PMC11750646_01_fonc-14-1420597-g001.jpg
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case_images: August 7, 2022, for no improvement. A subsequent CT scan showed an enlargement of the pulmonary lesion to 2.8cmx2.1cm, with no significant changes in the left 4th rib lesion, raising suspicions of a tumorous condition ( Figures 1A, B ). The Magnetic resonance imaging (MRI) on May 21, 2023, revealed bone destruction in the left iliac bone, accompanied by a surrounding mixed solid and cystic mass measuring approximately 9.0cm x 6.4cm, exhibiting a fluid-fluid level ( Figure 1D ). Additionally, a subcutaneous nodule measuring 1.1cmx1.0cm was identified in the right buttock, raising concerns about metastases from lung or rib tumors ( Figure 1E ). A subcutaneous nodule in the right buttock showed increased metabolic activity, again indicating potential metastasis from lung or rib tumors ( Figure 1C ). A CT examination revealed the presence of metastatic lesions in the brain and pleura ( Figures 1F, G ).
case_images: Lung AFH stained with hematoxylin and eosin (A). (A1) There is a distinct fibrous pseudocapsule and pericapsular sleeve of lymphoplasma cells around the tumor nodule (x40). Inset: Lymphoplasma cells densely distributed around the fibrosham capsule (x400), the scale represents 20mum. (B1) A pseudohematous hemorrhagic sac cavity may be seen within the tumor (x100). Slit hemorrhagic areas with hemosiderin deposition and lymphocyte plasmacytic infiltration may be seen within the tumor (x200). (D1) Tumors are mainly composed of fat spindle cells and ovoid cells (x200). (E1) There is more plasma cell infiltration within the tumor (x400). (F1) A mitotic image is visible within the tumor, indicated by arrows (x400). (B) Immunohistochemical staining and fluorescence in situ hybridization of AFH. Tumor cells were positive for Vimentin, CD99, CD68, Desmin (A2-D2), and negative for S-100 (E2). (F2) Fluorescence in situ hybridization analysis revealed fusion of EWSR1 and CREB1. The red fluorescence signal is the CREB1 (2q33) probe, and the green fluorescence signal is the EWSR1 (22q12) probe. The arrow indicates the yellow fluorescence signal, which indicates fusion of EWSR1 and CREB1. (magnification, x200 for all, tumor cells with brownish-yellow staining are immunohistochemically positive).
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case_images: PMC11750646_01_fonc-14-1420597-g002.jpg
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case_images: Multiple bleeding areas were observed within the tumor cell nests, presenting as pseudo-vascular cystic or cleft-like structures without endothelial cell lining ( Figure 2A ). Figures 2B A2-D2 ), weak positive staining (10%) for P53, negative staining for CD21, CD34, S-100 ( Figures 2B E2 ), Myoglobin, CK-P, P63, SMA, calponin, and CD21. Fluorescence in situ hybridization (FISH) analysis showed rearrangement of the EWSR1 gene with CREB1, confirming the presence of the EWSR1-CREB1 fusion gene ( Figures 2B F2 ).