PMC10157244_01
Primary hepatic angiosarcoma (PHA) in a 42-years-old woman. (A) T2-weighted axial MRI shows multiple variable-sized lesions with bright T2 signal intensity are located in both hepatic lobes. Mass lesions of PHA reveal a markedly heterogeneous architecture, with focal areas of high intensity along with septum-like or rounded areas of low intensity (indicated by yellow arrows). (B) Some lesions show annular enhancement at the edges; large masses show ruptures and hemorrhages (indicated by yellow arrows). (C) Ultrasound shows a 16cm inhomogeneous hypoechoic lesion in the right hepatic lobe (indicated by yellow arrows). Multiple masses scatter within both hepatic lobes. (D) Contrast-enhanced ultrasound shows the lesion (indicated by yellow arrows) in ill-defined, peripheral irregular isoenhancement, and necrotic and hemorrhagic areas within the tumor. (E) After selecting a target lesion with multimodal enhanced imaging, (indicated by yellow arrows), we performed core needle biopsy (indicated by white triangles) under the guidance of contrast-enhanced ultrasound. (F) PET-CT shows multiple hypermetabolism lesions with retroperitoneal lymph node metastasis in the liver.
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On day 1 of hospitalization, initial MRI of abdomen revealed that the patient had a large (16cm), irregular, dominant mass with multiple intrahepatic lesions in her liver, as well as trace free fluid in her abdominal cavity ( Figures 1A, B ). Gray-scale ultrasound and contrast-enhanced ultrasound showed multiple masses scattered in both hepatic lobes ( Figures 1C, D ), and there were necrotic and hemorrhagic areas within the tumor. After assessing the situation with multimodal enhanced imaging, we performed percutaneous core needle biopsy for a small lesion that was active and surrounded by liver parenchyma under the guidance of contrast-enhanced ultrasound ( Figure 1E ). On day 2, positron emission tomography (PET-CT) for staging showed multiple hypermetabolism lesions with retroperitoneal lymph node metastasis in the patient's liver ( Figure 1F ).
Histopathology staining of the core needle liver biopsy specimen H&E (200x magnification) shows cells with pleomorphic nuclei and frequent mitotic figures, solid nodules of similar atypical spindled endothelial cells and focal areas of with epithelioid morphology. Immunohistochemistry (200x magnification) shows the cells to be positive for CD31, CD34, ERG, Vimentin and Ki67, but negative for CK, H&E, hematoxylin and eosin.
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Immunohistochemical examination revealed positive staining of CD31(+), CD34(+), ERG(+), CK (-), Vimentin(+), Ki67(70%+), indicating tumor cells ( Figure 2 ).
The main treatment and imaging of this case. (A) Tumor imaging stains visible under DSA before TACE. (B) No tumor staining under DSA after TACE. (C) MRI one month after TACE shows no significant tumor progression. (D) Ultrasound two months after TACE shows slightly shrunk tumor. (E) MRI after the 8th cycle of treatment shows that the onset of tumor liquefaction, and necrosis and disintegration. (F) Ultrasound clearly shows tumor liquefaction and necrosis. (G) Ultra- sound-guided percutaneous catheter punctures into the pus and necrosis at the internal drainage site of the tumor (white arrow indicates the end of the drainage catheter). (H) A large amount of brown serous pus fluid drained with percutaneous catheter from the liquefied area of the thorax and tumor. (I) MRI after thoracic and abdominal drainage. (J) MRI at 15 months after diagnosis of PHA. (K) Contrast-enhanced ultrasound image at 15 months after diagnosis of PHA shows significant shrinkage of the main lesion without enhancement and no new lesions in the liver. (L) MRI at two years after diagnosis of PHA shows that shrunk lesions, and no activity or new lesion.
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After 17 months of treatment, the patient's follow-up MRI results showed that the tumor had shrunk to 7cm with internal coagulation necrosis, no active areas in the lesions, and no liver abscess ( Figure 3 ).
Timeline of patient's clinical course.
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The patient's clinical course timeline is shown in  Figure 4 .