Hepatic angiosarcoma

Case contributed by Dr Bruno Di Muzio

Presentation

Right upper quadrant pain.

Patient Data

Age: 45 years
Gender: Female
CT

CT Chest, Abdomen, and Pelvis

There is a new focal liver lesion in segment VII of the liver.  This is of mixed density with a cystic/fluid/water density component anteriorly measuring 2.3 cm, and slightly denser components inferiorly.  There is an impression of some enhancement and a nodular enhancement inferiorly. There is a 1.7 cm area of solid hyperenhancement in segment VIII.  A further area of solid enhancement is visible in the periphery of segment VI, measuring 11 cm, in a peripheral location. A small circumscribed low-density lesion in segment IVb, 6mm
There is an area of dense calcification in segment 2 anteriorly, as seen previously.

The portal vein is patent.  The biliary tree is not dilated.
Renal cysts.  Otherwise, kidneys, spleen, adrenal glands, pancreas are normal.  There is no free fluid, fluid collection, lymphadenopathy or mass in the abdomen or pelvis.

The lung grafts (not shown) are satisfactory without any evidence of consolidation or infiltrate, cavitation, or mass.  Pleural spaces clear.  Heart size borderline.  No pericardial effusion.  The visible bones are normal in appearance.
Conclusion:  Indeterminate liver lesions. The lesion in segment VII has mixed solid and cystic appearances and abnormal enhancement. Tumor is a possibility but an abscess should be considered.  MRI liver could be performed.

Ultrasound

US liver (selected images)

Ultrasound has confirmed the multiple new liver lesions to be solid and mostly hypoechoic. The largest one is in segment VII and has heterogeneous echotexture with some more echogenic component. Internal vascularity has been demonstrated in most of them. The features are those of malignancy. Core-biopsy recommended. 

Case Discussion

This patient has received bilateral lung transplants 5 years prior and has been chronically immunosuppressed. Considerations for atypical infection and PTLD were raised. Metastasis from an unknown primary would be a less likely consideration. 

One of the new liver lesions was biopsied using ultrasound:

Macroscopy:  Labeled "Liver lesion core biopsy". Tan core biopsy 20mm.
Microscopy: The sections of the core biopsy show hepatic parenchyma with preserved architecture. The sinusoids are expanded by atypical cells which track within the existing sinusoidal spaces without marked destruction of the adjacent hepatic plates. The tumor cells contain moderately pleomorphic, enlarged and irregular ovoid nuclei with finely granular chromatin and occasional nucleoli. Occasional tumor cells show vacuolisation fo the cytoplasm and occasional rudimentary intracytoplasmic lumina containing eosinophilic globules. Numerous mitotic figures, including atypical forms, are present. Within the region of the tumor, lobular and intra-sinusoidal clusters of small mature CD3 positive T lymphocytes are present.  Immunohistochemically the tumor cells show strong reactivity for CD31. No reactivity for CD68, CD20, Pax5, CD3 or CD5, EBV-LMP or HHV8 is seen within tumor cells.  There is no background myxoid matrix.
Conclusion: Liver lesion, core biopsy: Angiosarcoma.

Although a rare malignancy, hepatic angiosarcoma is still the third most common primary liver tumor

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Case information

rID: 65476
Published: 10th Jan 2019
Last edited: 7th Mar 2020
Inclusion in quiz mode: Included

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