Presentation
Left mid-forearm mass enlarging over the past year. Shortness of breath. Elevated ESR and CRP.
Patient Data
There is a large soft tissue mass at the lateral aspect of the left mid forearm, which is situated just superficial to the muscles with suspected invasion. The mass is relatively iso-intense on T1W, heterogeneously hyperintense on T2 fat sat sequences and heterogeneously enhancing on the post-contrast exam with some non-enhancing regions favoring necrosis.
Otherwise, the muscles are of normal signal intensity.
The bones are of normal bone marrow signal intensity and have normal cortical margins.
Multiple large heterogeneously enhancing masses are seen in the right hemithorax. One of them, which is located at the right lung apex, invades the adjacent chest wall causing first and second rib destruction. In addition, a similar well-defined mass is noted at the left para-aortic region.
A 6 mm gallstone is present.
Several small peripelvic cysts are noted in kidneys.
Advanced degenerative changes are seen in the lumbar spine accompanied by dextroscoliosis.
The patient underwent a tissue exam of the left mid-forearm mass.
Histopathology:
The section shows skin tissue with the proliferation of tumoral cells in the dermis composed of spindle cells with large vesicular pleomorphic nuclei and prominent nucleoli arranged as fascicles. Some giant cells, bizarrely shaped nuclei, frequent mitoses, and necrosis are seen.
IHC results on soft tissue tumor specimen:
Vimentin: positive
CD56: negative
Desmin: negative
SMA: negative
S100: negative
CK: negative
CD34: positive in malignant cells
Diagnosis:
High grade angiosarcoma.
Case Discussion
Angiosarcomas are rare and account for less than 1% of all sarcomas. They are aggressive masses that arise from vascular structures and frequently have metastases at the time of diagnosis. Radiologically, they appear quite similar to hemangiopericytomas and hemangioendotheliomas but are more aggressive with a poor prognosis.
Musculoskeletal angiosarcomas on cross-sectional imaging show a non-specific soft tissue mass, with a attenuation and signal intensity similar to muscle but demonstrating bright contrast enhancement. CTA or MRA may demonstrate large feeding vessels.
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