CASE PRESENTATION
An incidental finding of cardiomegaly was found on the chest x-ray of a
63-year-old Caucasian female. Follow-up out-patient CT scan revealed a
heterogeneous 108 x 57 x 89-mm pericardial lesion encasing the heart.
Histopathologic biopsy identified spindle cell, pericardial synovial
sarcoma with SS18-SSX1 fusion gene, positive for bcl2 and CD99. No
metastases were found on radiographic imaging. She had a negative
cardiac review of symptoms and echocardiography suggested no significant
pericardial tamponade or effusion at initial diagnosis. Her family
history was negative for cardiac concerns.
The patient completed six cycles of neoadjuvant doxorubicin and
ifosfamide with tumor response. She developed hemorrhagic cystitis,
febrile neutropenia, and significant anemia with her last chemotherapy
cycle requiring an ICU admission. Post-chemotherapy cardiac MRI
(Figure 1 , A-B ) suggested tumor fixation to the superior
cavoatrial junction, encasing but likely not invading the great vessels
and superior pulmonary veins, with potential for invasion of the roof of
the left atrium. A 3D model of the tumor was undertaken using theSketchfab platform (www.sketchfab.com) (Figure 1 ,C-D ) to provide better pre-operative anatomic and thus surgical
understanding. After multidisciplinary discussion and treatment, the
patient was offered operative intervention following completion of her
chemotherapy.