Table 2.
Etiology | Neoplasms of “perivascular epithelioid cells” |
Incidence | 0.1 to 0.22 % in general population 20% in tuberous sclerosis complex |
Gender ratio | F: M = 4:1 |
Age predilection | Middle aged female |
Imaging features | US: hyperechoic mass. “to and fro” color pattern on Doppler in aneurysm. CT: mass with fat attenuation. Calcification is absent. Aneurysm seen as a vascular enhancing mass with or without thrombosis in arterial phase MRI: bright mass on T1, T2 with drop in signal on fat suppressed sequences. |
Complications | Aneurysm formation, rupture, hemodynamic instability, secondary infection |
Predictors of aneurysm rupture | Aneurysm formation common in Tuberous sclerosis. Mean tumor diameter: 11.4 cm Mean aneurysm diameter: 13.3 mm |
Risk factors for tumor rupture | Pregnancy, large tumor and aneurysm size (see above). |
Prognosis | Tumor recurrence very rare in sporadic cases. In TSC, contra lateral tumor recurrence reported. |
Treatment options | Symptomatic rupture: embolisation/partial/radical nephrectomy. Small/asymptomatic aneurysm: embolisation. |