Liver Metastasis in solitary fibrous tumors (Hemangioperizytoma) – A case series

Introduction:

Solitary fibrous tumors (SFT) represent a rare entity of vascular CNS tumors. Extracranial metastasis develops in up to 28% of cases and can occur in multiple organ sites, predominantly bones, lung, pleura and liver up to 3 decades after initial diagnosis. Here we report a case series of three patients treated for liver metastasis from primary CNS SFT at our department.

Methods:

Clinical presentation, diagnostic workup and treatment of three patients referred to our department with liver metastasis after 8 to 15 years of diagnosis of primary SFT are reported.

Results:

Three patients with primary SFT of the CNS that developed liver metastasis were referred to our department. Two patients presented with right upper quadrant pain and liver metastasis were detected by abdominal ultrasound. One patient presented with severe hypoglycemia and diagnosis was made after work-up with PET/CT. Treatment decisions for locoregional treatment modalities such as sRFA, TACE, and liver resection were obtained by a multidisciplinary tumor board and required multiple interventions upon frequent recurrence of hepatic metastases. Despite frequent recurrence, all patients are currently alive at 150 days, 4 years and 5 years from initial diagnosis of liver metastasis and remain under active surveillance by full-body CT scan. Liver transplantation may be considered as a rescue option.

Conclusion:

Currently, no clear recommendations regarding the surveillance strategy after diagnosis of primary SFT of the CNS are established. Clinical practice is guided by individual case reports and ranges from CNS imaging only to full body scans. Our case series exemplifies the aggressive nature of this rare disease that frequently affects young patients and can result in metastasis even decades after diagnosis. Despite successful locoregional treatment in our patients, local recurrence is frequent and warrants life-long active surveillance at a center experienced with locoregional treatment options of the liver.