Surveillance for metastatic hemangiopericytoma-solitary fibrous tumors-systematic literature review on incidence, predictors and diagnosis of extra-cranial disease

Background: Intracranial hemangiopericytomas (HPC) and solitary fibrous tumors (SFTs) (HPC-SFT) are rare vascular tumors that resemble meningioma on imaging and predominantly affect young adults. HPC-SFT have a high rate of local recurrence with well-known propensity for extracranial metastases. This provides clinical dilemmas frequently encountered in oncology: (i) How should these patients be monitored long term? (ii) Which primary tumors are more likely to metastasize?

Objectives: This systematic review aims to identify the incidence, common locations and time to presentation of extra-cranial metastases of HPC-SFT. We will assess the effect of primary tumor location, treatment, grade, patient age, gender and effect of local recurrence on rates of extra-cranial metastasis and discuss the ideal techniques by which patients with intracranial HPC-SFT should be monitored for extra-cranial metastases.

Methods: Using PRISMA guidelines the authors searched Pubmed. Search terms included hemangiopericytoma, HPC, solitary fibrous tumor/ tumour, SFT, HPC-SFT, extra-cranial metastases, metastases, recurrence, monitoring, follow-up. Studies were identified up to 1st February 2018. Reference lists of identified articles were reviewed to detect other relevant citations. Data were extracted using a standard data collection form and results organized into (i) general study/patient characteristics, (ii) location of extra-cranial metastases, (iii) methods by which metastases were detected and followed up and (iv) characteristics of primary tumors.

Results: Seventy-one studies were identified. Mean recorded follow up ranged from 4 to 312 months. Mean age at diagnosis was 42.0 years. The overall rate of extra-cranial metastasis was 28% (n = 251/904). The minimum time to extracranial metastases was 3 months and the maximum time was 372 months. In the 71 studies identified, where site of extra-cranial metastasis was specified, there were 347 metastases in 213 patients. The most common sites for metastases were bone (location not specified) (19.6%) followed by lung and pleura (18.4%), liver (17.6%), and vertebrae (14.1%). Extra-cranial metastatic disease is typically diagnosed following symptomatic presentation. There is little documentation of methods used to monitor patients with extra-cranial HPC-SFT and no clear surveillance paradigm observed. Higher primary tumor grade (WHO Grade III) was associated with a 1.88 (p = 0.016) increased risk of extra-cranial metastasis. Location and treatment of primary tumor, local recurrence, patient age and gender were not.

Conclusion: Patients with intracranial HPC-SFT require periodic, long term monitoring for extra-cranial metastases. Metastases occur in any age group and can occur early and late. They vary in location and are typically diagnosed following symptomatic presentation. There is no suggested imaging modality for surveillance. Higher grade primary tumors have a greater risk of metastasis. Regular clinical review is essential with early imaging for symptoms of recurrence/metastasis with imaging modality dependent on clinical concern. Quality evidence for an imaging surveillance protocol in this heterogeneous group of patients is lacking. A multicenter study on appropriate surveillance may be of benefit.