Central nervous system solitary fibrous tumors: Case series in accordance with the WHO 2021 reclassification. Framework for patient surveillance.

PURPOSE

Solitary fibrous tumors (SFTs) are a rare type of mesenchymal tumors. The World Health Organization reclassified SFTs in 2021. Currently, guidelines concerning treatment and follow-up are lacking. We performed a retrospective case series with reclassification of SFTs, according to the most recent WHO classification, to explore tumor-behavior. The purpose is to build a framework for long-term patient surveillance.

METHODOLOGY

A retrospective case study was performed according to the PROCESS guidelines. Inclusion criteria were: patients operated on between 2013 and 2023 in two neurosurgical centers with the diagnosis of 'hemangiopericytoma' or SFT on histopathological stains. Patients were excluded if the original stains of the primary tumor were unavailable. The following demographic, radiologic and therapeutic parameters were included in the review: age, sex, original and reclassified anatomopathological diagnosis, location, extent of resection, use of postoperative radiotherapy, location of and time to recurrence, location of-and time to metastasis, and survival. Histological material was re-examined by experienced neuropathologists.

RESULTS

Ten patients were identified with a solitary fibrous tumor of the central nervous system (CNS) (three females) between 2013 and 2023. Age at diagnosis ranged from 38 up to 81. Eight patients were treated by gross total resection (GTR) and postoperative radiotherapy (RT) was applied in five cases. Initial WHO grading consisted of three grade I, two grade II, and six grade III lesions. Reclassification according to the WHO 2021 classification of CNS tumors resulted in seven reclassifications, all towards a lower grade. Four patients showed local recurrence, six to eight years after diagnosis, and five patients developed systemic metastases, nine to 13 years after diagnosis.

DISCUSSION

Although rare, SFT should be included in the differential diagnosis of intracranial tumors with extra-axial growth patterns. The current histological grade according to the WHO 2021 does not seem to account for local recurrence rate or systemic metastasis. When a solitary fibrous tumor is presumed, gross total resection is the recommended treatment. Lifelong patient follow-up is necessary due to the risk of delayed recurrence and distant metastasis, even after gross-total resection. We would advocate for the use of CT thorax-abdomen or full body PET in the detection of systemic metastases at diagnosis and during follow-up, however optimal intervals remain unclear.