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Malignant progression of cerebellopontine angle solitary fibrous tumors following radiation: illustrative case

Anna K. La Dine, Nida Fatima, Zachary R. Barnard, William H. Slattery, and Gregory P. Lekovic

BACKGROUND

Intracranial solitary fibrous tumors (ISFTs) are rare mesenchymal tumors originating in the meninges and constitute a heterogeneous group of clinical and biological behavior. Benign histotypes, such as hemangiopericytomas are now considered as a cellular phenotypic variant of this heterogenous group of rare spindle-cell tumors. IFSTs are poorly recognized and remain a diagnostic challenge due to rarity and resemblance to other brain tumors. Previously, IFSTs were thought to pursue a slow, indolent, and nonaggressive course, however, a growing body of literature based on longer follow-up demonstrates an unpredictable clinical course and an uncertain diagnosis.

OBSERVATIONS

A rare case report of malignant transformation of IFST following radiation therapy is reported. In this case a 60-year-old female who underwent gross total resection of the cerebellopontine angle tumor with histopathology consistent with solitary fibrous tumor followed by salvage stereotactic radiosurgery, presented with another recurrence after 2 years of surgery. The authors performed complete removal of the tumor with pathology now consistent with malignant solitary fibrous tumor. A recent follow-up magnetic resonance imaging did not show any recurrence or residual tumor, and the patient reports a generalized well-being.

LESSONS

This report will help to understand the natural history and unusual clinical behavior of these intracranial tumors.