Solitary fibrous tumor/hemangiopericytoma: treatment results based on the 2016 WHO classification

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OBJECTIVE

Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a new combined entity for which a soft-tissue–type grading system, ranging from grades I to III, has been introduced in the 2016 WHO classification of tumors of the CNS. The results of the treatment of this new disease entity require evaluation.

METHODS

The authors retrospectively reevaluated the pathological findings and medical records of patients with SFT/HPC. This study included 60 patients (27 men and 33 women, median age 42.5 years, range 13–69 years) treated at Severance Hospital between February 1981 and February 2016. Four, 40, and 16 patients were categorized as having SFT/HPC grades I, II, and III, respectively. Among these patients, SFTs diagnosed in 7 patients were regraded as grades I (n = 4), II (n = 2), and III (n = 1).

RESULTS

The median overall survival (OS) was 73.2 months (range 1.4–275.7 months), and the progression-free survival (PFS) after the first operation was 53.8 months (range 1.4–217.7 months). Six patients (10%) showed extracranial metastasis during a median period of 103.7 months (range 31.9–182.3 months). Nineteen patients (31.7%) presented with tumor recurrences. The patients in the grade III group had shorter PFS and OS, as well as a shorter period to extracranial metastasis, compared with patients in the grade II group. In the grade II group, patients who underwent gross-total resection showed longer PFS than those who underwent subtotal resection; however, there was no difference in OS. Patients who underwent adjuvant radiation therapy (RT) after surgery had longer PFS compared with that of patients who did not undergo adjuvant RT.

CONCLUSIONS

The SFT/HPC grade I group showed a relatively benign course compared with those of the other groups. The grade III group presented a course with a more aggressive nature than that of the grade II group. In the grade II group, the extent of resection and adjuvant RT was significantly associated with longer PFS. The long-term follow-up and periodic systemic evaluation are mandatory to detect systemic metastasis.

ABBREVIATIONS EOR = extent of resection; GKS = Gamma Knife surgery; GTR = gross-total resection; HPC = hemangiopericytoma; OS = overall survival; PFS = progression-free survival; RT = radiation therapy; SFT = solitary fibrous tumor; STR = subtotal resection.

Article Information

Correspondence Jong Hee Chang: Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea. changjh@yuhs.ac.

INCLUDE WHEN CITING Published online March 9, 2018; DOI: 10.3171/2017.9.JNS171057.

W.S.C. and J.H.C. contributed equally to this work and share senior authorship.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Kaplan-Meier survival curves showing a comparison of recurrence rate and OS between the GTR (n = 27) and STR (n = 13) groups in patients with WHO grade II tumors. Left: The STR group showed a statistically significant higher recurrence rate compared with the GTR group (p = 0.00, log-rank test). Right: Comparison of OS between the 2 resection groups showing no statistical significance (p = 0.315). Figure is available in color online only.

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    Kaplan-Meier survival curves showing a comparison of OS and PFS between initial surgery with adjuvant RT group (n = 25) and without adjuvant RT group (n = 15) in patients with WHO grade II tumors. Left: The surgery with adjuvant RT group showed a statistically significant longer PFS compared with the surgery without adjuvant RT group (p = 0.025, log-rank test). Right: Comparison of OS between the 2 groups showed no statistical significance (p = 0.946). Figure is available in color online only.

  • View in gallery

    Kaplan-Meier survival curves showing a comparison between the WHO grade II and III groups. Left: The PFS between the 2 groups showed no statistically significant difference (p = 0.869). Right: The OS of the WHO grade III group was significantly shorter than that of the WHO grade II group (p = 0.003, log-rank test). Figure is available in color online only.

  • View in gallery

    Axial MR images from a patient in the SFT/HPC WHO grade II group. A: The patient presented with a large tumor at the right-side temporal, petrous bone, cerebellopontine angle area. B: After 107 months of initial treatment, the patient showed a fourth tumor recurrence and underwent RT. C: After the fourth recurrence and RT, the follow-up imaging showed a good treatment response state. D: After the fifth recurrence of tumor, the patient underwent a fourth GKS.

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