Article
Malignant transformation to secondary gliosarcoma – incidence, risk factors and prognosis
Entstehung von sekundären Gliosarkomen durch maligne Transformation – Inzidenz, Risikofaktoren und Prognose
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Authors
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Daniela Pierscianek
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Marvin Darkwah Oppong
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Anna Michel
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Karsten Henning Wrede
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Ulrich Sure
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Ramazan Jabbarli
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
| Published: | June 26, 2020 |
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Outline
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Objective: Primary gliosarcoma (PGS) are rare malignant brain tumor entities. Even rarer is the occurrence of secondary gliosarcoma (SGS) due to malignant transformation, occurring mostly from primary glioblastoma (PGB). Current evidence regarding SGS is based on care reports or small case series. The aim of this study was to analyze the epidemiologic and clinical features of SGS patients based on large mono-centric series.
Methods: Of the institutional database, containing all consecutive patients with tumors of the central nervous system treated between 2001 and 2018, the cases with SGS were retrospectively collected. Demographic, clinical, radiographic and histological characteristics of the SGS patients were analyzed and compared to the counterpart cohorts with primary entities. Univariate and multivariate analyses were performed.
Results: During the observational period, SGS was documented in 20 cases, predominantly in male patients (60%). Mean age of the patients was 50.9 (±17.8) and 54.7 (±16.3) years at the time of primary diagnosis and malignant transformation respectively. The most common primary entity was PGB (50%), followed by 5 WHO-Grade III and 4 WHO-Grade II tumors, as well as 1 case of NOS CNS embryonal tumor. The incidence of SGS transformation was 0.8%, 0.6% and 0.6% for PGB, WHO Grade III and II tumors respectively (p=0.6477). The location of the tumor in the temporal lobe was significantly associated with the risk of SGS transformation among all WHO-Grade II-IV brain tumors (OR=5.0 [95% CI: 2.07 – 12.14], p=0.0002). Biological grading of the primary tumor inversely correlated with the timing of malignant transformation accounting in average 12.1 months, 12.7 and 138.6 months for PGB, WHO Grade III and II tumors respectively (p=0.0107). However, SGS survival after transformation was not associated with the primary WHO grading (p=0.9158). There was a trend towards decreasing rates of the MGMT methylation, when comparing PGB with PGS and SGS (38.6% vs 27.3% vs 16.7% respectively, p=0.0829).
Conclusion: Malignant transformation to SGC is a very rare complication of CNS tumors, which can occur between several weeks and many years after primary diagnosis. The risk of SGS development is not limited to PGB. Location of the tumor in the temporal lobe seems to increase the likelihood of SGS transformation.
