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Cheng-Bei Li, Lai-Rong Song, Da Li, Jian-Cong Weng, Li-Wei Zhang, Jun-Ting Zhang and Zhen Wu

OBJECTIVE

The overall survival and pertinent adverse factors for primary intracranial malignant melanoma (PIMM) have not been previously determined. This aim of this study was to determine the rates of progression-free survival (PFS) and overall survival (OS) and identify the adverse factors for PIMM.

METHODS

This study included 15 cases from the authors’ own series and 100 cases with detailed clinical data that were obtained from the literature from 1914 to 2018 using the Ovid Medline, EMBASE, PubMed, Cochrane, and EBSCO databases. Patient demographics, treatment (surgery, chemotherapy, and radiotherapy [RT]), PFS, and OS were reviewed. Data from prior publications were processed and used according to PRISMA guidelines.

RESULTS

Diffuse lesions were identified in 24 (20.9%) patients, who had a younger age (p < 0.001). The mean follow-up time was 16.6 months, and 76 (66.1%) deaths occurred. The 6-month, 1-year, 3-year, and 5-year OS rates of the whole cohort were 62.8%, 49.9%, 28.9%, and 17.2%, respectively, with an estimated median survival time (EMST) of 12.0 months. The multivariate analysis revealed that gross-total resection (GTR) (HR 0.299, 95% CI 0.180–0.497, p < 0.001), radiotherapy (HR 0.577, 95% CI 0.359–0.929, p = 0.024), and chemotherapy (HR 0.420, 95% CI 0.240–0.735, p = 0.002) predicted a better OS. The EMST was 5.0 months in patients with diffuse-type PIMM and 13.0 months in patients with the solitary type. Patients receiving GTR with adjuvant RT and/or chemotherapy (GTR + [RT and/or chemo]) had significantly higher 1-year and 5-year OS rates (73.0% and 40.1%, respectively) and a longer EMST (53 months) than patients who underwent GTR alone (20.5 months) or RT and/or chemotherapy without GTR (13.0 months).

CONCLUSIONS

Optimal outcomes could be achieved by radical resection plus postoperative radiotherapy and/or chemotherapy. Patients with diffuse PIMM have a more severe clinical spectrum and poorer survival than patients with solitary PIMM. Immunotherapy and targeted therapy show promise as treatment options for PIMM based on results in patients with brain metastases from extracranial melanoma.

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Jian-Cong Weng, Lai-Rong Song, Da Li, Liang Wang, Zhen Wu, Jun-Mei Wang, Gui-Lin Li, Wang Jia, Li-Wei Zhang and Jun-Ting Zhang

OBJECTIVE

Primary intracranial myxomas (PICMs) are extremely rare neoplasms, and their management and prognostic factors remain ambiguous. The authors aimed to elaborate the radiological features, evaluate the risk factors for progression-free survival (PFS), and propose a treatment protocol based on pooled data from cases treated at their institute and those found in the literature.

METHODS

Clinical data from all cases of PICMs treated at the authors’ institute and those cases reported in the English-language literature between 1987 and December 2017 were reviewed. The authors searched the Ovid MEDLINE, Embase, PubMed, and Cochrane databases using the keywords “myxoma” and “central nervous system,” “intracranial,” “cerebral,” “skull base,” “skull,” or “brain.” Previously published data were processed and used according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Risk factors in the pooled cohort were evaluated.

RESULTS

Cases from the authors’ institute included 21 males and 9 females, with a mean age of 35.7 ± 1.7 years. Gross-total resection (GTR) and non-GTR were achieved in 6 (20.0%) and 24 (80.0%) patients, respectively. After a mean follow-up of 86.7 ± 14.1 months, recurrence occurred in 6 (24%) patients, for a median PFS time of 85.2 months (range 36.0–136.0 months) and no deaths. In the literature between 1987 and 2017, 35 cases of PICM were identified in 14 males and 21 females with a mean age of 31.7 ± 3.2 years. GTR and non-GTR were achieved in 23 (65.7%) and 9 (25.7%) cases, respectively. After a mean follow-up of 25.8 ± 6.9 months (range 1.0–156.0 months), recurrence occurred in 4 (14.3%) patients, for a median PFS time of 11.0 months (range 3.0–36.0 months) and no deaths. Actuarial PFS rates at 1, 5, and 10 years were 93.0%, 80.6%, and 67.9%, respectively. A multivariate model demonstrated that GTR (HR 0.058, 95% CI 0.005–0.680, p = 0.023) was the only factor that favored PFS.

CONCLUSIONS

PICMs are rare neoplasms with a slightly higher occurrence in males. GTR was the only favorable factor for PFS. Based on statistical results, GTR alone, if tolerable, is advocated as the optimal treatment for PICM. Nevertheless, conservative excision may be preferred to avoid damage to vital structures. PICMs have a tendency to recur within a few years of the initial surgery if resection is incomplete; therefore, close postoperative follow-up is mandatory. Future studies with larger cohorts are necessary to verify the study findings.

Systematic review registration no.: CRD42018091517 (www.crd.york.ac.uk/prospero/)

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Jian-Cong Weng, Da Li, Liang Wang, Zhen Wu, Jun-Mei Wang, Gui-Lin Li, Wang Jia, Li-Wei Zhang and Jun-Ting Zhang

OBJECTIVE

Intracranial giant cell tumors (GCTs) are extremely rare neoplasms with dismal survival and recurrence rates. The authors aimed to confirm independent adverse factors for progression-free survival (PFS) and to propose an optimal treatment algorithm.

METHODS

The authors reviewed the clinical data of 43 cases of intracranial GCTs in their series. They also reviewed 90 cases of previously reported GCTs in the English language between 1982 and 2017 using Ovid MEDLINE, Embase, PubMed, and Cochrane databases with keywords of “giant cell tumor” or “osteoclastoma” and “skull,” “skull base,” “temporal,” “frontal,” “sphenoid,” or “occipital.” These prior publication data were processed and used according to PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Aforementioned risk factors for the authors’ series and the pooled cases were evaluated in patients not lost to follow-up (m = 38 and n = 128, respectively).

RESULTS

The authors’ cohort included 28 males and 15 females with a mean age of 30.5 years. Gross-total resection (GTR) was achieved in 15 (34.9%) patients. Fifteen patients (39.5%) who did not undergo GTR received postoperative radiotherapy with a mean total dose of 54.7 ± 4.1 Gy. After a mean follow-up of 71.3 months, 12 (31.6%) patients experienced recurrence, and 4 (10.5%) died of disease. The actuarial 5-year PFS and overall survival (OS) were 68.6% and 90.0% in the authors’ cohort, respectively. A multivariate Cox regression analysis verified that partial resection (HR 7.909, 95% CI 2.296–27.247, p = 0.001), no radiotherapy (HR 0.114, 95% CI 0.023–0.568, p = 0.008), and Ki-67 ≥ 10% (HR 7.816, 95% CI 1.584–38.575, p = 0.012) were independent adverse factors for PFS. Among the 90 cases in the literature, GTR was achieved in 49 (54.4%) cases. Radiotherapy was administered to 33 (36.7%) patients with a mean total dose of 47.1 ± 5.6 Gy. After a mean follow-up of 31.5 months, recurrence and death occurred in 17 (18.9%) and 5 (5.6%) cases, respectively. Among the pooled cases, the 5-year PFS and OS were 69.6% and 89.2%, respectively. A multivariate model demonstrated that partial resection (HR 4.792, 95% CI 2.909–7.893, p < 0.001) and no radiotherapy (HR 0.165, 95% CI 0.065–0.423, p < 0.001) were independent adverse factors for poor PFS.

CONCLUSIONS

GTR and radiotherapy were independent favorable factors for PFS of intracranial GCTs. Based on these findings, GTR alone or GTR plus radiotherapy was advocated as an optimal treatment; otherwise, partial resection plus radiotherapy with a dose ≥ 45 Gy, if tolerable, was a secondary alternative. Lack of randomized data of the study was stressed, and future studies with larger cohorts are necessary to verify these findings.

Systematic review no.: CRD42018090878 (crd.york.ac.uk/PROSPERO/)