Surgical management and long-term outcomes of primary intracranial leiomyosarcoma: a case series and review of literatur

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ORIGINAL ARTICLE

Surgical management and long-term outcomes of primary intracranial leiomyosarcoma: a case series and review of literature Gui-Jun Zhang 1,2 & Jian-Cong Weng 1 & Xu-Lei Huo 1 & Jun-Peng Ma 1 & Bo Wang 1 & Liang Wang 1 & Huan Li 1 & Da Li 1 & Jun-Ting Zhang 1 & Zhen Wu 1 Received: 15 May 2020 / Revised: 10 October 2020 / Accepted: 14 October 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020

Abstract Primary intracranial leiomyosarcomas (PILMSs) are extremely rare neoplasms, and their management remains unclear. The authors aimed to enunciate the radiological features and design a treatment protocol based on previously published cases combined with our series. Clinical data from all 12 cases of PILMS treated at their institute between 2008 and 2018 were reviewed. Meanwhile, they searched the Ovid MEDLINE, Embase, PubMed, Web of Science and Cochrane databases using the keywords “leiomyosarcoma” and “intracranial,” “central nervous system,” “cerebral,” or “brain” Previously published data were processed and used according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The authors’ cohort included 4 males (33.3%) and 8 females (66.7%) ranging in age from 13 to 67 years with a mean of 44.1 ± 5.0 years. Gross total resection (GTR), subtotal resection, and partial resection were achieved in 8 (66.7%), 3 (25%), and 1 (8.3%) patient, respectively, and only four patients (33.3%) received adjuvant therapy after surgery. After a mean follow-up of 30.5 ± 7.6 months, 6 patients (50%) experienced recurrences, and all of them died at the final. Twenty-seven patients (15 were male and 12 were female), in the literature cases, were identified between January 1980 and November 2019, presenting with PILMS. GTR and non-GTR were achieved in 21 (77.8%) and 6 (22.2%) patients, respectively. Postoperative radiotherapy was administrated in 17 patients (63.0%), and postoperative chemotherapy was administrated in 9 patients (33.3%), After a mean follow-up of 22.2 ± 4.1 months, recurrence and death occurred in 8 (36.4%) and 4 (14.8%) cases, respectively. In the pooled cases, the univariate analysis model revealed that only GTR was a significantly favorable factor for increased progression-free survival (hazard ratio 0.270, 95% confidence interval = 0.093–0.787, p = 0.016) and overall survival (hazard ratio 0.255, 95% confidence interval = 0.073–0.890, p = 0.032). GTR was recommended as an optimal treatment; meanwhile, postoperative radiation was also a choice to help increase the survival of patients of PILMS. Keywords Primary intracranial leiomyosarcoma . Treatment strategy . Prognosis

Introduction Gui-Jun Zhang, Jian-Cong Weng and Xu-Lei Huo contributed equally to this work. * Huan Li [email protected] * Zhen Wu [email protected] 1

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Nansihuan 11 Xilu 119, Fengtai District, Beijing, China

2

Present address: Department of Neurosurgery, West China Hospital, Sichuan University, N