Primary Intracranial Leiomyosarcoma: Review of the Literature and Presentation of a Case

Abstract

Background: Primary intracranial leiomyosarcoma (LMS) is a rare tumor of the central nervous system and therefore has only been reported sporadically. Methods: The MEDLINE database was searched for relevant case reports and series published in English from 1969 to March 2012. These papers were reviewed to identify clinical and histopathological features, treatment modalities, and prognoses of patients with primary intracranial LMS. Results: We reviewed 37 patients, including our patient, with primary intracranial LMS. Of these cases, 16 (43.2%) involved immunosuppression, 4 (10.8%) involved radiation-induced LMS, 3 (8.1%) cases involved a potential leiomyosarcomatous transformation of a brain lesion, and 7 (18.9%) cases, including the presented case, had no specific medical history. The treatment for these cases included resection (33/37, 89.2%), postoperative radiotherapy (20/37, 54.1%), and chemotherapy (7/37, 18.9%). Conclusions: The best method for the treatment of LMS could not be determined due to the limited number of cases that have been reported. However, optimized surgical resection, postoperative radiotherapy, and sarcoma-based chemotherapy may improve treatment outcomes.

Primary intracranial leiomyosarcoma among patients with AIDS in the era of new chemotherapeutic and biological agents

“…However, the presence of cellular atypia, coagulative necrosis and high mitotic index (10 or more mitosis per 10 hpf) favour a diagnosis of leiomyosarcoma 17. Most cases of leiomyosarcomas are metastatic from other primary sites 18. Our patients were diagnosed with PIL when no other primary sites were identified on whole-body imaging.…”

Low-grade Leiomyosarcoma of the Cavernous Sinus in an HIV Positive Patient: Case Report

“…Other reports suggest that epithelium cerebral blood vessels could be the site of origin of these sarcomas [5][6][7]. Its age of onset is quite wide since there are reports in patients with ages ranging from 4 to 75 years [3,[6][7][8]. There seems to be a predilection for male patients with a ratio of 2.3:1.6 [8].…”

“…The average duration of symptoms before diagnosis is approximately four months; these symptoms are closely related to the location of the tumor [9]. According to Zhang et al report, out of 37 patients, 18 (48.6%) had the dura as the tumor's site of origin, 10 (27.0%) had it in the convex set, five (13.5%) at the base of the skull and three (8.1%) in the tentorium cerebelli [8]. In the available reports we only found two patients who had, as in our case, the cavernous sinus as a site of origin [10,11].…”

“…Its age of onset is quite wide since there are reports in patients with ages ranging from 4 to 75 years [3,[6][7][8]. There seems to be a predilection for male patients with a ratio of 2.3:1.6 [8]. The average duration of symptoms before diagnosis is approximately four months; these symptoms are closely related to the location of the tumor [9].…”

“…An important point to note is that about 43.2% of intracranial leiomyosarcomas are related to immunosuppression; of the reported cases, only 13 are associated with HIV infection and of these, eight were related to EBV [8]. This association of leiomyosarcomas and HIV has been increasing in the last 10 years and it has been suggested that EBV can infect smooth muscle, at least in patients with acquired immunodeficiency syndrome (AIDS), and contribute to the pathogenesis of leiomyosarcomas [6].…”