Primary Intracranial Leiomyosarcoma in an Immunocompetent Adult

Abstract

A 58-year-old man with a prior history of coronary artery disease, hypertension, and radical prostatectomy for adenocarcinoma 6 years earlier presented with a 1-week history of headache and nausea, followed by a witnessed generalized tonic-clonic seizure. The history was positive for a 100 pack-year smoking history and an absence of intravenous drug use or sexual promiscuity. The physical examination, after resolution of the postictal state, was remarkable only for a tongue bite. Routine laboratory studies and toxicology screening were normal. An initial noncontrast computed tomography (CT) scan of the brain showed a questionable tiny arachnoid cyst adjacent to the right tentorium and ethmoid sinusitis (Fig 1). A magnetic resonance imaging (MRI) scan with contrast revealed a hypodense area with loss of gray-white differentiation on T1 imaging. Postgadolinium scan showed an enhanced 1.6 ϫ 1.3 cm mass in the left anteromedial temporal lobe (Figs 2A to 2C, arrows). There was also increased signal on T2 and flair images in the surrounding white matter suggestive of edema/mass effect (Figs 2D and 2E, arrows). There was a 0.65-cm area of increased signal on T2 and hypointense flair without enhancement on the postgadolinium measures in the right temporal region, likely representing a subarachnoid cyst. The differential included metastases, primary lymphoma, meningioma, and toxoplasmosis. A CSF

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

“…Primary intracranial smooth muscle tumors can arise from the leptomeningeal coatings, with the pluripotent mesenchymal stem cells in the dura-mater being the probable origin cells [5]. 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].…”

Primary extradural leiomyosarcoma involving cavernous sinus in an immunocompetent patient

“…[4,5] There is usually no sex predilection and the median duration of symptoms before presentation is nearly 4 months. [6] Intraparenchymal location of the tumor is mostly observed in systemic metastases and primary examples are rare. Systemic LMSs have a predilection for hematogenous spread to CNS and most frequent primary sites include uterus and gastrointestinal tract followed by retroperitoneum, lung and heart.…”

“…However, the prognosis of this tumor remains poor with the longest survival reported in literature being 32 months. [6] Patient survival is limited due to several reasons. Gross resection of tumor with adequate surgical margins isn't achievable in most cases and the behavior of tumor is usually aggressive with limited response to chemotherapeutic drugs.…”

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

“…Patient outcome was documented in 30/37(81.1%)cases(table2).Atotalof 9/30(24.3%)patientsdied,7asadirect consequenceofintracranialLMS,2due to postsurgical complications [25,31]. 13 patients did not experience tumor recurrence following complete resection,whilelocalrecurrencewasexperiencedby1patientduringfollow-up [3], and malignant pleural effusion was detectedinanotherpatient [4].Theoverall median survival period following resection of primary intracranial LMS was 96 months (95% confidence interval not applicable), and the 5-year sur- Zhang/Dong/Huang/Zhang/Ma/ Zhou/Shu/Cheng larimmunityismoreimportantthanhumoral immunity [33].…”

“…For vimentin, 15/37 (40.5%) cases were positive, while 2 cases were equivocal. In addition, only 1 study includeddetectionofotherspecificmarkersforsmoothmuscle cells,includingh-caldesmon [29]andcalponin [31].Negative immunostainingsincludedEMA(n=24),GFAP(n=15),and HMB-45(n=7). Of the 20/37 (54.1%) patients who received postoperative radiotherapy, the dosage, fractionation, administration methods, and tumor responses achieved were not well documented.…”