Resident Neurosurgery and Academic Registrar, Punjab Institute of Neurosciences Punjab Institute of Neurosciences Lahore, PK
Introduction: Leiomyosarcoma (LMS) is a rare malignancy of the smooth muscle cell and it can involve any organ, uterus being the most frequently affected organ. Lungs and liver are the frequently reported sites of metastasis. Cases involving the central nervous system (CNS) primarily without any evidence of systemic disease have emerged, questioning its oncologic origin from brain parenchyma and meninges. We aim to determine the epidemiology, clinico-pathological patterns and neuro-oncological management of PICLMS.
Methods: After an extensive literature search using PubMed Central and Google Scholar, twenty four case reports and one case series comprising 36 patients with PICLMS were retrieved and included using the Preferred Reporting Items for Systematic Reviews and Meta Analyses (PRISMA) guidelines, published between 2000 and 2023.
Results: Male preponderance of the neoplasm (55.56%) with the mean age of 43.10 ± 15.37 years was noted. Diagnosis of pediatric PICLMS was made among 13.89% of patients. Headache, head mass and motor neurological deficit were the most common presenting clinical manifestations among 17.02%, 13.83% and 11.70% patients, respectively. PICLMS arose from brain parenchyma in 75.03% cases, mostly involving the frontal lobe (19.44%) and left hemisphere (36.11%). Meningeal, venous and bony origins were also seen. Heterogenous mass was noted among 21.79% cases on MRI. Gross Total Resection was possible in 63.89% lesions. Smooth muscle actin, Glial Fibrillary Acidic Protein and S-100 were positive among 21.79%, 7.69% and 6.41 of patients on immunohistochemistry, respectively. A mean follow up of 21.32 ± 22.64 months was noted. Intracranial recurrence was noted in 33.33% patients.
Conclusion : Surgery and adjuvant radiotherapy are the mainstay of treatment of PICLMS, while meningioma an important differential diagnosis. Its highly variable presentation on T1-, T2- weighted and FLAIR images makes MRI an unreliable investigation to diagnose PICLMS pre-operatively. High recurrence rate, combined with aggressive behavior make keen follow up essential. Fruitful chemotherapeutic regimens can only be developed after seeking the oncologic origin of PICLMS.
