ReviewPakistan journal of medical sciences2025
Primary intracranial rhabdomyosarcoma: A systematic review of existing literature from 2000 to 2024.
Review in Pakistan journal of medical sciences, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Background and Objective: Primary Intracranial Rhabdomyosarcoma (PIRMS) represents a rare and poorly understood entity within brain tumours. The objective was to evaluate the clinicoradiological presentation and optimal management strategies for PIRMS. Methodology: A comprehensive literature search was conducted in PubMed Central and Google Scholar from 2000 to 2024. Only English-language, open-access articles with confirmed human PIRMS cases were included. Quality was assessed using Joanna Briggs tools, and data synthesis followed PRISMA guidelines, registered with PROSPERO with registration number CRD42024507092. A total of 176 articles were screened, and finally, 27 were included, comprising data on 39 patients. Result: About 22 (56.41%) patients were adults, and 17 (46.59%) were pediatric, with a male predominance of 23 cases (58.67%). Most patients (25) were Asians (64.1%). Headache, facial nerve palsy, and nausea/vomiting in 58.97% (23), 28.21% (11) and 25.64% (10) were the most common findings. Imaging showed ventriculomegaly and haemorrhage, in 10.26% (4) each on computed tomography, and hyperintensities on T1WI in 20 cases (51.28%). About 17.95% (7) of lesions were in the pineal region. Gross-total resection was performed in 15 cases (38.46%). On histopathology, rhabdomyoblasts and round cells were found in 25.64% (10) and 38.46% (15). The mean follow-up of 28 patients was 14.39 +- 17.68 months. Conclusion: Despite limitations, PIRMS remains difficult to diagnose preoperatively, with histopathology as the gold standard. Treatment response varies, but adjuvant chemoradiotherapy and gross total resection improve outcome.
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