• Charles-Henry Mallereau, Mario Ganau, Julien Todeschi, Hélène Cebula, Marie-Des-Neiges Santin, Guillaume Virbel, Raoul Pop, Benoit Lhermitte, Francois Proust, and Salvatore Chibbaro.
• Neurosurgery Department, Strasbourg University Hospital, Strasbourg, France. Electronic address: mallereau.charles@gmail.com.
• World Neurosurg. 2020 Jun 1; 138: 363-368.

BackgroundRhabdomyosarcoma is a rare malignant tumor originating from striated muscle cells. It accounts for only 3% of all soft tissue sarcomas in adults, and its metastases can also reach the central nervous system. Only sporadic cases of primary brain rhabdomyosarcoma (PBRMS) have been reported so far.Case DescriptionWe discuss the atypical presentation and diagnostic challenge of PBRMS in a 65-year-old man. He presented with a 3-day history of progressive right hemiparesis caused by an unspecific left frontoparietal heterogeneously enhancing lesion. Total body computed tomography and positron emission tomography scans performed at baseline did not reveal other secondarisms. The patient underwent radical excision of the lesion, which allowed to establish the diagnosis, with immunohistochemical staining positive for desmin and myogenin. Stereotactic radiotherapy guaranteed local disease control; nonetheless, the patient also required adjuvant chemotherapy when he developed large right lung metastases 6 months postoperatively.ConclusionsPBRMS can be hardly distinguished from other malignant brain tumors during preoperative radiologic workup; only histology can raise the suspicion of primary or metastatic rhabdomyosarcoma, depending on the presence of other distant lesions. Our review of the literature demonstrates that prognosis is poor: 44% of patients die within 1 year from diagnosis. Overall, survival seems to correlate with radical resection, tolerance of stereotactic or if necessary full neuraxis radiotherapy, and adjuvant chemotherapy. Given the high relapse rate, close monitoring and restaging are imperative.Copyright © 2020 Elsevier Inc. All rights reserved.

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