{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,6,5]],"date-time":"2025-06-05T11:31:33Z","timestamp":1749123093988},"reference-count":17,"publisher":"Scientific Scholar","content-domain":{"domain":["surgicalneurologyint.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:sec id=\"st1\">\n<jats:title>Background: <\/jats:title>\n<jats:p>Alveolar rhabdomyosarcoma (ARMS) shows a predilection for the peripheral extremities and is very rarely identified as a primary in the brain. Here, we report a case of ARMS with multiple lesions exclusively within the central nervous system (CNS).<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"st2\">\n<jats:title>Case Description: <\/jats:title>\n<jats:p>A 20-year-old man presented to our hospital with a gradually increasing headache and disturbance of consciousness. Neuroimaging showed hydrocephalus and multiple tumor lesions, including in the brainstem and cerebellum, with uniform gadolinium enhancement on T1-weighted magnetic resonance imaging, as well as spinal cord seeding. Cerebrospinal fluid (CSF) analysis showed a slightly elevated cell count (6\/\u03bcL; normal, &lt;5\/\u03bcL) and highly elevated protein (153 mg\/dL). In addition, atypical cells were cytologically identified in the CSF. No other laboratory findings were abnormal. Emergency ventricular drainage was performed to control cerebral pressure, followed by a biopsy to confirm the diagnosis. Histological examination revealed a fascicular arrangement of oval cells with eosinophilic cytoplasm and tumor cells with pleomorphic nuclei and prominent nucleoli. Immunohistochemical studies showed negative results for glial fibrillary acidic protein and positive results for desmin and myogenin. In addition, molecular analysis revealed that this tumor had the H3F3A p.Lys28Met mutation and no paired box (PAX)3-forkhead box O1 (FOXO1) or PAX7-FOXO1 fusion genes. ARMS was, therefore, diagnosed. Chemotherapy and radiotherapy were subsequently initiated, but tumor growth could not be controlled, and the patient died 6 months after surgery.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"st3\">\n<jats:title>Conclusion: <\/jats:title>\n<jats:p>This report describes an extremely rare case of ARMS arising exclusively within the CNS.<\/jats:p>\n<\/jats:sec>","DOI":"10.25259\/sni_299_2024","type":"journal-article","created":{"date-parts":[[2024,6,28]],"date-time":"2024-06-28T11:41:59Z","timestamp":1719574919000},"page":"221","update-policy":"http:\/\/dx.doi.org\/10.25259\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["An extremely rare case of primary alveolar rhabdomyosarcoma in the central nervous system"],"prefix":"10.25259","volume":"15","author":[{"given":"Masahiro","family":"Nishikawa","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Ehime University School of Medicine, Toon, Ehime, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akihiro","family":"Inoue","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Ehime University School of Medicine, Toon, Ehime, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kyoko","family":"Moritani","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Ehime University School of Medicine, Toon, Ehime, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mari","family":"Kagajo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Ehime University School of Medicine, Toon, Ehime, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Riko","family":"Kitazawa","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology, Ehime University School of Medicine, Toon, Ehime, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takeharu","family":"Kunieda","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Ehime University School of Medicine, Toon, Ehime, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"10894","published-online":{"date-parts":[[2024,6,28]]},"reference":[{"key":"10.25259\/SNI_299_2024\/ref-1","doi-asserted-by":"crossref","first-page":"778","DOI":"10.1016\/S0046-8177(86)80198-8","article-title":"Tumors of skeletal muscle","volume":"17","author":"Agamanolis","year":"1986","journal-title":"Hum Pathol"},{"key":"10.25259\/SNI_299_2024\/ref-2","first-page":"211","article-title":"WHO classification of tumours: Soft tissue and bone tumours","author":"Agaram","year":"2020","edition":"5th ed"},{"key":"10.25259\/SNI_299_2024\/ref-3","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1186\/s13256-024-04471-w","article-title":"Primary alveolar rhabdomyosarcoma of the brain: A case report","volume":"18","author":"Al Mahmasani","year":"2024","journal-title":"J Med Case Rep"},{"key":"10.25259\/SNI_299_2024\/ref-4","doi-asserted-by":"crossref","first-page":"164","DOI":"10.4103\/0973-029X.180987","article-title":"Alveolar rhabdomyosarcoma of maxilla","volume":"20","author":"Ananthaneni","year":"2016","journal-title":"J Oral Maxillofac Pathol"},{"key":"10.25259\/SNI_299_2024\/ref-5","doi-asserted-by":"crossref","first-page":"bcr2013203257","DOI":"10.1136\/bcr-2013-203257","article-title":"Pleomorphic rhabdomyosarcoma of the cerebellopontine angle in an adult: A review of literature","volume":"30","author":"Caporlingua","year":"2014","journal-title":"BMJ Case Rep"},{"key":"10.25259\/SNI_299_2024\/ref-6","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1023\/A:1005884202389","article-title":"Primary rhabdomyosarcoma of the brain: Observations on a case with clinical and radiological evidence of cure","volume":"36","author":"Celli","year":"1998","journal-title":"J Neurooncol"},{"key":"10.25259\/SNI_299_2024\/ref-7","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1002\/cncr.11550","article-title":"Rhabdomyosarcoma in adults. 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