Abstract
Abstract
Background
Schwannomas are neoplasms that typically arise from the myelin sheath of peripheral nerves and rarely originate within the brain parenchyma. Some case reports present schwannomas arising from the brainstem, but regrowth of the tumor and the efficacy of postoperative irradiation have not been examined. In addition, the genetic background of schwannomas arising from the brainstem has not been investigated.
Case presentation
A 21-year-old male presented with diplopia, dysphagia, and left-sided hemiparesis, dysesthesia, and ataxia. Intracranial imaging showed a heterogeneous mass with a cystic lesion in the pontomedullary junction. Since the tumor caused obstructive hydrocephalus, the patient underwent subtotal tumor resection. A histopathologic evaluation aided a diagnosis of brainstem intraparenchymal schwannoma. Gradual postoperative mass regrowth was recognized. Three-dimensional conformal radiotherapy was performed on the residual mass and surgical cavity. No tumor regrowth was observed 4 years after surgery. To investigate the genetic background of the tumor, target sequences for 36 genes, including NF2, SMARCB1, and LZTR1, and microsatellite analysis for loss of 22q did not show any somatic variants or 22q loss.
Conclusions
We suggest that brainstem schwannomas might differ from conventional schwannomas in their genetic background.
Funder
Japan Society for the Promotion of Science
Takeda Science Foundation
Publisher
Springer Science and Business Media LLC
Subject
Genetics(clinical),Genetics
Reference33 articles.
1. Bigner DD, McLendon RE, Bruner JM, Russell DS. Russell and Rubinstein’s pathology of tumors of the nervous system. 6th ed. Oxford University Press; 1998.
2. Lin J, Feng H, Li F, Zhao B, Guo Q. Intraparenchymal schwannoma of the medulla oblongata. Case report. J Neurosurg. 2003;98(3):621–4.
3. Ramos AA, Vega MA, Valencia HS, Garcia JC, Perez VC. Intraparenchymal schwannoma involving the brainstem in a young woman. Pediatr Neurol. 2013;48(6):472–4.
4. Sharma AK, Savardekar AR, Nandeesh BN, Arivazhagan A, Rao MB. Intrinsic brainstem schwannoma—a rare clinical entity and a histological enigma. J Neurosci Rural Pract. 2016;7(2):302–4.
5. Agnihotri S, Jalali S, Wilson MR, Danesh A, Li M, Klironomos G, et al. The genomic landscape of schwannoma. Nat Genet. 2016;48(11):1339–48.
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