Right lower limb weakness as an unusual initial presentation in pediatric medulloblastoma
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Résumé
The 13-year-8-month-old boy suffered from lower back pain 2 weeks ago and progressive right lower limb weakness and numbness from bilateral feet ascending to the lumbar region since 4 days ago. There was no accompanying headache, vomit, and bowel or bladder dysfunction. Neurologic examination revealed right lower limb weakness with muscle power grade 1/5 distally and 3/5 proximally and diminished sensations below T7 dermatome. Furthermore, the anal tone was mildly loose. Deep tendon reflex of right lower limb increased. Dysdiadochokinesia was not present. The finger-nose-finger test was normal, and so were tandem gait and Romberg test. Ophthalmological examination revealed no papilledema. Initially, spinal cord lesion was impressed. Magnetic resonance imaging (MRI) of the spine (Fig. 1A and B) showed intradural extramedullary enhanced nodular lesion at the T7 level and linear enhancement at C7 and T2 to T9. Leptomeningeal carcinomatosis was suspected. Brain MRI (Fig. 2A–C) revealed a mass with hypointensity on T1-weighted images, hyperintensity on T2-weighted images, and strong enhancement at the superior cerebellar region. The cerebellar tumor was removed. Histopathology and immunohistochemical staining showed tumor cells with Homer–Wright rosettes (Supplementary Figure A) and negative for beta-catenin (nuclear; Supplementary Figure B), GAB-1 (Supplementary Figure C), and YAP-1 (Supplementary Figure D) staining. The pathological diagnosis was non-WNT/non-SHH medulloblastoma. Because of progressive right lower limb weakness, T7 nodular lesion was resected 1 week later, and the histopathology and immunohistochemical staining showed the same cerebellar mass. The right lower limb weakness improved after craniospinal irradiation with total dose 36GyE/20fx, plus a further boost to the brain/spinal tumor bed region to 54GyE/30fx, and adjuvant systemic chemotherapy with cisplatin and etoposide.Figure 2Brain magnetic resonance imaging (MRI): (A) T1-weighted axial imaging revealed hypointensity lesion (arrow). (B) T2-weighted axial imaging revealed hyperintensity lesion (arrow). (C) Post-contrast T1-weighted axial imaging revealed a mass lesion with strong enhancement at the superior portion of the cerebellar region (arrow).View Large Image Figure ViewerDownload Hi-res image Download (PPT) Medulloblastoma accounts for 20% of all brain tumors and around 40% of primary posterior fossa tumors in children.1Ostrom Q.T. Gittleman H. Farah P. Ondracek A. Chen Y. Wolinsky Y. et al.CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2006–2010.Neuro Oncol. 2013; 15: ii1-ii56Crossref PubMed Scopus (1043) Google Scholar The clinical presentation of medulloblastoma is mainly related to mass effect and/or obstructive hydrocephalus; however, medulloblastoma primarily presenting with symptoms related to spinal metastasis is rare.2Park T.S. Hoffman H.J. Hendrick E.B. Humphreys R.P. Becker L.E. Medulloblastoma: clinical presentation and management. Experience at the hospital for sick children, Toronto, 1950–1980.J Neurosurg. 1983; 58: 543-552Crossref PubMed Scopus (236) Google Scholar Pezeshkpour et al. reported a 0.01% prevalence of spinal drop metastasis as the initial presenting symptoms after analyzing 18,000 central nervous system tumors.3Pezeshkpour G.H. Henry J.M. Armbrustmacher V.W. Spinal metastases. A rare mode of presentation of brain tumors.Cancer. 1984; 54: 353-356Crossref PubMed Scopus (32) Google Scholar The authors declared no potential conflicts of interest with respect to research, authorship, and/or publication of this article. This study was approved by our Institutional Review Board (No. 201900616B0).
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