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DOI: 10.1055/s-0042-1757726
Primary Leptomeningeal Medulloblastoma in Adults: A Diagnostic Challenge—Case Report and Systematic Review
Abstract
Primary leptomeningeal medulloblastoma (PL-MB) in adults is a rare disease with a severe prognosis. A 35-year-old woman presented with headaches, diplopia, and gait ataxia, with triventricular hydrocephalus and descent of the cerebellar tonsils beyond the foramen magnum. Endoscopic third ventriculostomy was performed. Six months later, headaches recurred. Dilatation of the supratentorial ventricular system and massive cerebellar swelling without contrast-enhancing nodularities were reported. Occipitocervical decompression with duraplasty was performed. A bioptic diagnosis of PL-MB was made. Craniospinal irradiation and chemotherapy were administered. After 18 months, no recurrence was observed.
Few cases of PL-MB have been reported: patients die before treatment or within a few days after surgery. Our long-term survival could be ascribable to a slow clinical presentation and an early diagnosis that allowed surgical treatment and the administration of a combined chemoradiotherapy protocol. Cerebellar swelling, even without associated enhancing lesions, with or without hydrocephalus, should be a neuroradiological alarm sign, and PL-MB should be considered.
Keywords
Chiari-like malformation - endoscopic third ventriculostomy - obstructive hydrocephalus - occipitocervical decompression - primary leptomeningeal medulloblastomaPublication History
Article published online:
29 October 2022
© 2022. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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References
- 1 Louis DN, Perry A, Reifenberger G. et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 2016; 131 (06) 803-820
- 2 Roberts RO, Lynch CF, Jones MP, Hart MN. Medulloblastoma: a population-based study of 532 cases. J Neuropathol Exp Neurol 1991; 50 (02) 134-144
- 3 Blessing MM, Alexandrescu S. Embryonal tumors of the central nervous system: an update. Surg Pathol Clin 2020; 13 (02) 235-247
- 4 Hankey GJ, Khangure MS, Spagnolo D, Quinlan MF. Adult onset medulloblastoma cerebelli with leptomeningeal dissemination and coincidental primary hyperparathyroidism. Australas Radiol 1989; 33 (01) 111-115
- 5 David KM, Casey AT, Hayward RD, Harkness WF, Phipps K, Wade AM. Medulloblastoma: is the 5-year survival rate improving? A review of 80 cases from a single institution. J Neurosurg 1997; 86 (01) 13-21
- 6 Asadollahi M, Shayanfar N, Rezaiyan B, Hasibi M. A rare presentation of medulloblastoma in adults as primary leptomeningeal involvement. Iran J Neurol 2012; 11 (01) 30-33 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3829233/
- 7 Ferrara M, Bizzozero L, Fiumara E, D'Angelo V, Corona C, Colombo N. “Primary” leptomeningeal dissemination of medulloblastoma. Report of an unusual case. J Neurosurg Sci 1989; 33 (02) 219-223 https://pubmed.ncbi.nlm.nih.gov/2795197/
- 8 Guo X, Zhong D, Ma W. Primary leptomeningeal medulloblastoma: a rare case. Clin Neurol Neurosurg 2012; 114 (08) 1181-1184
- 9 McGlacken-Byrne SM, Gorman KM, Lagan NK, Robinson I, Farrell M, King MD. Identification of leptomeningeal medulloblastoma with contrast-enhanced magnetic resonance imaging: a devastating differential of acute disseminated encephalomyelitis. J Paediatr Child Health 2018; 54 (08) 929-930
- 10 Mehta RI, Cutler AR, Lasky III JL. et al. “Primary” leptomeningeal medulloblastoma. Hum Pathol 2009; 40 (11) 1661-1665
- 11 Rushing EJ, Smith AB, Smirniotopoulos JG, Douglas AF, Zeng W, Azumi N. Occult leptomeningeal large cell medulloblastoma in an adult. Clin Neuropathol 2009; 28 (03) 188-192
- 12 Suman R, Santosh V, Anandh BA. Primary leptomeningeal medulloblastoma. Pediatr Neurosurg 2007; 43 (06) 544-545
- 13 Millard NE, De Braganca KC. Medulloblastoma. J Child Neurol 2016; 31 (12) 1341-1353
- 14 Ayan I, Kebudi R, Bayindir C, Darendeliler E. Microscopic local leptomeningeal invasion at diagnosis of medulloblastoma. Int J Radiat Oncol Biol Phys 1997; 39 (02) 461-466
- 15 Rutka JT, Hoffman HJ. Medulloblastoma: a historical perspective and overview. J Neurooncol 1996; 29 (01) 1-7
- 16 Noiphithak R, Mektripop N, Thamwongskul C. Rapidly progressive medulloblastoma initially mimicking idiopathic intracranial hypertension and Chiari I malformation: a case report. Int J Surg Case Rep 2021; 85: 106147
- 17 Packer RJ. Childhood medulloblastoma: progress and future challenges. Brain Dev 1999; 21 (02) 75-81
- 18 Kajtazi NI, Nahrir S, Al Shakweer W, Al Ghamdi J, Al Fakeeh A, Al Hameed M. Malignant idiopathic intracranial hypertension revealed a hidden primary spinal leptomeningeal medulloblastoma. BMJ Case Rep 2021; 14 (07) e243506
- 19 Griffiths PD, Coley SC, Romanowski CAJ, Hodgson T, Wilkinson ID. Contrast-enhanced fluid-attenuated inversion recovery imaging for leptomeningeal disease in children. AJNR Am J Neuroradiol 2003; 24 (04) 719-723 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8148663/
- 20 Budka H, Pilz P, Guseo A. Primary leptomeningeal sarcomatosis. Clinicopathological report of six cases. J Neurol 1975; 211 (01) 77-93