СONGENITAL MEDULLOBLASTOMA: PRENATAL AND POSTNATAL MRI FINDINGS
https://doi.org/10.21294/1814-4861-2019-18-6-122-128
Abstract
Background. Medulloblastoma represents the most common malignant pediatric brain neoplasm accounting for 15–20 % of brain tumors in childhood. However, congenital medulloblastomas are extremely rare and mostly diagnosed postnatally. to the best of our knowledge, only one case was reported where this tumor was detected antenatally by ultrasound. MRI features of fetal medulloblastoma have not been described to date. Material and methods. We present MRI findings of fetal medulloblastoma progression from the 31st week of gestation until 5.5 months of postnatal life. Histopathological, genetic and molecular parameters were studied. after partial tumor removal and chemotherapy, MRI images demonstrated complete tumor response to therapy. the follow-up period was 1.5 years. Results. Fetal MRI performed on the 31st gestational week revealed a midline cerebellar lesion of ≤2 cm in size with minor t2 hypointensity and t1 hyperintensity. additionally, a restricted diffusion on aDc-maps depicted the lesion most conspicuously. the advantage of DWI over traditional t1- and t2-VI was associated with a relatively lower diffusion coefficient in the tumor (0.63×10–3 mm2/s) compared with the surrounding medulla (0.98×10-3 mm2/s). Histopathological, genetic and molecular studies revealed desmoplastic medulloblastoma with high nodularity, cMYc/NMYc-negative, МхR+. combined treatment using 5 cycles of HIt-sKK chemotherapy by HIt MED 2014 protocol (version 2017) in a highly specialized pediatric oncology center resulted in a stable tumor remission. Conclusion. Fetal MRI with DWI is the most effective approach for early detection of congenital medullobastoma. Early prenatal diagnosis, as well as postnatal verification and classification of congenital medulloblastomas is critical for timely treatment planning and good outcome.
Keywords
congenital medulloblastoma,
fetal brain tumor,
magnetic resonance imaging,
diffusion weighted images
Supporting agencies: The work was supported by Russian Science Foundation (the project №19-75-20142). The authors thank the patient’s parents for their help in collecting the history of the disease and MRI images obtained in other medical institutions where the child was treated (Federal Center for Neurosurgery (Novosibirsk, Russia), Burdenko Institute (Moscow, Russia), Children’s National Research Center Oncology named after Dmitry Rogachev (Moscow, Russia). We thank Ministry of Science and Higher Education of the RF for access to MRI equipment.
About the Authors
A. M. Korostyshevskaya
International Tomography Center of the Siberian Branch of the Russian Academy of Sciences
Russian Federation
Russian Federation
MD, PhD, DSc, Leading Researcher, Head of Medical Diagnosis Department
Researcher ID (WOS): D-3588-2018. Author ID (Scopus): 47061318800
3а, Institutskaya street, 630090, Novosibirsk, Russia
L. I. Papusha
Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology
Russian Federation
Russian Federation
MD, PhD, Leading Researcher
AuthorID (РИНЦ): 877210. Author ID (Scopus) 42962084100
1, samory Mashel street, 117997, Moscow, Russia
A. A. Savelov
International Tomography Center of the Siberian Branch of the Russian Academy of Sciences
Russian Federation
Russian Federation
PhD in Physical and Mathematical Sciences, Senior Researcher
ResearcherID (WOS): D-3539-2018. Author ID (Scopus): 16067478200
3а, Institutskaya street, 630090, Novosibirsk, Russia
References
1. Rickert C.H., Paulus W. Epidemiology of central nervous system tumors in childhood and adolescence based on the new WHO classification. Childs Nerv Syst. 2001; 17: 503–511. doi: 10.1007/s003810100496.
2. Isaacs H.I. Jr. Perinatal brain tumors: a review of 250 cases. Pediatr Neurol 2002; 27: 249–261. doi: 10.1016/s0887-8994(02)00472-1.
3. Nishio S., Morioka T., Fukui M. Medulloblastoma in the first year of life: A report of five cases. J Clin Neurosci 1998; 5: 265–269. doi: 10.1016/s0967-5868(98)90060-1.
4. Rickert C.H. Neuropathology and prognosis of foetal brain tumours. Acta Neuropathol. 1999 Dec; 98(6): 567–76. doi: 10.1007/s004010051120.
5. Mitchell D., Rojiani A.M., Richards D., Yachnis A.T., Powell S.Z. Congenital CNS primitive neuroectodermal tumor: Case report and review of the literature. Pediatr Pathol Lab Med 1995; 15: 949–56. doi: 10.3109/15513819509027031.
6. Korostyshevskaya A.M., Prihod’ko I.Y., Savelov A.A., Yarnykh V.L. Direct Comparison Between Apparent Diffusion Coefficient and Macromolecular Proton Fraction as Quantitative Biomarkers of the Human Fetal Brain Maturation. J Magn Reson Imaging. 2019 Jul; 50(1): 52–61. doi: 10.1002/jmri.26635.
7. Korostyshevskaya A., Kurganova A., Savelov A., Yarnyh V. Microstructural white matter abnormalities in isolated ventriculomegaly decrease during pre- and post-natal brain maturation. Annual of Neurology. 2016; 80(suppl 20): S355.
8. Gilbertson R.J., Ellison D.W. The origins of medulloblastoma subtypes. Annu Rev Pathol. 2008; 3: 341–365. doi: 10.1146/annurev.pathmechdis.3.121806.151518.
9. von Bueren A.O., von Hoff K., Pietsch T., Gerber N.U., WarmuthMetz M., Deinlein F., Zwiener I., Faldum A., Fleischhack G., Benesch M., Krauss J., Kuehl J., Kortmann R.D., Rutkowski S. Treatment of young children with localized medulloblastoma by chemotherapy alone: Results of the prospective, multicenter trial HIT 2000 confirming the prognostic impact of histology. Neuro Oncol. 2011 Jun; 13(6): 669–79. doi: 10.1093/neuonc/nor025.
10. Poretti A., Meoded A., Huisman T.A. Neuroimaging of pediatric posterior fossa tumors including review of the literature. J Magn Reson Imaging. 2012 Jan; 35(1): 32–47. doi: 10.1002/jmri.22722.
11. Gilles F.H. Myelination in the neonatal brain. Hum Pathol 1976; 7: 244–248. doi: 10.1016/s0046-8177(76)80035-4
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For citations:
Korostyshevskaya A.M., Papusha L.I., Savelov A.A. СONGENITAL MEDULLOBLASTOMA: PRENATAL AND POSTNATAL MRI FINDINGS. Siberian journal of oncology. 2019;18(6):122-128. (In Russ.) https://doi.org/10.21294/1814-4861-2019-18-6-122-128
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