Agrawal, A

Balpande, DN

Pathak, CS

English

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http://www.bioline.org.br/js  East and Central African Journal of Surgery Volume 14 Number 1 – March/April 2009          73 Cystic Medulloblastoma in a child A. Agrawal1, A. Agrawal2, D.N. Balpande3, C.S. Pathak3 1Associate Professor in Neurosurgery, 2Associate Professor in Pathology,  3Professor in Pediatrics,   Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha (India) Correspondence to: Dr Amit Agrawal, Fax: +91-7152-2231902. Email: <dramitagrawal@gmail.com>  Introduction  Medulloblastoma is a commonly encountered, highly malignant posterior fossa midline cerebellar tumour of children, with a tendency to early recurrence and dissemination through the cerebrospinal fluid1-8. On computed tomography, majority of the medulloblastomas have a typical appearance, consisting of a mass of uniformly increased density that enhances homogeneously after contrast enhancement with sharp demarcation of the neoplasm and there may be associated hydrocephalus2-8.  Case Report  This 14 year male child presented with headache, vomiting and swaying while walking to either side of 1 month duration and loss of vision of 15 days duration. On examination, the child was conscious and well oriented. He had bilateral papilloedema. There was no perception of light or projection of rays in both the eyes. Eye movements were normal and he had bilateral cerebellar signs. There were no other neurological deficits. CT scan (Figure-1) showed a mass in the midline of the posterior fossa, comprising a solid isodense component on the right and a cystic hypodense component on the left, having a density slightly higher than cerebrospinal fluid. There was diffuse enhancement of the solid component. The fourth ventricle was compressed and displaced anteriorly and to the right with associated hydrocephalus.   Figure 1. Plain CT scan showing a midline posterior fossa mass with a high-density area on the left and a low-density area on the right (A-D), the solid component showed marked homogeneous enhancement after contrast administration (E-H) http://www.bioline.org.br/js  East and Central African Journal of Surgery Volume 14 Number 1 – March/April 2009          74  Figure 2. Histopatlogical examination showed highly undifferentiated areas composed of cells with small hyperchromatic nuclei and inconspicuous cytoplasm (H & E 40x)  The child underwent midline suboccipital craniectomy, vermis was split, there was a big cyst containing xanthochromic fluid that was evacuated an the bulk of the grayish,, soft friable, highly vascular tumor was resected. Histopathological examination showed the tumor cells with poor cytoplasm, oval nuclei and formed Homer Wright rosettes (Figure-2). A diagnosis of cerebellar medulloblastoma was made. Following surgery he improved in his headache, vomiting and cerebellar signs but there was no improvement in his vision. He was subjected to cranio spinal irradiation and is on regular follow up with no fresh symptoms.  Discussion   Up to 47% patients of medulloblastoma can have atypical features on CT scan5 and these include cystic or necrotic component, calcification, hemorrhage, lack of contrast enhancement, eccentric location, rarely direct transtentorial extension5,9. Cystic or necrotic component is less common in children than in adults5,10-12. Presence of these atypical features on CT scan can makeit difficult to differentiate a medulloblastoma from other posterior fossa tumors i.e. ependymoma and astrocytoma in children5. In a child is who have a central well-marginated tumor which exhibits uniformly increased density on pre-contrast scans and homogeneous contrast enhancement there is 85-90% likelihood it to be a medulloblastoma, 5 however the presence of atypical features on CT scan in the first decade of life should not be considered against the diagnosis of medulloblastoma. 5,9  References  1. Giordana MT, Schiffer P, Schiffer D. Prognostic factors in medulloblastoma. Childs Nerv Syst. 1998;14(6):256-62.  2. Naidich TP, Lin JP, Leeds NE, Pudlowski RM, Naidich JB. Primary tumors and other masses of the cerebellum and fourth yentricle: differential diagnosis by computed tomography. Neuroradiology 1977; 14: 153- 174. 3. Zimmerman RA, Bilaniuk LT, Pahlajani H. Spectrum of medulloblastomas demonstrated by computed tomography. Radiology 1978; 126:137-141. 4. Probst FP, Liliequist B. Assessment of posterior fossa tumors in infants and children by means of computed tomography. Neuroradiology 1979; 18:9-18. 5. Zee CS, Segall HD, Miller C et al  Less common CT features of medulloblastoma. Radiology 1982; 144 : 97-102. http://www.bioline.org.br/js  East and Central African Journal of Surgery Volume 14 Number 1 – March/April 2009          75 6. Maleci A, Cervoni L, Delfini R. Medulloblastoma in children and in adults: a comparative study. Acta Neurochir 1992; 119:62-67. 7. Haie-Meder C, Song PY. Medulloblastoma: differences in adults and children: regarding Frost et al., IJROBP 32:951-957; 1995 and Prados et al., IJROBP 32:1145-1152; 1995. Int J Radiation Oncology Biol Phys 1995;32:1255-1257. 8. Tortori-Donati P, Fondelli MP, Rossi A, et al. Medulloblastoma in children: CT and MRI findings. Neuroradiology 1996,38:352-359 9. Hubbard JL, Scheithauer BW, Kispert DB, et al. Adult cerebellar medulloblastomas: the pathological, radiographic, and clinical disease spctrum. J Neurosurg 1989;70:536-544. 10. Kingsley DPE, Kendall BE. The CT scanner in posterior fossa tumors of childhood. Br J Radiol 1979; 52:769-776. 11. Mahapatra AK, Paul HK, Sarkar C. Cystic medulloblastoma. Neuroradiology. 1989;31(4):369-70. 28. Bourgouin PM, Tampieri D, Grahovac SZ et al : CT and MRI imaging findings in adults with cerebellar medulloblastoma : Comparison with findings in children. Am Jr Roentgen 1992; 159: 609-612.

Cystic Medulloblastoma in a child

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Cystic Medulloblastoma in a child

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