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Neuroradiology Case of
the Week
Case 233
Ashwani K. Sharma, MD and P-L Westesson, MD, PhD, DDS
Clinical
Presentation: Patient is a two-month-old male who presents with bulging fontanelles.
Imaging Findings: MR study revealed a large isointense, strongly enhancing mass lesion with large cystic component in the right posterior cerebral hemisphere. There is no enhancement in the wall of the cystic component. There is perilesional edema and significant mass effect and midline shift to left side. In view of the large cystic with solid nodular enhancement and small age, radiological differential diagnosis includes: dysplastic infantile gangliocytoma, juvenile pilocytic astrocytoma, and primitive neuroectodermal tumor.
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Figure 1A&B: T1-weighted coronal (A) and axial (B) post-contrast images show a large mass lesion with cystic (9x8x8cm) and solid (5.5x5.2x5cm) enhancing nodular component in the right posterior cerebral hemisphere with mass effect and midline shift to left side. Mass is outside the ventricular system and is displacing the right lateral ventricle anteriorly.
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Diagnosis: Dysplastic infantile gangliocytoma
Discussion: Desmoplastic infantile gangliogliomas (DIG) are massive cystic tumors, typically occurring in the cerebral hemispheres of infants. Desmoplastic infantile ganglioglioma was first described by VandenBerg et al. [1] in 1987. Together with superficial astrocytoma, they are also grouped as desmoplastic supratentorial neuroepithelial tumors (DSNT) of infancy. DSNTs of infancy form a rare but distinctive group of tumors occurring in children less than 2 years of age.
DIGs are, for the most part, hemispheric in location. Majority are in the frontal and/or parietal regions, although temporal and, at times, occipital involvement has been recognized. Solid component is closely attached to the dura, and intensely enhances with contrast, surrounded by a parenchymal cystic component and mass effect. Contrast enhancement is intense in DIGs and extends to the meninges [2]. Regardless of whether the image is obtained on a CT or MRI scan, the size of the tumor is its most striking characteristic. Neither cerebrospinal fluid seeding nor extracranial metastases has been reported [3].
Most of these tumors are amenable to surgical excision because of their surface location and distinct firmness with clear demarcation from the surrounding normal brain. No adjuvant therapy is recommended after total excision, despite the fact that the lesion has a low malignant potential. Long-term prognosis after total surgical excision has been excellent in all the reported series.
References:
- VandenBerg SR, May EE, Rubinstein LJ, Herman MM, Perentes E, Vinores SA, Collins VP, Park TS. Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential ("desmoplastic infantile gangliogliomas"). Report on 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg. 1987 Jan;66(1):58-71. [Medline]
- Duffner PK, Cohen ME: The long-term effects of central nervous system therapy on children with brain tumors, in Cohen ME, Duffner PK (eds): Brain Tumors in Children. Philadelphia, W.B. Saunders Company, 1991, pp 479-495.
- Duffner PK, Burger PC, Cohen ME, Sanford RA, Krischer JP, Elterman R, Aronin PA, Pullen J, Horowitz ME, Parent A, et al. Desmoplastic infantile gangliogliomas: an approach to therapy. Neurosurgery. 1994 Apr;34(4):583-9; discussion 589. [Medline]
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