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Neuroblastoma

CLINICAL HISTORY:
This 17-year old girl is known to have von Recklinghausen disease with "café-au-lait" spots and cutaneous neurofibromata. Now she suffers a partial complex epileptic seizure with secondary generalization.

NEUROLOGICAL EXAMINATION in the immediate post-ictal state shows a slight paresis and slight hypoesthesia on the right side.

CT:
a) NON-CONTRAST SCAN (IMAGE 1): A slightly hyperdense mass (arrowheads) is seen, possibly with some calcification, located in the left frontal paramedian region. The lesion is broadly inserted on the falx cerebri (arrow), which is slightly displaced to the right.

b) CONTRAST-ENHANCED SCAN (IMAGE 2): There is intense and homogeneous contrast enhancement of the lesion. Again it is obvious that the lesion is broadly implanted on the falx cerebri (arrowheads).

MRI:
a) TRANSAXIAL T2-WEIGHTED (2500/90/1) SPIN-ECHO SEQUENCE (IMAGE 3): The mass (arrowheads) is confirmed in the left frontal parasagittal region. Again it is obvious that the lesion is broadly implanted on the falx cerebri (small arrows). The overall signal of the lesion is hyperintense, but with some intratumoral signal drop-outs, possibly due to calcification or cysts.

b) TRANSAXIAL T1-WEIGHTED (600/15/1) SPIN-ECHO SEQUENCE (IMAGE 4): The lesion (arrowheads) is hypointense, again with some cysts or calcification.

c) TRANSAXIAL (IMAGE 5) AND CORONAL (IMAGE 6 AND 7) GADOLINIUM-ENHANCED SPIN-ECHO SEQUENCES: There is intense but inhomogeneous enhancement of the tumor. In the more inferior aspect some less enhancing regions can be recognized. Again the lesion is broadly implanted on the dura mater of the falx cerebri, with evidence of a small dural tail (arrowhead).

RADIOLOGICAL DIFFERENTIAL DIAGNOSIS: Based on the location in the parasagittal region and the broad implantation on the falx, diagnosis is made of falx meningioma.

SURGICAL FINDINGS: At surgery, a grey very soft, but well-defined tumor was found. The tumor was not inserted on the falx nor on the dura mater of the convexity. The major part of the tumor seemed to be extraaxial, but infiltrated the white matter of the left frontal lobe. Treatment was completed with craniospinal radiotherapy.

ANATOMOPATHOLOGY is neuroblastoma supratentorial PNET Grade IV.

FOLLOW-UP: The patient died 1 year later.

Search also:
Primary cerebral neuroblastoma (supratentorial PNET)

 

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Fig. 1

Neuroblastoma, Fig. 1
Neuroblastoma, Fig. 2
Neuroblastoma, Fig. 3
Neuroblastoma, Fig. 4
Neuroblastoma, Fig. 5
Neuroblastoma, Fig. 6
Neuroblastoma, Fig. 7