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Primitive neuroectodermal tumor

CLINICAL HISTORY:
This 63 year old man experienced sudden right facial nerve paresis and motor aphasia. Within 3 days there was good clinical recovery. A CT performed in another hospital was interpreted as a left middle cerebral artery distribution infarction. Now the patient notes episodes of the same complaints, with progressive right arm paresis.

NEUROLOGICAL EXAMINATION shows gait instability and at Romberg test a tendency to fall to the right. There is a motor aphasia, with paresis of the right arm and central facial nerve paresis.

MRI:
a) TRANSAXIAL T2-WEIGHTED (2500/90/1) SPIN-ECHO SEQUENCE (IMAGES 1 AND 2): A markedly inhomogeneous tumor is seen in the left pre-Rolandic region of. The lesion is ovoid, and composed of cystic components alternating with more hypointense solid components. Minimal midline shift is seen.

b) TRANSAXIAL T1-WEIGHTED (600/15/1) SPIN-ECHO SEQUENCE (IMAGES 3 AND 4): On this sequence the tumor appears more homogeneous. The internal structure of the tumor again becomes apparent, with a multilocular composition.

c) TRANSAXIAL (IMAGES 5 AND 6) AND CORONAL (IMAGE 7) GADOLINIUM-ENHANCED SPIN-ECHO SEQUENCES: The lesion shows inhomogeneous enhancement, with a more intensely enhancing nodule laterally, diffuse moderate enhancement of the non-cystic components and peripheral septa-like enhancement among the cystic parts of the tumor.

RADIOLOGICAL DIFFERENTIAL DIAGNOSIS is very broad, but points to a malignant tumor.

SURGICAL FINDINGS: At surgery, the tumor was soft, poorly vascularised, partly cystic and has a blue-grey appearance. The tumor macroscopically resembled a malignant glioma. Despite the radiological appearance the tumor was poorly delineated. Subtotal resection was performed. Adjuvant total cranial radiotherapy was given.

ANATOMOPATHOLOGY points to PNET Grade IV.

FOLLOW-UP: The patient died 6 months after surgery.

Search also:
Primary cerebral neuroblastoma (supratentorial PNET)

 

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Fig. 1

Primitive neuroectodermal tumor, Fig. 1
Primitive neuroectodermal tumor, Fig. 2
Primitive neuroectodermal tumor, Fig. 3
Primitive neuroectodermal tumor, Fig. 4
Primitive neuroectodermal tumor, Fig. 5
Primitive neuroectodermal tumor, Fig. 6
Primitive neuroectodermal tumor, Fig. 7