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Craniopharyngioma, case 2

CLINICAL HISTORY:
This 16-year old girl has primary amenorrhea. For the past 6 months she complains of headache. Two months ago she suffered a sudden bifrontal headache with neck pain. This was accompanied by blurred vision, diplopia, tinnitus on the left and paresthesias in the 3rd and 4th fingers of the left hand.

NEUROLOGICAL EXAMINATION reveals bilateral papilledema. The patient has a cushingoid facies. There is oculomotor and abducens nerve paresis on the left side and diminished visual acuity on the left side.

CT:
a) NON-CONTRAST SCAN (IMAGE 1): A densely calcified mass is seen (arrow) in the suprasellar region.

b) CONTRAST-ENHANCED SCAN (IMAGES 2 AND 3): The mass is composed of a densely calcified component (arrow image 2) immediately above the sella turcica. More superiorly a cystic component (arrow image 3) is present, occupying almost the entire 3rd ventricle.

MRI:
a) TRANSAXIAL T2-WEIGHTED (2500/90/1) SPIN-ECHO SEQUENCE (IMAGES 4 AND 5): Both components, the calcified portion (arrow image 4) and the cystic component (arrowheads image 5), can easily be recognized. Almost total obliteration of the 3rd ventricle is noted as is supratentorial hydrocephalus.

b) CORONAL T1-WEIGHTED (440/20/1) SPIN-ECHO SEQUENCE (IMAGE 6): The mass is isointense to brain. The lesion extends up to the foramen of Monro (arrow) with substantial elevation of both lateral ventricles.

c) CORONAL (IMAGES 7, 8 AND 9) AND SAGITTAL (IMAGE 10) GADOLINIUM-ENHANCED SPIN-ECHO SEQUENCES: There is inhomogeneous enhancement of the inferior, calcified part of the lesion (arrowheads). The cystic component within the 3rd ventricle shows linear enhancement of its wall. Multiple daughter cysts (arrow) are found within the wall of the cyst.

RADIOLOGICAL DIAGNOSIS is calcified craniopharyngioma.

SURGICAL FINDINGS: At surgery, performed through a transventricular approach, a cystic mass was seen bulging into the foramen of Monro. Through the cyst wall cholesterol crustals could be seen, attached to the wall. The entire 3rd ventricle appeared to be occupied by a large thick walled cyst. After opening of the cyst a yellow-green substance was be evacuated. Fragments of the calcified portion of the tumor were removed, but the part that was firmly inserted on the hypothalamus was left in place. Treatment was completed with radiotherapy.

ANATOMOPATHOLOGY (IMAGE 11) adamantinomatous craniopharyngeoma, gr. I: cysts delineated by squamous epithelial tumor cells. Note the peripheral nuclear palisading (H&E).

FOLLOW-UP: After one year the lesion is now stable on Ct. The oculomotor problems have disappeared and the visual field is increasing.

 

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Fig. 1

Craniopharyngioma, case 2, Fig. 1
Craniopharyngioma, case 2, Fig. 2
Craniopharyngioma, case 2, Fig. 3
Craniopharyngioma, case 2, Fig. 4
Craniopharyngioma, case 2, Fig. 5
Craniopharyngioma, case 2, Fig. 6
Craniopharyngioma, case 2, Fig. 7
Craniopharyngioma, case 2, Fig. 8
Craniopharyngioma, case 2, Fig. 9
Craniopharyngioma, case 2, Fig. 10
Craniopharyngioma, case 2, Fig. 11