Our patient was diagnosed as having Gorlin-Goltz syndrome with numerous BCCs and
falx cerebri calcifications, which are among the major criteria, and macrocephaly, frontal bossing, hypertelorism, and presence of medullablastoma, which are among the minor criteria.
The edematous range determined a mass effect over the left LV, which it compressed, and over the
falx cerebri, with a 7-8 mm right-deviation.
In this reported case, our patient had 3 of the major criteria, namely, calcification of
falx cerebri and cerebellum, palmar pits, andbasal cell carcinomawhich existedbefore the age of 20.
Facial abnormalities incude macrocephaly, broad facies, frontal and biparietal bossing, mild mandibular prognathism, odontogenic keratocysts ofjaws, misshapen and/or carious teeth, cleft lip and palate, ectopic calcification of
falx cerebri.
The wound trace stops at the
falx cerebri. No gross penetration of the cerebrum.'
Our subsequent studies of unembalmed cadavers went on to reveal fascia [tissues] hanging from the free border of the
falx cerebri [the projection, of the dura mater--the fibrous membrane forming the outermost covering of the brain and spinal cord--separating the hemispheres of the cerebrum--the largest portion of the brain].
Chondrosarcoma arising from the
falx cerebri. SurgNeruol.
1) compressing the left frontal lobe and abutting
falx cerebri, which shows intense homogenous enhancement on T1 post contrast study (Fig.
No evidence of calcification of
falx cerebri was detected during the CT scan.
At autopsy, the dura of the anterior
falx cerebri was indurated and thickened (as much as 0.5 cm).
Coronal imaging of the brain (Figure 3) demonstrates a monoventrical with lack of interhemispheric fissure and
falx cerebri. At birth a single midline orbit and a probiscus were present.
Other common abnormalities include ectopic calcifications of the
falx cerebri, tentorium cerebelli, and diaphragma sellae, and rib abnormalities, such as bifid, fused, or hypoplastic ribs.