Craniopharyngioma

Craniopharyngioma occurs at any age, although more often in children, accounting for 5% to 10% of childhood intracranial tumors. It is a slowly growing tumor that originates from remnant epithelial cells of the craniopharyngeal duct (Rathke pouch). It is situated in

figure 1 1.36

Malignant peripheral nerve tumor. The tumor shows great hypercellularity, with spindle-shaped cells forming bundles, nuclear pleomorphism, and multinucleated giant cell.

figure 1 1.36

Malignant peripheral nerve tumor. The tumor shows great hypercellularity, with spindle-shaped cells forming bundles, nuclear pleomorphism, and multinucleated giant cell.

the suprasellar region, less often in the sella, and may extend into the third ventricle.

Clinically, the tumor presents with visual impairment, endocrine and hypothalamic dysfunction, and symptoms and signs of raised ICP. Cognitive decline and personality changes are not unusual. The sella turcica may be enlarged, and calcifications are present on radiograph. CT scan and MRI show mixed signal intensities due to cystic and solid components and calcifications. The solid component enhances with contrast in CT and Tl-weighted MR images.

Grossly, most craniopharyngiomas are cystic with a thick, machine-oil-like contents. The solid portion of the tumor forms nodules in the cyst's wall (Fig. 11.37). Histologically, the tumor consists of multistratified squamous epithelial cells. Two types are distinguished:

• The adamantinomatous type, in which the cells form strands and cords, and calcifications, amorphous masses of keratin (wet keratin), and cholesterol clefts are characteristic (see Fig. 11.37A,B).

• The papillary type, in which the cells rest on a fibro-vascular stoma. This type lacks calcifications and cholesterol crystals. Glial reaction and Rosenthal fibers are conspicuous around the tumor, which

figure 1 i.37a

Craniopharyngioma. A 55-year-old woman presented with headaches and declining memory of 3 years' duration and excessive daytime sleepiness of 1 year's duration. On examination, the optic discs were pale, and the visual acuity severely diminished. She lay motionless and speechless with a vacant expression, resembling akinetic mutusim. Her temperature was intermittently elevated, and she needed to be spoon-fed. A. Sagittal section of the brain shows a large cyst with small tumorous mural modules. The cyst is loosely attached to the infundibulum, compresses the diencephalons, and obliterates the third ventricle. The lumen is filled with machine-oil-like material. B. The mural nodules consist of squamous epithelial cells arranged in small islands (adamantinomatous pattern) (HE).

figure 1 i.37a

Craniopharyngioma. A 55-year-old woman presented with headaches and declining memory of 3 years' duration and excessive daytime sleepiness of 1 year's duration. On examination, the optic discs were pale, and the visual acuity severely diminished. She lay motionless and speechless with a vacant expression, resembling akinetic mutusim. Her temperature was intermittently elevated, and she needed to be spoon-fed. A. Sagittal section of the brain shows a large cyst with small tumorous mural modules. The cyst is loosely attached to the infundibulum, compresses the diencephalons, and obliterates the third ventricle. The lumen is filled with machine-oil-like material. B. The mural nodules consist of squamous epithelial cells arranged in small islands (adamantinomatous pattern) (HE).

figure 1 i.37b

Craniopharyngioma in a 75-year-old man. A. A contrast CT scan shows a large cystic mass within the third ventricle. B. Macrosection of the craniopharyngioma within the third ventricle (LFB-CV). The mural nodules contain (C) squamous epithelial cells and (D.) homogenous keratin material. E. The cyst wall is loosely attached to the ventricular wall; it contains astrocytic fibers and Rosenthal fibers (HE).

adheres to the neural parenchyma and sometimes invades it.

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