9.12.8Craniopharyngioma

9.12.8.1Incidence, Pathogenesis, and Pathology

• Up to 5% of all brain tumors

• Solid and cystic parts of epithelial tissue containing keratin, often with calcification (radiologically visible)

• Histologically well-differentiated tissue with malignant clinical course infiltrating the surrounding normal structures and tissue

– Two subtypes: (1) Adamantinomatous type resembling the embryonic tooth and (2) papillary type

• Extends into intraor suprasellar area

• May result in destruction of adjacent or neural bony structures

9.12.8.2Differential Diagnosis

•Residual tissue of the embryonic Rathke pouch is believed to be the origin

•Extensive optic glioma or suprasellar germ cell tumor

9.12.8.3 Clinical Manifestations

•Headache, vomiting, visual field deficiencies, blindness

•Growth retardation in about 50% of children

•Variable endocrine deficiencies, with delayed puberty

•Neurobehavioral abnormalities

•Growth hormone deficiency in over 70% of children

9.12.8.4 Radiological Diagnosis

CT and MRI reveal a cystic mass, often with calcifications

9.12.8.5 Therapy

•Surgical procedure:

–Upfront surgery is not necessary in all patients, depending on clinical manifestation

–Tumor excision of focal tumor with small risk of neurological or endocrinological sequelae, especially of cystic parts of tumor

–Preoperative endocrine substitution therapy

–Where there is a significant risk of endocrine dysfunction, neurological morbidity, and neurobehavioral disturbances, partial tumor resection followed by irradiation may be considered

•Radiotherapy:

–In radical resection of tumor: 40–50 Gy, but higher doses with hyperfractionation

–High rate of morbidity

•Chemotherapy:

–Only in exceptional situations, depending on tumor type (diffuse infiltrating character)

–Effective drugs and combinations: see above under “Astrocytic Tumors”