1. Clinical case vignette

A 42-year-old woman presented, as a transfer from an outside hospital, with increased forgetfulness, fatigue, as well as intermittent double vision leading to accidents. In addition, she complained of increased thirst and urination. She was concurrently taking lithium and lorazepam for psychiatric reasons. Imaging with CT revealed a large suprasellar mass extending into the third ventricle (Figure 1). The patient had laboratory studies performed (Table 1) and underwent a formal ophthalmology examination, which revealed red desaturation and a depressed visual field consistent with compressive optic neuropathy.

© 2016 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and eproduction in any medium, provided the original work is properly cited. © 2018 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

3. Subtypes of craniopharyngioma

4. Origin of craniopharyngioma

5. Clinical presentation

Craniopharyngiomas are benign lesions which arise from the neuroepithelium in the sellar region. They are classically subdivided into two distinct entities based on both genetic and morphologic differences. Adamantinomatous CP (aCP), primarily seen in childhood, occurs more commonly than papillary CP (pCP), which is more often seen in adults [2]. In histologic sections, adamantinomatous CP is poorly circumscribed, often multi-cystic and calcified, and is associated with β-catenin and epidermal growth factor receptor (EGFR) overexpression. Papillary CP, on the other hand, is well-circumscribed, less calcified, characterized by solid components, and displays less adherence to surrounding structures [2]. Furthermore, pCP is made up of fibrovascular stroma lined by well-differentiated squamous epithelium [2, 3].

Modern Management of Craniopharyngioma http://dx.doi.org/10.5772/intechopen.74512 205

In terms of histologic appearance, aCP usually shows nests and trabeculae of epithelium in fibrocollagenous stroma, with peripheral cells showing nuclear palisading, loose central cells termed "stellate reticulum," and abundant keratin, cholesterol crystals, necrosis, and inflammation. Papillary CP is well circumscribed, composed of the cores of fibrovascular stroma lined by well-differentiated squamous epithelium that may separate to form pseudopapillae which resembles squamous papilloma and without xanthogranulomatous inflammation. In molecular staining of these tumors, the lack of expression of CK8 and CK20 keratin suggests a craniopharyngioma, which differentiates them from Rathke's cleft cyst or pituitary pars intermedia. More recently, VE1 staining has also been utilized to identify BRAF mutations which can help to differentiate between Rathke's cleft cyst and craniopharyngioma [4].

Craniopharyngiomas were long thought to arise as an embryonic malformation from the anterior superior margin of the pituitary from residual Rathke's pouch. Due to their embryonic origin, they may even co-opt the blood supply of the wall and floor of the ventricle. More modern studies have demonstrated that aCP can arise due to paracrine actives of β-Catenin

A triad of symptoms, involving visual impairment, neurological decline, and cognitive compromise, is generally seen in patients presenting with CP. The extent of morbidity associated with CP is closely related to both the specific tumor location and its size. Hypothalamic disease in patients can present as obesity (>50%), diabetes insipidus, thermoregulation disorder, somnolence, sleep apnea, and arrhythmia. Hypothalamic lesions, in particular, are associated with increased rates of neurocognitive decline, and the importance of these neuropsychological issues is evident in that fact that many of these patients continue to report cognitive issues at follow-up, preventing return to previous performance at work or school. Clinically significant hypopituitarism, usually involving several anterior pituitary hormones, occurs in the majority

mutated cells, whereas pCP can arise via metaplastic transformation [2].

Figure 1. A–C. 4.0 3.3 4.0 cm, suprasellar mass with cystic and solid components extending into the third ventricle. No calcifications or hydrocephalus is seen.


Table 1. Patient's laboratory findings.
