5. Clinical presentation

2. Overview

No calcifications or hydrocephalus is seen.

204 Brain Tumors - An Update

Table 1. Patient's laboratory findings.

>55 years.

Craniopharyngiomas (CPs) have had a prominent place in neurosurgery due to both the technical difficulty and controversy regarding the optimal treatment of these benign tumors. Harvey Cushing famously described craniopharyngiomas in 1936 as "the most forbidding of the intracranial tumors." Seventy years later, Rutka still wrote: "There is no other primary brain tumor that evokes more passion, emotion, and as a result, controversy than does the CP" [1]. Craniopharyngiomas comprise 1–2% of all brain tumors and occur in a bimodal distribution, with 40% of cases occurring between age 5–15 years and 60% occurring at ages

Figure 1. A–C. 4.0 3.3 4.0 cm, suprasellar mass with cystic and solid components extending into the third ventricle.

Thyroid function tests TSH 0.17, T4 4.9, T3 71, Free T4 0.8

Laboratory study Result Urine specific gravity 1.020 Prolactin 13

Luteinizing hormone < 0.1 Follicle stimulating hormone < 0.1 IGF-1 201

The differential diagnosis for craniopharyngioma can include a variety of entities, including pituitary macroadenoma, metastasis, Rathke's cleft cyst, colloid cyst, glioma, meningioma, germinoma, abscess, sarcoid, or aneurysm. Imaging characteristics usually include a solid cystic lesion, speckled with calcifications in 50–80% of craniopharyngiomas (especially pediatric patients), as well a presentation with hypopituitarism and diabetes insipidus, which influ-

ence clinical thinking toward establishing this diagnosis.

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 of patients presenting with CP. In nearly 90% of patients who are present with hypopituitarism in the long term, there is a significantly higher mortality risk related to both cardiovascular and cerebrovascular mortality, with an especially higher risk in females compared to males. The evidence from multiple cohorts of patients suggests that the increased exposure to sex hormones also manifests as cardiovascular risk greater in females compared to males [5].
