New outlook on the diagnosis, treatment and follow-up of childhood-onset craniopharyngioma

Abstract

28Childhood-onset craniopharyngiomas are rare embryonal malformations of low-grade

Hypothalamic involvement in craniopharyngioma—Implications for surgical, radiooncological, and molecularly targeted treatment strategies

“…Accordingly, it is controversial whether personalized molecularly targeted treatment approaches could significantly change these frustrating perspectives for patients with primary hypothalamic involvement of CP. Unfortunately, treatment options for hypothalamic obesity and neuropsychological deficits due to hypothalamic syndrome are not yet available . Future studies analyzing molecular mechanisms leading to hypothalamic invasion and controlled trials on treatment for hypothalamic comorbidities after CP are warranted …”

“…However, long‐term quality of survival is impaired due to disease and/or treatment‐related effects on hypothalamic–pituitary axes and optic structures. The incidence rate in CP is 0.5–2.0 cases per 1,000,000 population/year . A bimodal age distribution with peak incidence rates in children of ages 5–14 years and adults of ages 50–74 years has been reported.…”

“…Novel findings for molecular pathology of pCP, that is, activating mutations in BRAF (V600E), resulted in the development of personalized, molecularly targeted therapies . Further treatment options for CP consist of neurosurgical resection, irradiation, and intracystic chemotherapy (interferon‐alpha) . In the past, radical surgical strategies such as gross‐total resection were the preferred treatment of choice for CP.…”

“…Radiation therapy is effective in achieving long‐term disease control in childhood CP . With regard to current recommendations on less radical surgical strategies, resulting in higher rates of residual tumors, modern techniques of irradiation play a major role in current multidisciplinary treatment approaches for CP.…”

“…Advances in development of radio oncological treatment modalities such as proton beam therapy allow for more reduction of target volume and increased margins of safety. The results of prospective trials comparing treatment efficacy and neuropsychological and cognitive late effects between patients treated with proton beam therapy and modern methods of photon irradiation should be available soon …”