Craniopharyngiomas (CPs) are clinically aggressive tumors because of their invasive behavior and recalcitrant tendency to recur after therapy. There are 2 types based on their distinct histology and molecular features: the papillary craniopharyngioma (PCP), which is associated with BRAF-V600E mutations and the adamantinomatous craniopharyngioma (ACP), characterized by mutations in CTNNB1 (encoding β-catenin). Patients with craniopharyngioma show symptoms linked to the location of the tumor close to the optic pathways, hypothalamus, and pituitary gland, such as increased intracranial pressure, endocrine deficiencies, and visual defects. Treatment is not specific and mostly noncurative, and frequently includes surgery, which may achieve gross total or partial resection, followed by radiotherapy. In cystic tumors, frequent drainage is often required and intracystic instillation of drugs has been used to help manage cyst refilling. More recently targeted therapies have been used, particularly in PCP, but also now in ACP and clinical trials are underway or in development. Although patient survival is high, the consequences of the tumor and its treatment can lead to severe comorbidities resulting in poor quality of life, in particular for those patients who bear tumors with hypothalamic involvement. Accordingly, in these patients at risk for the development of a hypothalamic syndrome, hypothalamus-sparing treatment strategies such as limited resection followed by irradiation are recommended. In this review, we provide an update on various aspects of CP, with emphasis on recent advances in the understanding of tumor pathogenesis, clinical consequences, management, and therapies.

中文翻译：

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颅咽管瘤的当代生物学见解和临床管理。

颅咽管瘤 (CPs) 是临床侵袭性肿瘤，因为它们具有侵袭性行为和治疗后顽固的复发倾向。根据其不同的组织学和分子特征，有 2 种类型：与 BRAF-V600E 突变相关的乳头状颅咽管瘤 (PCP) 和以 CTNNB1（编码 β- 连环蛋白）突变为特征的硬质颅咽管瘤 (ACP)。颅咽管瘤患者表现出与肿瘤靠近视神经通路、下丘脑和垂体的位置相关的症状，例如颅内压升高、内分泌不足和视力缺陷。治疗不是特异性的，而且大多是非治愈性的，通常包括手术，手术可能会完全或部分切除，然后进行放疗。在囊性肿瘤中，经常需要经常引流，囊内药物滴注已被用于帮助控制囊肿再充盈。最近使用了靶向治疗，特别是在 PCP 中，但现在也在 ACP 中使用，临床试验正在进行或正在开发中。虽然患者存活率很高，但肿瘤及其治疗的后果可能导致严重的合并症，从而导致生活质量差，特别是对于那些患有下丘脑受累肿瘤的患者。因此，在这些有发生下丘脑综合征风险的患者中，建议采用保留下丘脑的治疗策略，例如有限切除后放疗。在这篇综述中，我们提供了 CP 各个方面的更新，重点是对肿瘤发病机制、临床后果、