Center for Cancer and Blood Disorders, Phoenix Children's Hospital, Phoenix, Arizona, USA.
Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Neuro Oncol. 2023 Feb 14;25(2):224-233. doi: 10.1093/neuonc/noac221.
Craniopharyngioma is a histologically benign tumor of the suprasellar region for which survival is excellent but quality of life is often poor secondary to functional deficits from tumor and treatment. Standard therapy consists of maximal safe resection with or without radiation therapy. Few prospective trials have been performed, and response assessment has not been standardized.
The Response Assessment in Pediatric Neuro-Oncology (RAPNO) committee devised consensus guidelines to assess craniopharyngioma response prospectively.
Magnetic resonance imaging is the recommended radiologic modality for baseline and follow-up assessments. Radiologic response is defined by 2-dimensional measurements of both solid and cystic tumor components. In certain clinical contexts, response to solid and cystic disease may be differentially considered based on their unique natural histories and responses to treatment. Importantly, the committee incorporated functional endpoints related to neuro-endocrine and visual assessments into craniopharyngioma response definitions. In most circumstances, the cystic disease should be considered progressive only if growth is associated with acute, new-onset or progressive functional impairment.
Craniopharyngioma is a common pediatric central nervous system tumor for which standardized response parameters have not been defined. A RAPNO committee devised guidelines for craniopharyngioma assessment to uniformly define response in future prospective trials.
颅咽管瘤是一种发生于鞍上区的组织学良性肿瘤，其生存率很高，但由于肿瘤和治疗引起的功能缺陷，生活质量往往较差。标准治疗包括最大限度的安全切除肿瘤，联合或不联合放射治疗。虽然已经开展了一些前瞻性试验，但尚未对其进行标准化的反应评估。
儿科神经肿瘤学反应评估（RAPNO）委员会制定了评估颅咽管瘤的前瞻性共识指南。
磁共振成像（MRI）是用于基线和随访评估的推荐影像学方法。影像学反应通过实性和囊性肿瘤成分的二维测量来定义。在某些临床情况下，根据其独特的自然史和对治疗的反应，可能会对实性和囊性疾病的反应进行不同的考虑。重要的是，委员会将与神经内分泌和视觉评估相关的功能终点纳入颅咽管瘤反应定义中。在大多数情况下，只有当囊性疾病的增长与新发或进行性功能损害相关时，才应考虑其为进展性疾病。
颅咽管瘤是一种常见的儿童中枢神经系统肿瘤，尚未定义其标准化的反应参数。RAPNO 委员会制定了颅咽管瘤评估指南，以便在未来的前瞻性试验中统一定义反应。
