Balasubramanian Kishore, Andrade de Almeida Romulo Augusto, Kharbat Abdurrahman F, Haider Ali S, Dunn Ian F, Graffeo Christopher S
Texas A&M University College of Medicine, Houston, Texas, USA; Department of Neurosurgery, University of Oklahoma College of Medicine, Oklahoma City, Oklahoma, USA.
Department of Neurosurgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
World Neurosurg. 2025 Jan;193:754-769. doi: 10.1016/j.wneu.2024.10.051. Epub 2024 Nov 13.
Mixed gangliocytoma-pituitary adenomas (MGPAs) are very rare sellar neoplasms. Contemporary understanding of their natural history, clinical characteristics, optimal management strategies, and prototypical outcomes remains poorly understood.
Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, PubMed, Embase, Web of Science, and Cochrane were searched to include studies of patients with histologically confirmed mixed gangliocytomas. Clinical features, management strategies, and outcomes were analyzed.
Thirty three studies reporting 78 patients met study criteria and were included. Median age at diagnosis was 47 years, with a female predominance (71%). Common presenting symptoms included headache (49%), acromegaly (43%), and visual disturbance (20%). Growth hormone and prolactin were the most commonly elevated hormones. Magnetic resonance imaging identified local infiltration in 86% of patients, most frequently in the cavernous sinus. Transsphenoidal resection was the primary treatment strategy, which achieved a gross total resection in 43%. Postoperative pituitary function was normal in 92%. Median follow-up was 21 months, during which time 14% of patients required secondary intervention for persistent hormonal hypersecretion. Following these secondary interventions and as of last follow-up, radiographic and/or biochemical control was confirmed in all patients, with an overall survival rate of 97%.
MGPAs are rare, slow-growing tumors that present with a combination of endocrinological and neurological symptoms. MGPAs are optimally managed with transsphenoidal resection, which appears to achieve favorable rates of symptomatic relief and local control despite the high incidence of local infiltration and relatively low attendant gross total resection rate.
混合性节细胞胶质瘤-垂体腺瘤（MGPAs）是非常罕见的鞍区肿瘤。目前对其自然病史、临床特征、最佳治疗策略及典型预后的了解仍很有限。
按照系统评价和Meta分析的首选报告项目指南，检索了PubMed、Embase、Web of Science和Cochrane数据库，纳入组织学确诊为混合性节细胞胶质瘤患者的研究。分析其临床特征、治疗策略及预后。
33项研究报告了78例符合研究标准的患者并被纳入。诊断时的中位年龄为47岁，女性占优势（71%）。常见的首发症状包括头痛（49%）、肢端肥大症（43%）和视力障碍（20%）。生长激素和催乳素是最常升高的激素。磁共振成像显示86%的患者有局部浸润，最常见于海绵窦。经蝶窦切除术是主要的治疗策略，43%的患者实现了全切除。92%的患者术后垂体功能正常。中位随访时间为21个月，在此期间14%的患者因持续性激素分泌过多需要二次干预。经过这些二次干预后，截至最后一次随访，所有患者均确认影像学和/或生化指标得到控制，总生存率为97%。
MGPAs是罕见的、生长缓慢的肿瘤，表现为内分泌和神经症状的组合。经蝶窦切除术是MGPAs的最佳治疗方法，尽管局部浸润发生率高且全切除率相对较低，但该方法似乎能取得良好的症状缓解率和局部控制率。
