Elarjani Turki, Alhuthayl Meshari Rashed, Alhindi Hindi, Kanaan Imad N
Department of Neurological Surgery, University of Miami MILLER School of Medicine Jacson Memorial Hospital, Miami, Florida, United States.
Neurosciences Center, King Faisal Specialist Hospital and Research Center.
Surg Neurol Int. 2021 Oct 25;12:539. doi: 10.25259/SNI_664_2021. eCollection 2021.
Malignant craniopharyngioma is a rare tumor with few published case reports. It can form or transform from a benign variant and is associated with a dismal survival rate. We reviewed the literature for all published cases and studied the effect of radiation on the rate of malignant transformation. We analyzed the effect of chemotherapy on survival.
We used various search engines to locate literature from 1980 onward and identified 31 case reports, one of which was excluded. Statistical analysis using the SAS software was conducted, and a significant value was identified if < 0.05.
There was equal distribution among male and female patients. The average age at malignant diagnosis is 31.11 years (±15.16) and 12.19 years (±8.41) for the average interval of benign tumor progression to malignancy. The most common clinical presentation was visual loss and/or field deficits in 26/30 patients (86%). Almost 11/30 patients (37%) had endocrinological deficits, with panhypopituitarism as the most common in 8/11 patients (73%). Fifteen patients received radiation before malignant transformation (47%) and demonstrated no effect on malignant transformation ( = 0.379). Gross total resection was achieved in 2/30 patients. The average time to mortality postoperatively is 5.3 months ± 4.3. Ten patients received chemotherapy, and five were alive at last follow-up ( = 0.115).
Malignant craniopharyngioma carries a dismal prognosis with no apparent benefits of radiation therapy and chemotherapy on survival.
恶性颅咽管瘤是一种罕见肿瘤，发表的病例报告较少。它可由良性变体形成或转变而来，且生存率较低。我们回顾了所有已发表病例的文献，并研究了放疗对恶性转化发生率的影响。我们分析了化疗对生存率的影响。
我们使用各种搜索引擎查找1980年以来的文献，共识别出31例病例报告，其中1例被排除。使用SAS软件进行统计分析，若P<0.05则确定为有显著意义。
男性和女性患者分布均衡。恶性诊断时的平均年龄为31.11岁（±15.16），良性肿瘤进展至恶性的平均间隔时间为12.19岁（±8.41）。最常见的临床表现是视力丧失和/或视野缺损，26/30例患者（86%）出现此症状。近11/30例患者（37%）有内分泌缺陷，其中全垂体功能减退在8/11例患者（73%）中最为常见。15例患者在恶性转化前接受了放疗（47%），且放疗对恶性转化无影响（P = 0.379）。30例患者中有2例实现了全切除。术后平均死亡时间为5.3个月±4.3。10例患者接受了化疗，最后一次随访时有5例存活（P = 0.115）。
恶性颅咽管瘤预后较差，放疗和化疗对生存率无明显益处。
