Mezmezian Mónica Beatriz, Fernandez Ugazio Gonzalo, Paparella María Luisa
Clin Neuropathol. 2020 Jan/Feb;39(1):25-31. doi: 10.5414/NP301189.
Malignant transformation in craniopharyngiomas is a very uncommon event and scarcely mentioned in the World Health Organization (WHO) Classification of Tumors of the Central Nervous System (CNS). So far, there are only 34 reported cases.
We report an additional case in a 63-year-old woman who was diagnosed with craniopharyngioma 47 years ago. We reviewed the literature in order to define the histological features of malignant craniopharyngioma and its overlap with odontogenic tumors.
Our case presented morphology of mixed odontogenic ghost cell/ameloblastic carcinoma. Analyzing all reported cases, 18 of them presented malignization as squamous cell carcinoma (SCC), 1 as odontogenic ghost cell carcinoma, 2 as ameloblastic carcinoma, and 10 cases were mentioned just as malignant craniopharyngiomas.
We concluded that SCC represented only half of the malignant cases, while the morphology of ~ 11% of them was comparable with ameloblastic or odontogenic ghost cell carcinomas and 28% lacked a specific histological diagnosis. Most cases were fatal, which makes it necessary to include the entity of malignant craniopharyngioma in the WHO Classification of Tumors of the CNS as a high-grade tumor defining its histological variability.
颅咽管瘤的恶性转化是一种非常罕见的事件,在世界卫生组织(WHO)中枢神经系统(CNS)肿瘤分类中很少被提及。到目前为止,仅有34例报道病例。
我们报告了1例63岁女性的病例,该患者47年前被诊断为颅咽管瘤。我们回顾了文献,以确定恶性颅咽管瘤的组织学特征及其与牙源性肿瘤的重叠情况。
我们的病例呈现出混合性牙源性影细胞/成釉细胞瘤的形态。分析所有报道病例,其中18例表现为鳞状细胞癌(SCC)恶变,1例为牙源性影细胞癌,2例为成釉细胞瘤,10例仅被提及为恶性颅咽管瘤。
我们得出结论,SCC仅占恶性病例的一半,约11%的病例形态与成釉细胞瘤或牙源性影细胞癌相似,28%缺乏特定的组织学诊断。大多数病例是致命的,这使得有必要将恶性颅咽管瘤作为一种高级别肿瘤纳入WHO中枢神经系统肿瘤分类,明确其组织学变异性。