Department of Neurosurgery, La Princesa University Hospital, C/Diego de León 62, 28006, Madrid, Spain.
Department of Neurosurgery, Puerta de Hierro University Hospital, Madrid, Spain.
Pituitary. 2018 Aug;21(4):393-405. doi: 10.1007/s11102-018-0889-z.
A heterogeneous group of epithelial cystic tumors developed at the infundibulum and the third ventricle disconcerted pathologists at the dawn of the twentieth century. Very little was known at that time about the physiological role played by the pituitary gland, and there was almost complete ignorance regarding the function of the hypothalamus. Acromegaly, or enlargement of acral body parts, described in 1886 by Pierre Marie, was the only disease linked to primary hypertrophies of the pituitary gland, known as "pituitary strumas". A growing number of young patients manifesting an unexplained combination of physical and mental symptoms, including absent or delayed sexual maturation, progressive obesity, abnormal somnolence, and dementia-like changes in behavior were reported to present large solid-cystic tumors which characteristically expanded within the infundibulum and third ventricle, above an anatomically intact pituitary gland. Between 1899 and 1904, five seminal autopsy studies from different countries thoroughly described the anatomical relationships and histological features of this newly recognized type of infundibular tumors. These cases were instrumental in fostering the systematic investigation of similar lesions by the Austrian pathologist Jakob Erdheim (1874-1937), who in 1904 was able to classify these infundibulo-tuberal cysts under the common category of hypophyseal duct tumors. The pioneering American neurosurgeon Harvey Cushing (1869-1939) unsuccessfully attempted to surgically remove one of these cysts, for the first time in history, in 1902. The term "craniopharyngioma", chosen by Cushing in 1929 to designate these lesions, would eventually prevail over Erdheim's more accurate denomination, which linked their origin to squamous cell remnants derived from the embryological structures that give rise to the pituitary gland. This paper presents a comprehensive, renewed account of the five clinical-pathological reports which laid the groundwork for the proper clinical diagnosis, topographic conceptualization and pathological categorization of craniopharyngiomas.
在 20 世纪初，一种在漏斗和第三脑室发育的异质上皮性囊腺瘤使病理学家感到困惑。当时，人们对垂体的生理功能几乎一无所知，对下丘脑的功能也几乎一无所知。肢端肥大症，即四肢部分的增大，1886 年由皮埃尔·玛丽（Pierre Marie）描述，是唯一与垂体原发性肥大相关的疾病，被称为“垂体腺瘤”。越来越多的年轻患者表现出无法解释的身体和精神症状的组合，包括性成熟缺失或延迟、进行性肥胖、异常嗜睡和行为上类似痴呆的变化，这些患者表现出大的实性囊腺瘤，这些肿瘤特征性地在漏斗和第三脑室内部扩张，位于解剖上完整的垂体上方。在 1899 年至 1904 年期间，来自不同国家的五项开创性尸检研究彻底描述了这种新认识的漏斗肿瘤的解剖关系和组织学特征。这些病例有助于奥地利病理学家雅各布·埃尔德海姆（Jakob Erdheim）对类似病变进行系统研究，他在 1904 年能够将这些漏斗-管肿瘤归类为垂体管肿瘤。美国神经外科先驱哈维·库欣（Harvey Cushing）于 1902 年首次尝试手术切除其中一个囊肿，这在历史上尚属首次。库欣于 1929 年选择的术语“颅咽管瘤”最终取代了埃尔德海姆更准确的命名，该命名将其起源与来自形成垂体的胚胎结构的鳞状细胞残余物联系起来。本文全面更新了奠定颅咽管瘤正确临床诊断、拓扑概念化和病理分类基础的五项临床病理报告。
