José M. Roda, José M. Pascual and Pedro Borrego
Rodrigo Carrasco and José M. Pascual
Ruth Prieto and José M. Pascual
Norman McOmish Dott (1897–1973) developed surgical neurology in Edinburgh, Scotland, and was a scholar of worldwide renown. One of Dott's most notable contributions to neurosurgery was his understanding of hypothalamic physiology, mostly acquired through the comprehensive study of patients with lesions involving this region of the diencephalon, particularly craniopharyngiomas (CPs). Recognition of symptoms caused by hypothalamic disturbances allowed him to predict the accurate anatomical relationships between CPs and the hypothalamus, despite the rudimentary radiological methods available during the 1930s. His sophisticated knowledge permitted Dott to perform radical removals of CPs originating within the third ventricle floor with acceptable success. Between 1934 and 1937, he operated on 4 CP cases originating in the hypothalamus, achieving a satisfactory postoperative outcome in 3 of the 4 patients. Aware of the strong attachment of hypothalamic CPs to the infundibulo-tuberal area, Dott used a double transbasal and transventricular approach to these lesions, a strategy providing an optimal view and control of the tumor boundaries. The decisive mentorship of several legendary figures of physiology and neurosurgery greatly influenced Dott's surgical evolution. The experimental pituitary gland work he performed with Sir Edward Sharpey-Schäfer at the beginning of his career stirred Dott's curiosity about the issue of hypothalamus-pituitary relationships. As a result, he decided to move to Peter Bent Brigham Hospital (Boston, Massachusetts) in 1923, to train in neurosurgery and neuropathology under the guidance of the leaders in these fields, Harvey Williams Cushing (1869–1939) and Percival Sylvester Bailey (1892–1973). They inspired the young Dott and shared with him their clinical and pathological expertise, in addition to their surgical strategies for best approaching and removing these challenging tumors. In time, Dott would come to surpass his mentors. This paper aims to credit Norman M. Dott for his decisive, modern contributions to hypothalamic CP surgery.
José M. Pascual, Ruth Prieto and Paolo Mazzarello
Sir Victor Horsley (1857–1916) is considered to be the pioneer of pituitary surgery. He is known to have performed the first surgical operation on the pituitary gland in 1889, and in 1906 he stated that he had operated on 10 patients with pituitary tumors. He did not publish the details of these procedures nor did he provide evidence of the pathology of the pituitary lesions operated on. Four of the patients underwent surgery at the National Hospital for Neurology and Neurosurgery (Queen Square, London), and the records of those cases were recently retrieved and analyzed by members of the hospital staff. The remaining cases corresponded to private operations whose records were presumably kept in Horsley's personal notebooks, most of which have been lost.
In this paper, the authors have investigated the only scientific monograph providing a complete account of the pituitary surgeries that Horsley performed in his private practice, La Patologia Chirurgica dell'Ipofisi (Surgical Pathology of the Hypophysis), written in 1911 by Giovanni Verga, Italian assistant professor of anatomy at the University of Pavia. They have traced the life and work of this little-known physician who contributed to the preservation of Horsley's legacy in pituitary surgery. Within Verga's pituitary treatise, a full transcription of Horsley's notes is provided for 10 pituitary cases, including the patients' clinical symptoms, surgical techniques employed, intraoperative findings, and the outcome of surgery. The descriptions of the topographical and macroscopic features of two of the lesions correspond unmistakably to the features of craniopharyngiomas, one of the squamous-papillary type and one of the adamantinomatous type. The former lesion was found on necropsy after the patient's sudden death following a temporal osteoplastic craniectomy. Surgical removal of the lesion in the latter case, with the assumed nature of an adamantinomatous craniopharyngioma, was successful. According to the evidence provided in Giovanni Verga's monograph, it can be claimed that Sir Victor Horsley was not only the pioneer of pituitary gland surgery but also the pioneer of craniopharyngioma surgery.
Ruth Prieto and José M. Pascual
Percival S. Bailey (1892–1973) was a scholar, neuroscientist, neuropathologist, and neurosurgeon who made decisive contributions in the field of neuro-oncology. Far less known are his groundbreaking insights into understanding hypothalamic physiology through the study of craniopharyngiomas. As one of Harvey W. Cushing’s most talented trainees, Bailey was instrumental in developing Cushing’s project of a histologically based prognostic classification of brain tumors. He worked at Peter Bent Brigham Hospital on and off between 1919 and 1928, owing to frequent clashes with his mentor. A major cause of this long-term conflict was Bailey’s 1921 experimental demonstration of the hypothalamic origin of diabetes insipidus and Fröhlich’s syndrome. This finding challenged Cushing’s view that both alterations were due to pituitary gland insufficiency. In a seminal monograph written with John F. Fulton in 1929, both authors provided the first comprehensive account of the specific hypothalamic disturbances caused by tumors that originated within the infundibulum and third ventricle. The methodical study of Cushing’s craniopharyngioma specimens allowed Bailey to recognize the close contact between these lesions and hypothalamic nuclei, a key concept that Bailey originally advanced for proper surgical planning. This article aims to credit Bailey for his pioneering definition of craniopharyngiomas as tumors with a true intrahypothalamic position.
José R. Iglesias, Jesús Marin, Mercedes Salaices and Octavio Pascual
✓ The cerebral and cerebellar pia mater and arachnoid of 90 adults and 15 fetuses have been studied in order to determine whether the number of arachnoidal neural elements is sufficiently high to dismiss the possibility of their being purely heterotopic or displaced structures. With the aid of various histological techniques and formaldehyde (noradrenaline)-induced fluorescence, the presence of a reticulum composed of many short adrenergic neurons was demonstrated, intimately associated with the sympathetic perivascular innervation. Although this network seems to participate in some aspects of peripheral neuroendocrine function, its possible role in physiologically or pathologically regulating cerebral blood flow is as yet unsettled.
Conquest of third ventricle craniopharyngiomas
José M. Pascual, Ruth Prieto, Marta Navas and Rodrigo Carrasco
Jose M. Pascual
José María Pascual, Ruth Prieto, Rodrigo Carrasco and Laura Barrios
Accurate diagnosis of the topographical relationships of craniopharyngiomas (CPs) involving the third ventricle and/or hypothalamus remains a challenging issue that critically influences the prediction of risks associated with their radical surgical removal. This study evaluates the diagnostic accuracy of MRI to define the precise topographical relationships between intraventricular CPs, the third ventricle, and the hypothalamus.
An extensive retrospective review of well-described CPs reported in the MRI era between 1990 and 2009 yielded 875 lesions largely or wholly involving the third ventricle. Craniopharyngiomas with midsagittal and coronal preoperative and postoperative MRI studies, in addition to detailed descriptions of clinical and surgical findings, were selected from this database (n = 130). The position of the CP and the morphological distortions caused by the tumor on the sella turcica, suprasellar cistern, optic chiasm, pituitary stalk, and third ventricle floor, including the infundibulum, tuber cinereum, and mammillary bodies (MBs), were analyzed on both preoperative and postoperative MRI studies. These changes were correlated with the definitive CP topography and type of third ventricle involvement by the lesion, as confirmed surgically.
The mammillary body angle (MBA) is the angle formed by the intersection of a plane tangential to the base of the MBs and a plane parallel to the floor of the fourth ventricle in midsagittal MRI studies. Measurement of the MBA represented a reliable neuroradiological sign that could be used to discriminate the type of intraventricular involvement by the CP in 83% of cases in this series (n = 109). An acute MBA (< 60°) was indicative of a primary tuberal-intraventricular topography, whereas an obtuse MBA (> 90°) denoted a primary suprasellar CP position, causing either an invagination of the third ventricle (pseudointraventricular lesion) or its invasion (secondarily intraventricular lesion; p < 0.01). A multivariate model including a combination of 5 variables (the MBA, position of the hypothalamus, presence of hydrocephalus, psychiatric symptoms, and patient age) allowed an accurate definition of the CP topography preoperatively in 74%–90% of lesions, depending on the specific type of relationship between the tumor and third ventricle.
The type of mammillary body displacement caused by CPs represents a valuable clue for ascertaining the topographical relationships between these lesions and the third ventricle on preoperative MRI studies. The MBA provides a useful sign to preoperatively differentiate a primary intraventricular CP originating at the infundibulotuberal area from a primary suprasellar CP, which either invaginated or secondarily invaded the third ventricle.