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José María Pascual and Ruth Prieto

From the very beginning of his career, Harvey Williams Cushing (1869–1939) harbored a deep interest in a complex group of neoplasms that usually developed at the infundibulum. These were initially known as “interpeduncular” or “suprasellar” cysts. Cushing introduced the term “craniopharyngioma” for these lesions, which he believed represented one of the most baffling problems faced by neurosurgeons. The patient who most influenced Cushing's thinking was a 16-year-old seamstress named “Mary D.,” whom he attended in December 1901, exactly the same month that Alfred Fröhlich published his seminal article describing an adiposogenital syndrome in a young boy with a pituitary cyst. Both Cushing's and Fröhlich's patients showed similar symptoms caused by the same type of tumor. Notably, Cushing and Fröhlich had met one another and became good friends in Liverpool the summer before these events took place. Their fortunate relationship led Cushing to realize that Fröhlich's syndrome represented a state of hypopituitarism and provided a useful method of diagnosing interpeduncular cysts. It is noteworthy that Cushing's very first neurosurgical procedure on a pituitary tumor was performed in the case of Mary D.'s “interpeduncular cyst,” on February 21, 1902. Cushing failed to remove this lesion, which was later found during the patient's autopsy. This case was documented as Pituitary Case Number 3 in Cushing's masterpiece, The Pituitary Body and Its Disorders, published in 1912. This tumor was considered “a teratoma”; however, multiple sources of evidence suggest that this lesion actually corresponded to an adamantinomatous craniopharyngioma. Unfortunately, the pathological specimens of this lesion were misplaced, and this prompted Cushing's decision to retain all specimens and documents of the cases he would operate on throughout his career. Accordingly, Mary D.'s case crystallized the genesis of the Cushing Brain Tumor Registry, one of Cushing's major legacies to neurosurgery. In this paper the authors analyze the case of Mary D. and the great influence it had on Cushing's conceptions of the pituitary gland and its afflictions, and on the history of pituitary surgery.

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Ruth Prieto and José María Pascual

The decisive role Dr. Harvey Cushing (1869–1939) played in medicine goes far beyond the development of neurosurgery. His scientific devotion and commitment to patient care made him an ethical model of strict professionalism. This paper seeks to analyze the decisions Cushing made with the challenging case of HW, an adolescent boy with a craniopharyngioma (CP) involving the third ventricle. Cushing’s earlier failure to successfully remove two similar lesions alerted him to the proximity of HW’s tumor and the hypothalamus. Consequently, he decided to use the chiasm-splitting technique for the first time, with the aim of dissecting the CP-hypothalamus boundaries under direct view. Unexpectedly, HW suffered cardiac arrest during the surgery, but Cushing did not give up. He continued with the operation while his assistants performed resuscitation maneuvers. Such determined and courageous action allowed Cushing to succeed in an apparently hopeless case. Cushing’s unwavering willingness to save patients’ lives, even under extreme circumstances, was a fundamental trait defining his identity as a neurosurgeon. Analyzing the way Cushing dealt with HW’s case provides valuable lessons for neurosurgeons today, particularly the importance of assuming proactive attitudes and, in certain cases, making painstaking efforts to overcome daunting situations to save a life.

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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.

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José María Pascual, Ruth Prieto and Laura Barrios

OBJECTIVE

Harvey Cushing (1869–1939) coined the term “craniopharyngioma” (CP) in 1929 to describe a kaleidoscopic group of epithelial tumors involving the hypothalamic-pituitary axis. Throughout his career, he endured a long struggle to accurately diagnose and safely remove these complex lesions, and his resulting surgical series has never before been analyzed in depth. The authors here conduct such an analysis.

METHODS

In this study, the authors retrospectively examined the CP patient records available in the Cushing Brain Tumor Registry, as well as those CP cases reported by Cushing in medical monographs and scientific reports.

RESULTS

Cushing’s CP series comprises a total of 124 tumors (CP124) compatible with a CP diagnosis. Among this series are 92 cases that could be pathologically verified (CP92). This subcohort showed a bimodal age distribution (41% aged ≤ 19 years old) and a balanced sex distribution. Clinical evolution up to diagnosis was longer than 3 years in half of the patients. Typical symptoms found at diagnosis were severe headache (94%), visual deficits (97%), panhypopituitarism (76%), psychiatric disturbances (47%), and abnormal somnolence (47%). The highest rate of endocrine deficits occurred in patients younger than 19 years of age (p < 0.001), whereas hypothalamic disturbances were observed mainly in adults between 30 and 49 years (p = 0.02). Hydrocephalus was present in 63% of the patients, predominantly involving the younger subgroup (p < 0.001). Preoperative diagnosis was based on clinical signs, funduscopic exams, and skull radiographs, the latter study showing suprasellar calcifications in 64% of cases. The majority of tumors (61%) had developed within the third ventricle (3V) or had invaded it. The adamantinomatous histological variant was the predominant one (73%). Squamous-papillary CPs occurred only in adults older than 40 years of age (p < 0.001). Strong CP adherences to the hypothalamus were demonstrated in 63% of cases. The infundibulo-tuberal and sellar/suprasellar–3V CP topographies were associated with the highest rates of hypothalamic dysfunction before surgery (p < 0.001), surgical hypothalamic injury (p < 0.001), and severe postoperative morbidity and/or mortality (p = 0.009). Both topographies showed the strongest adherences to the hypothalamus and 3V (p < 0.001).

CONCLUSIONS

Cushing’s CP series comprises severely ill patients with tumors in the late stages of progression, with a high rate of tumors developing primarily within the hypothalamus (infundibulo-tuberal CPs) or invading this structure from the sellar/suprasellar regions. Craniopharyngioma topography was the fundamental variable influencing the clinical manifestations, tumor features, and patient outcomes in this series.

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Ruth Prieto, José María Pascual and Laura Barrios

Charles H. Frazier (1870–1936), one of the pioneers of neurosurgery in the US, is known worldwide for devising surgical procedures to relieve trigeminal neuralgia and intractable pain. Less well-known are his substantial contributions to understanding and treating pituitary and parahypophyseal lesions. Along with Bernard Alpers, he defined Rathke’s cleft tumors as a different pathological entity from adenomas and hypophyseal stalk tumors (craniopharyngiomas [CPs]). The surgical challenge posed by CPs piqued Frazier’s interest in these lesions, although he never published a complete account of his CP series. An examination of the Charles Frazier papers at the College of Physicians of Philadelphia allowed the authors to identify 54 CPs that he had treated during his career. In the early 1910s, Frazier developed the subfrontal approach, which would become the primary surgical route to access these lesions, providing better control of the adjacent vital neurovascular structures than the transsphenoidal route hitherto used. Nevertheless, strong adhesions between CPs and the third ventricle floor, the major reason underlying Frazier’s disappointing results, moved him to advocate incomplete tumor removal followed by radiotherapy to reduce both the risk of hypothalamic injury and CP recurrence. This conservative strategy remains a judicious treatment for CPs to this day.

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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.

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Ruth Prieto, José María Pascual and Laura Barrios

OBJECTIVE

Harvey Cushing (1869–1939) developed pioneering surgical techniques for craniopharyngioma (CP) removal. This study exhaustively analyzes the pathological variables and surgical strategies that influenced Cushing’s results in his entire series of CP patients.

METHODS

The CP records from Cushing’s Brain Tumor Registry were carefully reviewed, as were his CP cases published in medical monographs and scientific reports.

RESULTS

One hundred twenty-four tumors with characteristics typical of CP comprise Cushing’s entire series (CP124). Cushing performed 198 surgical procedures in the patients in whom these tumors were treated, with a 23% mortality rate within the first 2 months after surgery. Three periods in Cushing’s CP surgical career can be differentiated: an early period (1901–1917, 39 patients) characterized by his use of the transsphenoidal approach and limited cyst drainage procedures, an intermediate period (1919–1925, 42 patients) in which the subfrontal approach was the standard procedure and maximal removal was attempted, and a late period (1926–1932, 43 patients) characterized by the use of air ventriculography for topographical diagnosis and limited resections via a transventricular approach. Among Cushing’s CP series were 92 cases that were pathologically verified (CP92). In this subcohort, the unilateral subfrontal approach was predominantly used (72% of cases), followed by the transsphenoidal (15%) and frontal transcortical-transventricular (8%) approaches. Drainage of the CP cystic component or partial excision of the solid component was achieved in 61% of the cases, subtotal removal in 23%, and macroscopic total removal in 10%. Satisfactory outcomes were obtained in 55% of the patients in CP92, whereas poor outcomes and/or death related to hypothalamic injury was observed in 28%. Postoperative symptoms related to hypothalamic dysfunction occurred 53% of the time. The subfrontal approach yielded the highest rates of radical removal (p < 0.001) and good outcomes (p = 0.01). Partial removals were associated with the highest rates of poor outcomes, including death (p = 0.009). Cushing’s removal of CPs with a primary infundibulo-tuberal topography or showing third ventricle invasion was associated with the highest rates of hypothalamic injury (p < 0.001) and the worst outcomes (p = 0.009).

CONCLUSIONS

Harvey Cushing’s techniques and surgical philosophy varied substantially throughout his career. The experience he gained with this large CP series made him aware of the importance of limiting the extent of tumor removal and leaving untouched the tumor portion strongly adhered to the hypothalamus.

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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.

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Ruth Prieto, José M. Pascual and Laura Barrios

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Ruth Prieto and Jose María Pascual