Joseph Engel (1816-1899), author of a meaningful dissertation on tumors of the pituitary infundibulum: his report on the oldest preserved whole craniopharyngioma specimen.

Joseph Engel (1816-1899) was a Viennese anatomist and pathologist trained under the mentorship of Carl von Rokitansky (1804-1878), the man who laid the foundations of gross anatomical pathology. In 1839, Engel completed his first scientific project: the dissertation entitled "Über den Hirnanhang und den Trichter" (About the pituitary gland and the infundibulum). This work analyzed the pathological and clinical characteristics of the pituitary and infundibulum tumor specimens collected at the Vienna Pathologic-Anatomical Museum. This little-known work represents one of the earliest attempts to determine the function of the pituitary gland-infundibulum complex. Among the 12 pituitary/infundibulum tumors examined in Engel's dissertation, one of the cases (no. 10) was instrumental for the definition of hypophyseal duct tumors, or craniopharyngiomas (CPs). This huge cyst, approximately the size of a goose egg (6 × 6 × 4.5 cm), was found in 1828 during the autopsy of a 33-year-old patient who suffered from severe headache, blindness, apathy, and finally somnolence. The cyst had replaced the hypophysis and extended upwards into the hypothalamic region and downwards into the sphenoid sinus, its inferior pole protruding through the soft palate. In 1904, the Viennese pathologist Jakob Erdheim (1874-1937) re-examined this lesion and conclusively categorized it as a hypophyseal duct tumor after a detailed histological study. The original tumor specimen corresponding to this CP case is still preserved at the Narrenturm, the circular building within the old Allgemeines Krankenhaus (Vienna General Hospital) that today holds the pathological collections of Vienna's Federal Pathologic-Anatomical Museum. To the best of our knowledge, this tumor is very probably the oldest preserved whole CP specimen in the world. This paper presents a comprehensive review of Engel's dissertation, the pioneering pathological work on pituitary and infundibulum tumors which laid the groundwork for the proper clinical, topographic, and pathological categorization of craniopharyngiomas.

Craniopharyngiomas Primarily Involving the Hypothalamus: A Model of Neurosurgical Lesions to Elucidate the Neurobiological Basis of Psychiatric Disorders.

OBJECTIVE: This study provides a systematic review and meta-analysis of psychiatric disorders caused by craniopharyngiomas and the hypothalamic alterations underlying these symptoms. METHODS: We investigated a collection of 210 craniopharyngiomas reported from 1823 to 2017 providing detailed clinical and pathologic information about psychiatric disturbances, including 10 of our own series, and compared the hypothalamic damage in this cohort with the present in a control cohort of 105 cases without psychiatric symptoms. RESULTS: Psychiatric disorders occurred predominantly in patients with craniopharyngiomas developing primarily at the infundibulotuberal region (45%) or entirely within the third ventricle (30%), mostly affecting adult patients (61%; P < 0.001). Most tumors without psychic symptoms developed beneath the third ventricle floor (53.5%; P < 0.001), in young patients (57%; P < 0.001). Psychiatric disturbances were classified in 6 major categories: 1) Korsakoff-like memory deficits, 66%; 2) behavior/personality changes, 48.5%; 3) impaired emotional expression/control, 42%; 4) cognitive impairments, 40%; 5) mood alterations, 32%; and 6) psychotic symptoms, 22%. None of these categories was associated with hydrocephalus. Severe memory deficits occurred with damage of the mammillary bodies (P < 0.001). Mood disorders occurred with compression/invasion of the third ventricle floor and/or walls (P < 0.012). Coexistence of other hypothalamic symptoms such as temperature/metabolic dysregulation or sleepiness favored the emergence of psychotic disorders (P < 0.008). Postoperative psychiatric outcome was better in strictly intraventricular craniopharyngiomas than in other topographies (P < 0.001). A multivariate model including the hypothalamic structures involved, age, hydrocephalus, and hypothalamic symptoms predicts the appearance of psychiatric disorders in 81% of patients. CONCLUSIONS: Craniopharyngiomas primarily involving the hypothalamus represent a neurobiological model of psychiatric and behavioral disorders.

Cystic tumors of the pituitary infundibulum: seminal autopsy specimens (1899 to 1904) that allowed clinical-pathological craniopharyngioma characterization.

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.

Giovanni Verga (1879-1923), author of a pioneering treatise on pituitary surgery: the foundations of this new field in Europe in the early 1900s.

The field of pituitary surgery was born in the first decade of the twentieth century in Europe, and it evolved rapidly with the development of numerous innovative surgical techniques by some of the founding fathers of neurosurgery. This study investigates the pioneering Italian treatise on pituitary surgery, La Patologia Chirurgica dell'Ipofisi (Surgical Pathology of the Hypophysis), published in 1911 by Giovanni Verga (1879-1923), a surgeon from Pavía and one of Golgi's disciples. This little-known monograph compiles the earliest experience on pituitary surgery through the analysis of the first 50 procedures performed between 1903 and 1911. We conducted a biographical survey of Giovanni Verga and the motivations for his work on pituitary surgery. In addition, a systematic analysis of all original reports and historical documents about these pituitary procedures referenced in Verga's treatise was carried out. Verga's treatise provides a summary of the techniques employed and surgical outcomes for the first 50 attempted procedures of pituitary tumor removal. This monograph is the only scientific source that includes a complete account of the series of 10 pituitary tumors operated on by Sir Victor Horsley in the 1900s. Three major types of surgery were employed: (i) palliative procedures of craniectomy (n = 6); (ii) transcranial approaches to the pituitary gland, either subfrontal or subtemporal (n = 13); and (iii) transphenoidal routes to expose the sella turcica, either using an upper transnasal-transethmoidal approach (n = 19) or a lower sublabial/endonasal-transeptal one (n = 12). An operative mortality rate of 36% (n = 17) was observed in these early series. The pathological nature of the tumors operated on was available in 42 cases. There were 28 adenomas and 15 craniopharyngiomas. Sir Victor Horsley (1857-1916) and the Viennese surgeons Anton von Eiselsberg (1860-1939) and Oskar Hirsch (1877-1965) were the leading European figures in the development of pituitary surgery. Giovanni Verga's treatise La Patologia Chirurgica dell'Ipofisi is a fundamental, pioneering book in the history of pituitary surgery, a work that compiles the foundations of this field in Europe and the only authoritative source providing a complete record of pituitary procedures performed by Sir Victor Horsley.

Craniopharyngioma adherence: a comprehensive topographical categorization and outcome-related risk stratification model based on the methodical examination of 500 tumors.

OBJECTIVE Craniopharyngioma (CP) adherence strongly influences the potential for achieving a radical and safe surgical treatment. However, this factor remains poorly addressed in the scientific literature. This study provides a rational, comprehensive description of CP adherence that can be used for the prediction of surgical risks associated with the removal of these challenging lesions. METHODS This study retrospectively analyzes the evidence provided in pathological, neuroradiological, and surgical CP reports concerning 3 components of the CP attachment: 1) the intracranial structures attached to the tumor; 2) the morphology of the adhesion; and 3) the adhesion strength. From a total of 1781 CP reports published between 1857 and 2016, a collection of 500 CPs providing the best information about the type of CP attachment were investigated. This cohort includes autopsy studies (n = 254); surgical studies with a detailed description or pictorial evidence of CP adherence (n = 298); and surgical CP videos (n = 61) showing the technical steps for releasing the attachment. A predictive model of CP adherence in hierarchical severity levels correlated with surgical outcomes was generated by multivariate analysis. RESULTS The anatomical location of the CP attachment occurred predominantly at the third ventricle floor (TVF) (54%, n = 268), third ventricle walls (23%, n = 114), and pituitary stalk (19%, n = 94). The optic chiasm was involved in 56% (n = 281). Six morphological patterns of CP attachment were identified: 1) fibrovascular pedicle (5.4%); 2) sessile or patch-like (21%); 3) cap-like (over the CP top, 14%); 4) bowl-like (around the CP bottom, 13.5%); 5) ring-like (encircling central band, 19%); and 6) circumferential (enveloping the entire CP, 27%). Adhesion strength was classified in 4 grades: 1) loose (easily dissectible, 8%); 2) tight (requires sharp dissection, 32%); 3) fusion (no clear cleavage plane, 40%); and 4) replacement (loss of brain tissue integrity, 20%). The types of CP attachment associated with the worst surgical outcomes are the ring-like, bowl-like, and circumferential ones with fusion to the TVF or replacement of this structure (p < 0.001). The CP topography is the variable that best predicts the type of CP attachment (p < 0.001). Ring-like and circumferential attachments were observed for CPs invading the TVF (secondary intraventricular CPs) and CPs developing within the TVF itself (infundibulo-tuberal CPs). Brain invasion and peritumoral gliosis occurred predominantly in the ring-like and circumferential adherence patterns (p < 0.001). A multivariate model including the variables CP topography, tumor consistency, and the presence of hydrocephalus, infundibulo-tuberal syndrome, and/or hypothalamic dysfunction accurately predicts the severity of CP attachment in 87% of cases. CONCLUSIONS A comprehensive descriptive model of CP adherence in 5 hierarchical levels of increased severity-mild, moderate, serious, severe, and critical-was generated. This model, based on the location, morphology, and strength of the attachment can be used to anticipate the surgical risk of hypothalamic injury and to plan the degree of removal accordingly.

Jakob Erdheim (1874-1937): father of hypophyseal-duct tumors (craniopharyngiomas).

Jakob Erdheim (1874-1937) was a Viennese pathologist who identified and defined a category of pituitary tumors known as craniopharyngiomas. He named these lesions "hypophyseal duct tumors" (Hypophysenganggeschwülste), a term denoting their presumed origin from cell remnants of the hypophyseal duct, the embryological structure through which Rathke's pouch migrates to form part of the pituitary gland. He described the two histological varieties of these lesions as the adamantinomatous and the squamous-papillary types. He also classified the different topographies of craniopharyngiomas along the hypothalamus-pituitary axis. Finally, he provided the first substantial evidence for the functional role of the hypothalamus in the regulation of metabolism and sexual functions. Erdheim's monograph on hypophyseal duct tumors elicited interest in the clinical effects and diagnosis of pituitary tumors. It certainly contributed to the development of pituitary surgery and neuroendocrinology. Erdheim's work was greatly influenced by the philosophy and methods of research introduced to the Medical School of Vienna by the prominent pathologist Carl Rokitansky. Routine practice of autopsies in all patients dying at the Vienna Municipal Hospital (Allgemeines Krankenhaus), as well as the preservation of rare pathological specimens in a huge collection stored at the Pathological-Anatomical Museum, represented decisive policies for Erdheim's definition of a new category of epithelial hypophyseal growths. Because of the generalized use of the term craniopharyngioma, which replaced Erdheim's original denomination, his seminal work on hypophyseal duct tumors is only referenced in passing in most articles and monographs on this tumor. This article is intended to pay tribute to Erdheim's fundamental breakthroughs, his discovery of craniopharyngiomas and their functional damage to the hypothalamus. On these fundamental achievements, Jakob Erdheim should be recognized as the true father of craniopharyngiomas.

Premio Sixto Obrador 2013. Modelo topográfico de 3 ejes para el tratamiento quirúrgico de los craneofaringiomas. Parte II: Evidencias anatómicas y neurorradiológicas que definen el modelo de clasificación de 3 ejes y su utilidad para predecir el riesgo quirúrgico individual / The 2013 Sixto Obrador Award. A triple-axis topographical model for surgical planning of craniopharyngiomas. Part II: Anatomical and neuroradiological evidence to define triple-axis topography and its usefulness in predicting individual surgical risk

INTRODUCCIÓN Y OBJETIVOS: Este estudio analiza las evidencias patológicas y de imagen de resonancia magnética que definen la topografía de los craneofaringiomas y permiten una clasificación de las lesiones según el riesgo de daño hipotalámico que estas asocian. MATERIAL Y MÉTODOS: Se ha realizado un análisis sistemático de los métodos de clasificación topográfica empleados en las series quirúrgicas de craneofaringiomas descritas en la literatura (n = 145 series, 4.588 craneofaringiomas). También se analizaron las relaciones topográficas de casos individuales intervenidos y bien descritos de la literatura (n = 224 casos) y de casos estudiados en autopsias (n = 201 casos). Finalmente, se analizaron y compararon los estudios prequirúrgicos y posquirúrgicos de imagen de resonancia magnética de craneofaringiomas bien descritos (n = 130) para establecer un modelo diagnóstico topográfico en 3 ejes de la lesión, que permite anticipar cualitativamente el riesgo quirúrgico asociado de daño hipotalámico. RESULTADOS: Las 2 principales variables pronósticas que definen la topografía del craneofaringioma son su posición con respecto al diafragma selar y la afectación del suelo del tercer ventrículo. Un modelo diagnóstico de 5 variables, que son: edad del paciente, existencia de hidrocefalia o de alteraciones del comportamiento, posición relativa de los hipotálamos y el valor del ángulo mamilar, permiten diferenciar craneofaringiomas supraselares que comprimen el tercer ventrículo (craneofaringiomas seudointraventriculares) de lesiones estrictamente intraventriculares o aquellas con un crecimiento primario en el suelo del tercer ventrículo (craneofaringiomas infundibulotuberales o no estrictamente intraventriculares). CONCLUSIONES: Un modelo de clasificación topográfica de los craneofaringiomas en 3 ejes que incluya el grado de infiltración del hipotálamo es útil para la planificación del abordaje y el grado de resección. Los craneofaringiomas infundibulotuberales representan un 42% de los casos y muestran una adherencia fuerte y circunferencial al suelo del tercer ventrículo, asociando un riesgo de daño hipotalámico del 50%. El abordaje transesfenoidal endoscópico permite valorar la adherencia tumoral hipotalámica bajo visión directa

INTRODUCTION AND OBJECTIVES: This study evaluates the pathological and magnetic resonance imaging evidence to define the precise topographical relationships of craniopharyngiomas and to classify these lesions according to the risks of hypothalamic injury associated with their removal. MATERIAL AND METHODS: An extensive, systematic analysis of the topographical classification models used in the surgical series of craniopharyngiomas reported in the literature(n = 145 series, 4,588 craniopharyngiomas) was performed. Topographical relationships of well-described operated craniopharyngiomas (n = 224 cases) and of non-operated cases reported in autopsies (n = 201 cases) were also analysed. Finally, preoperative and postoperative magnetic resonance imaging studies displayed in craniopharyngiomas reports (n = 130) were compared to develop a triple-axis model for the topographical classification of the selesions with qualitative information regarding the associated risk of hypothalamic injury. RESULTS: The 2 major variables with prognostic value to define the topography of a craniopharyngioma are its position relative to the sellar diaphragm and its degree of invasion of the third ventricle floor. A multivariate diagnostic model including 5 variables -patient age, presence of hydrocephalus and/or psychiatric symptoms, the relative position of the hypothalamus and the mammillary body angle- makes it possible to differentiate suprasellar craniopharyngiomas displacing the third ventricle upwards (pseudointraventricular craniopharyngiomas) from either strictly intraventricular craniopharyngiomas or lesions developing primarily within the third ventricle floor (infundibulo-tuberal or not strictly intraventricular craniopharyngiomas). CONCLUSIONS: A triple-axis topographical model for craniopharyngiomas that includes the degree of hypothalamus invasion is useful in planning the surgical approach and degree of resection. Infundibulo-tuberal craniopharyngiomas represent 42% of all cases. These lesions typically show tight, circumferential adhesion to the third ventricle floor, with their removal being associated with a 50% risk of hypothalamic injury. The endoscopically-assisted extended transsphenoidal approach provides a proper view to assess the degree and extension of craniopharyngioma adherence to the hypothalamus

Premio Sixto Obrador 2013. Modelo topográfico de 3 ejes para el tratamiento quirúrgico de los craneofaringiomas. Parte I: Evolución histórica del diagnóstico topográfico y métodos de clasificación de los craneofaringiomas / The 2013 Sixto Obrador Award. A triple-axis topographical model for surgical planning of craniopharyngiomas. Part I: Historical review of the topographical diagnosis and classification schemes of craniopharyngiomas

INTRODUCCIÓN Y OBJETIVOS Este estudio revisa la evolución histórica de los hallazgos patológicos, neurorradiológicos y quirúrgicos que han influido en el desarrollo de los conceptos sobre la topografía de los craneofaringiomas y en los diversos métodos de clasificación topográfica de estas lesiones. MATERIAL Y MÉTODOS Se ha realizado un análisis sistemático de los métodos de clasificación topográfica empleados en las series quirúrgicas de craneofaringiomas descritas en la literatura (n = 145 series, 4.588 craneofaringiomas), con el objetivo de describir los hallazgos anatómicos fundamentales que han guiado el desarrollo de las clasificaciones topográficas empleadas a lo largo de la historia. Estos hallazgos se han comparado con las relaciones topográficas de casos individuales bien descritos de craneofaringiomas intervenidos (n = 224 casos), así como de casos no operados estudiados en autopsias (n = 201 casos). RESULTADOS: Las 2 principales variables que definen la topografía de un craneofaringioma son su posición con respecto al diafragma selar y su grado de invasión del suelo del tercer ventrículo. Los tumores supraselares que desplazan hacia arriba el suelo del tercer ventrículo (craneofaringiomas seudointraventriculares) pueden extirparse totalmente de forma segura y deben distinguirse de los tumores que han crecido de forma primaria en el suelo del tercer ventrículo. CONCLUSIONES: Un modelo de clasificación topográfica de los craneofaringiomas en 3 ejes que incluya el grado de infiltración del hipotálamo es útil para la planificación del abordaje y el grado de resección. Los craneofaringiomas infundibulotuberales asocian el mayor riesgo de daño hipotalámico, en torno al 50%. El abordaje transesfenoidal endoscópico permite valorar la topografía de la lesión y su grado de adherencia tumoral hipotalámica bajo visión directa

INTRODUCTION AND OBJECTIVES: This study reviews the historical evolution of pathological, neuroradiological and surgical evidence that influenced the topographical concepts andclassification schemes of craniopharyngiomas. MATERIAL AND METHODS: An extensive, systematic analysis of the surgical series of craniopharyngiomas reported in the literature was performed (n= 145 series, 4,588 tumours) todescribe the fundamental anatomical findings guiding the topographical classification schemes used for this tumour throughout history. These findings were compared with topographical relationships reported for well-described operated craniopharyngiomas (n = 224 cases) as well as for non-operated cases studied in autopsies (n = 201 cases). RESULTS: Two major variables define the topography of a craniopharyngioma: its position relative to the sellar diaphragm and its degree of invasion of the third ventricle floor. Suprasellarlesions displacing the third ventricle floor upwards (pseudointraventricular craniopharyngiomas) are amenable to safe, radical resection and must be differentiated from lesions developing primarily within the third ventricle floor (infundibulo-tuberal or not strictly intraventricular craniopharyngiomas). The latter group typically shows tight, circumferential adhesion to the third ventricle floor and represents approximately 40% of all cases. CONCLUSIONS: A triple-axis topographical model for craniopharyngiomas that includes the degree of hypothalamus invasion is useful in planning surgical approach and degree of resection. The group of infundibulo-tuberal craniopharyngiomas associates the highest risk of hypothalamic injury (50%). The endoscopically-assisted extended transsphenoidal approach provides a proper view to assess the topography of the craniopharyngioma and its degree of adherence to the hypothalamus

[The 2013 Sixto Obrador Award. A triple-axis topographical model for surgical planning of craniopharyngiomas. Part I: historical review of the topographical diagnosis and classification schemes of craniopharyngiomas]. / Premio Sixto Obrador 2013. Modelo topográfico de 3 ejes para el tratamiento quirúrgico de los craneofaringiomas. Parte I: Evolución histórica del diagnóstico topográfico y métodos de clasificación de los craneofaringiomas.

INTRODUCTION AND OBJECTIVES: This study reviews the historical evolution of pathological, neuroradiological and surgical evidence that influenced the topographical concepts and classification schemes of craniopharyngiomas. MATERIAL AND METHODS: An extensive, systematic analysis of the surgical series of craniopharyngiomas reported in the literature was performed (n=145 series, 4,588 tumours) to describe the fundamental anatomical findings guiding the topographical classification schemes used for this tumour throughout history. These findings were compared with topographical relationships reported for well-described operated craniopharyngiomas (n=224 cases) as well as for non-operated cases studied in autopsies (n=201 cases). RESULTS: Two major variables define the topography of a craniopharyngioma: its position relative to the sellar diaphragm and its degree of invasion of the third ventricle floor. Suprasellar lesions displacing the third ventricle floor upwards (pseudointraventricular craniopharyngiomas) are amenable to safe, radical resection and must be differentiated from lesions developing primarily within the third ventricle floor (infundibulo-tuberal or not strictly intraventricular craniopharyngiomas). The latter group typically shows tight, circumferential adhesion to the third ventricle floor and represents approximately 40% of all cases. CONCLUSIONS: A triple-axis topographical model for craniopharyngiomas that includes the degree of hypothalamus invasion is useful in planning surgical approach and degree of resection. The group of infundibulo-tuberal craniopharyngiomas associates the highest risk of hypothalamic injury (50%). The endoscopically-assisted extended transsphenoidal approach provides a proper view to assess the topography of the craniopharyngioma and its degree of adherence to the hypothalamus.

[The 2013 Sixto Obrador Award. A triple-axis topographical model for surgical planning of craniopharyngiomas. Part II: Anatomical and neuroradiological evidence to define triple-axis topography and its usefulness in predicting individual surgical risk]. / Premio Sixto Obrador 2013. Modelo topográfico de 3 ejes para el tratamiento quirúrgico de los craneofaringiomas. Parte II: Evidencias anatómicas y neurorradiológicas que definen el modelo de clasificación de 3 ejes y su utilidad para predecir el riesgo quirúrgico individual.

INTRODUCTION AND OBJECTIVES: This study evaluates the pathological and magnetic resonance imaging evidence to define the precise topographical relationships of craniopharyngiomas and to classify these lesions according to the risks of hypothalamic injury associated with their removal. MATERIAL AND METHODS: An extensive, systematic analysis of the topographical classification models used in the surgical series of craniopharyngiomas reported in the literature (n=145 series, 4,588 craniopharyngiomas) was performed. Topographical relationships of well-described operated craniopharyngiomas (n=224 cases) and of non-operated cases reported in autopsies (n=201 cases) were also analysed. Finally, preoperative and postoperative magnetic resonance imaging studies displayed in craniopharyngiomas reports (n=130) were compared to develop a triple-axis model for the topographical classification of these lesions with qualitative information regarding the associated risk of hypothalamic injury. RESULTS: The 2 major variables with prognostic value to define the topography of a craniopharyngioma are its position relative to the sellar diaphragm and its degree of invasion of the third ventricle floor. A multivariate diagnostic model including 5 variables -patient age, presence of hydrocephalus and/or psychiatric symptoms, the relative position of the hypothalamus and the mammillary body angle- makes it possible to differentiate suprasellar craniopharyngiomas displacing the third ventricle upwards (pseudointraventricular craniopharyngiomas) from either strictly intraventricular craniopharyngiomas or lesions developing primarily within the third ventricle floor (infundibulo-tuberal or not strictly intraventricular craniopharyngiomas). CONCLUSIONS: A triple-axis topographical model for craniopharyngiomas that includes the degree of hypothalamus invasion is useful in planning the surgical approach and degree of resection. Infundibulo-tuberal craniopharyngiomas represent 42% of all cases. These lesions typically show tight, circumferential adhesion to the third ventricle floor, with their removal being associated with a 50% risk of hypothalamic injury. The endoscopically-assisted extended transsphenoidal approach provides a proper view to assess the degree and extension of craniopharyngioma adherence to the hypothalamus.

Development of intracranial approaches for craniopharyngiomas: an analysis of the first 160 historical procedures.

OBJECT: The development of surgical procedures for the removal of craniopharyngiomas (CPs) was greatly influenced by the enormous topographical and morphological heterogeneity displayed by these lesions. In this study the authors reviewed the intracranial approaches designed to treat CPs during the early historical period (1891-1938) with the aim of finding the CP topographical and pathological features that influence patient outcomes. METHODS: The authors conducted a systematic retrospective review of well-described cases of surgically treated CPs in publications from the period 1891-1938. Valuable information regarding the diagnosis of the lesion, type of craniotomy performed, CP topography, and outcome was selected from 418 reports included in medical publications from this period. The type of surgical procedure used, degree of tumor removal, CP position and histological variety, and clinical evidence of postoperative hypothalamic injury were the variables analyzed with the aim of defining their influence on the final patient outcome. RESULTS: A collection of 160 cases was eligible for analysis. Craniopharyngioma topography was significantly related to the existence of postoperative hypothalamic damage and the degree of tumor removal achieved (p < 0.001). The infundibulo-tuberal, or not strictly intraventricular, topography was associated with the highest rate of hypothalamic injury (84%) and impossibility of tumor removal (51%). This topography also showed the worst prognosis (p = 0.001). Additional variables correlated with patient outcome were the presence of hypothalamic damage, type of surgical approach used, and degree of tumor removal. Patients having a poor outcome, suffering from permanent coma, or dying after surgery presented with symptoms of hypothalamic injury in 40% of cases (p < 0.001). The surgical approach associated with the best outcome was the transsphenoidal (58%), followed by the subfrontal (45%) and the transcallosal (45%). Subtotal resection of the lesion yielded the best postoperative results, with only 17% of patients dying or suffering from a poor outcome, in contrast to the 39% reported for gross-total removal of the lesion (p = 0.001). CONCLUSIONS: Two major variables influenced the results of early surgical experience with CPs for the period from 1891 to 1938: 1) the inaccuracy in defining CP topography with the diagnostic methods available at that time; and 2) the ignorance about the risks associated with the dissection of lesions showing tenacious adherence to the hypothalamus. The degree of functional and morphological disturbance of the hypothalamus caused by a CP remains a fundamental variable helping the surgeon to predict the risks associated with the radical excision of the tumor and patient outcome.