, Volume 21, Issue 4, pp 393–405 | Cite as

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

  • José M. Pascual
  • Ruth Prieto
  • Maria Rosdolsky
  • Sewan Strauss
  • Inés Castro-Dufourny
  • Verena Hofecker
  • Eduard Winter
  • Rodrigo Carrasco
  • Walter Ulrich


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.


Craniopharyngioma Fröhlich’s syndrome Hypothalamus Infundibulum Jakob Erdheim Pituitary gland Rathke’s pouch Sir Frederick Mott 



The authors wish to thank Crystal Smith, Reference Librarian of the Department of History of Medicine at the National Library of Medicine, National Institutes of Health, Bethesda, Maryland, for her kind assistance during the process of searching and retrieving articles and monographs used in this study. The authors are also indebted to the staff at the Francis Countway Medical Library at Harvard Medical School, Boston, Massachusetts, for their invaluable help in obtaining some of the original research material used for this study. We would specially like to express our acknowledgement to the staff in the Narrenturm and the Jakob Erdheim Institute for their kind assistance and the original pictorial material provided for this study. Finally, the authors wish to express their gratitude to George Hamilton for his critical review of the language and style of the manuscript.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • José M. Pascual
    • 1
  • Ruth Prieto
    • 2
  • Maria Rosdolsky
    • 3
  • Sewan Strauss
    • 4
  • Inés Castro-Dufourny
    • 5
  • Verena Hofecker
    • 6
  • Eduard Winter
    • 6
  • Rodrigo Carrasco
    • 7
  • Walter Ulrich
    • 8
  1. 1.Department of NeurosurgeryLa Princesa University HospitalMadridSpain
  2. 2.Department of NeurosurgeryPuerta de Hierro University HospitalMadridSpain
  3. 3.Independent Medical TranslatorJenkintownUSA
  4. 4.Department of PediatricsHospital Dahme-SpreewaldKönigs WusterhausenGermany
  5. 5.Department of EndocrinologySureste University HospitalMadridSpain
  6. 6.Pathologisch-anatomische Sammlung im Narrenturm-NHMViennaAustria
  7. 7.Department of NeurosurgeryRamón y Cajal University HospitalMadridSpain
  8. 8.Department of Pathology and Clinical BacteriologyJakob Erdheim InstituteViennaAustria

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