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

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Abstract

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.

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Abbreviations

CP:

Craniopharyngioma

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Acknowledgments

Maria Rosdolsky, Jakob Erdheim’s great-niece and co-author of this study, feels very honored and thankful to the opportunity Dr. Pascual gave her to participate in this study. She collaborated with Mr. Eduard Winter, the director of the pathological-anatomical museum in Vienna who found specimens that Erdheim had probably used for his studies of craniopharyngioma, took pictures of the specimens, and also made copies of Erdheim’s autopsy reports. This study was supported by the Sixto Obrador Award from the Spanish Society of Neurosurgery (SENEC) granted to the best national research study of neurosurgery in 2013. The authors wish to especially 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 Lucretia MacLure and all 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. Finally, the authors wish to express their gratitude to the staff in the Narrenturm and the Jakob Erdheim Institute for their kind assistance and the original pictorial material provided for this study.

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The authors declare that they have no conflict of interest.

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Correspondence to José M. Pascual.

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Die Augen sind gut, aber sie schauen nicht an

-The eyes are good, but they do not observe

Jakob Erdheim

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Pascual, J.M., Rosdolsky, M., Prieto, R. et al. Jakob Erdheim (1874–1937): father of hypophyseal-duct tumors (craniopharyngiomas). Virchows Arch 467, 459–469 (2015). https://doi.org/10.1007/s00428-015-1798-4

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