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

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.

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

“…These results confirm numerous autopsy and surgical observations that have presented evidence that approximately 40% of CPs grow within the infundibulum and the adjacent TVF and show a strong adherence to these structures. [28][29][30][31][32][33][34][35][36][37][38] A marked correlation between the morphology of the CP attachment and the degree of adhesion strength was identified in our analysis. These components depended on the particular location of the tumor and the anatomical structures involved (see Figs.…”

Craniopharyngioma recurrence: the impact of tumor topography

“…According to Erdheim's embryogenetic theory, CPs originate from epithelial cell nests, remnants of the craniopharyngeal duct, which are deposited preferentially at the upper and lower ends of the pars tuberalis. 21 This theory could explain why CP topographies are concentrated around 2 categories, the suprasellar-pseudointraventricular and the infundibulotuberal (Fig. 2).…”

“…The embryonic primordium of the pars tuberalis is initially located at the lower part of Rathke's pouch, in close contact with the primitive stomodeum, although it eventually undergoes a forward and upward rotation toward the rudimentary median eminence, at the base of the diencephalon. 21,22 The morphology and extension of the pars tuberalis in adults varies from a narrow tongue covering only the anterior aspect of the pituitary stalk and median eminence to a pillowcase-like wrapping of the stalk and covering the entire ventral aspect of the median eminence and tuber cinereum. According to Erdheim's embryogenetic theory, CPs originate from epithelial cell nests, remnants of the craniopharyngeal duct, which are deposited preferentially at the upper and lower ends of the pars tuberalis.…”

“…2). 21,22 Depending on the time the migration of epithelial remnants occurs in relation to the formation of the pia mater and arachnoid layers that cover the TVF, CPs may develop at different depths across the thickness of the infundibular wall, from extracerebral intraarachnoid lesions expanding in the suprasellar area to subpial, truly intracerebral lesions developing within the neural tissue of the infundibulum and/or tuber cinereum. 6 The high variability regarding the strength and extent of CP adherence to neighboring structures observed in the first surgical procedures must be related to the original topography of the lesion, and it will unquestionably influence the rate of ulterior recurrence.…”

Craniopharyngiomas: An Appropriate Surgical Treatment based on Topographical and Pathological Concepts

“…In addition, Erdheim had shown a particular interest in the pathological alterations involving the pituitary gland since the beginning of his career and was able to study in detail the collection of old brain specimens with pituitary tumors gathered at the Pathological Anatomical Museum in Vienna, nowadays stored at the Narrenturm (the former hospital for insane patients in Vienna) [16,17]. The methodical study of a set of cystic lesions developed at the infundibulum in these brain specimens eventually led him to define the new pathological category of "hypophysengangsgeschwülste" (hypophyseal duct tumor), a term under which he grouped the heterogeneous epithelial growths developing either from the pituitary stalk or from the infundibulum (presumably from the pars tuberalis, the thin layer of glandular adenohypophyseal tissue wrapping around the pituitary stalk and infundibulum) [14,16]. Erdheim showed great originality in relating the presence of squamous epithelial cell nests within the pars tuberalis, with the embryological development of CPs [14].…”