Abstract
Purpose
Infundibulo-tuberal syndrome groups endocrine, metabolic and behavioral disturbances caused by lesions involving the upper neurohypophysis (median eminence) and adjacent basal hypothalamus (tuber cinereum). It was originally described by Henri Claude and Jean Lhermitte in 1917, in a patient with a craniopharyngioma. This study investigates the clinical, pathological and surgical evidence verifying the infundibulo-tuberal syndrome caused by craniopharyngiomas (CPs).
Methods
A systematic retrospective review of craniopharyngiomas reported in French literature between 1705 and 1973 was conducted. A total of 128 well described reports providing a comprehensive clinical and pathological description of the tumors were selected. This series represents the historical French cohort of CPs reported in the pre-CT/MRI era.
Results
Three major syndromes caused by CPs were categorized: pituitary syndrome (35 %), infundibulo-tuberal syndrome (52 %) and hypothalamic syndrome (49 %). CP topography was significantly related to the type of syndrome described (p < 0.001). Infundibulo-tuberal syndrome occurred in CPs which replaced or invaded the third ventricle floor. In contrast, the majority of sellar/suprasellar CPs growing below the third ventricle showed a pituitary syndrome (82 %). Cases with hypothalamic syndrome were characterized by anatomical integrity of the pituitary gland and stalk (p = 0.033) and occurred predominantly in adults older than 41 years old (p < 0.005). Among infundibulo-tuberal symptoms, abnormal somnolence was not related with the presence of hydrocephalus. All squamous-papillary CPs presented psychiatric disturbances (p < 0.001).
Conclusion
This historical CP cohort evidences a clinical-topographical correlation between the patient’s type of syndrome and the anatomical structures involved by the tumor along the hypophysial-hypothalamic axis.
Similar content being viewed by others
Abbreviations
- CPs:
-
Craniopharyngiomas
- CT:
-
Computed tomography
- DI:
-
Diabetes insipidus
- IT:
-
Infundibulo-tuberal
- MRI:
-
Magnetic resonance imaging
- TVF:
-
Third ventricle floor
- 3V:
-
Third ventricle
References
Anderson E, Haymaker W (1974) Breakthroughs in hypothalamic and pituitary research. In: Swaab DF, Schade JP (eds): Integrative hypothalamic activity. Prog. Brain. Res. Elselvier, Amsterdam, vol 41, pp 1–70
Fröhlich A (1901) Ein fall von tumor der hypophysis cerebri ohne akromegalie. Wien Klin Rundschau 15(883–886):906–908
Erdheim J (1904) Über Hypophysengangsgeschwulste und Hirmcholesteatome. Sitzungsb Kais Akad Wissen Math Naturw Klin 113:537–726
Cushing H (1932) Intracranial. Tumors Notes upon a series of two thousand verified cases with surgical mortality percentages pertaining thereto. Charles C. Thomas, Springfield, pp 93–98
Claude H, Lhermitte J (1917) Le syndrome infundibulaire dans un cas de tumeur du troisième ventricule. Presse Med 41:417–418
Ben-Shlomo A, Melmed S (2011) Hypothalamic regulation of anterior pituitary function. In: Melmed S (ed) The pituitary, 3rd edn. Academic Press, Elsevier, Amsterdan, pp 21–45
Le Gros Clark WE, Beattie J, Riddoch G, Dott NM (1938) The hypothalamus. Morphological, functional, clinical and surgical aspects. Oliver and Boyd, Edinburgh
Rodríguez EM, Blázquez JL, Guerra M (2010) The design of barriers in the hypothalamus allows the median eminence and the arcuate nucleus to enjoy private milieus: the former opens to the portal blood and the latter to the cerebrospinal fluid. Peptides 31:757–776
Pascual JM, González-Llanos F, Barrios L, Roda JM (2004) Intraventricular craniopharyngiomas: topographical classification and surgical selection based on an extensive overview. Acta Neurochir (Wien) 146:785–802
Pascual JM, Prieto R, Carrasco R (2011) Infundibulo-tuberal or not strictly intraventricular craniopharyngioma: evidence for a major topographical category. Acta Neurochir (Wien) 153:2403–2425
Vieussens R (1705) Novum Vasorum Corporis Humani Systema. Amstelodami, Apud Paulum Marret
Vicq d´Azyr F (1808) Encyclopédie méthodique ou par ordre de matières: Médecine, vol 8. Agasse, Paris, p 178
Rayer P (1823) Observations sur les maladies de l´appendice sus-sphénoidal (glande pituitaire) du cerveau. Arch Gen Med 2:350–367
Rullier R (1823) Assemblée générale du 7 octobre. Arch Gen Med 3:302
Mackenzie W (1844) Amaurose par hypertrophie de la glande pituitaire. In: Mackenzie, W: Traité pratique que des maladies des yeux, B. Dusillon, pp 696–697
Michel M (1860) Pathology of the pituitary body. Charlest Med J Rev 15:145–175
Andral G (1832) Disease of the brain. In: Andral G (ed) A treatise on pathological anatomy, vol 2. Samuel Wood and Sons, New York, pp 445–507
Thirion E (1835) Observation. Bull Acad R Med Belg 5:297–299
Lebert H (1851) Cancer et affections confondues avec le cancer dans les centres nerveux. In: Lebert H (ed) Traité pratique des maladies cancéreuses et des affections curables confondues avec le cancer. J.B. Baillière, Paris, pp 738–839
Boyce R, Beadles CF (1893) A further contribution to the study of the pathology of the hypophysis cerebri. J Pathol Bacteriol 1:359–383
Tardieu A (1866) Tumeur de la base du cerveau. Bull Soc Anat Paris 9:195–197
Galezowski X (1860) Tumeurs de la base du crâne donnant lieu à une amaurose. In: X Galezowsky (ed) Étude ophtalmoscopique sur les altérations du nerf optique et sur les maladies cérébrales dont elles dépendent. L. Leclerc, Paris, pp 138–154
Soca F (1900) Sur un cas de sommeil prolongé pendant sept mois par tumeur de l´hypophyse. Nouv Iconogr Salpêtrière 2:101–115
Babinski J (1900) Tumeur du corps pituitaire sans acromégalie et avec arrêt de développement des organes génitaux. Rev Neurol 8:531–533
Cestan R, Halberstadt (1903) Epithélioma kystique de l´hypophyse sans hypertrophie du squelette. Rev Neurol 9:1180–1183
Caussade G, Laubry C (1909) Sarcome de la glande pituitaire sans acromégalie. Arch Med Exp Anat Pathol 21:173–189
Boudet G, Clunet J (1910) Contribution à l´étude des tumeurs épithéliales primitives de l´encéphale. Arch Méd Exp Anat Pathol 12:379–411
Lereboullet P, Mouzon J, Cathala J (1921) Infantilisme dit hypophysaire par tumeur du troisième ventricule. Intégrité de l´hypophyse. Rev Neurol 2:154–159
Claude H, Schoeffer H (1921) Tumeur du troisième ventricule avec compression de l´hypophyse et sans syndrome infudibulaire. Rev Neurol 1:25–32
Thomas A, Jumentié J, Chausseblanche L (1923) Léthargie intermittente traduisant l´éxistence d´une tumeur du troisième ventricule. Observation anatomoclinique. Rev Neurol 2:67–73
Sainton P, Péron N (1923) Épithélioma du lobe antérieur de l´hypophyse à symptomatologie cérébrale sans reactions hypophysaires. Rev Neurol 30:277–283
Jumentié J, Chausseblanche L (1924) Formes cliniques des tumeurs du troisième ventricule. Presse Med 21:225–228
Orzechowski C, Mitkus W (1925) De la forme parkinsonienne des tumeurs de la región infundíbulo-hypophysaire. Rev Neurol 32:1–17
Cornil L, Baudot R, Mathieu L (1925) Tumeur du tractus pharyngo-hypophysaire. Rev Med Est 53:141–147
Van Bogaert L, Nyssen R (1924) Tumeur de la region hypophysaire avec syndrome parkinsonien. Encéphale 19:547
Van Bogaert L, Nyssen R, Ley RA (1927) Tumeur du troisième ventricule avec syndrome parkinsonien et thalamique; troubles de la glycorégulation; étude anatomique. J Neurol Psychiatr 10:779–787
Bourguet J (1927) Quatre cas d´infantilisme hypophysaire. Bull Mem Soc Chir Paris 19:818–833
Vincent C, David M (1929) Trois cas de tumeurs de la poche de cranio-pharyngée (Poche de Rathke). Rev Neurol 36:208–209
Vincent C, Puech P, David M (1931) Sur deux cas de tumeur de la región hypophysaire. Traitement chirurgical. Guérison. Rev Neurol 38:288–306
Rouquier A, Hoerner G (1931) Tumeur kystique du 3º ventricule développée aux dépens des vestiges embrionnaires de la poche de Rathke. Réaction méningée asseptique primitive transitoire. Étude anatomoclinique. Rev Neurol 38:649–657
Obregia A, Dimolesco A, Constantinesco S (1932) Syndrome infundibulo-tubérien avec troubles mentaux complexes par tumeur suprasellaire du troisième ventricule. Encéphale 27:93–106
Puech P, Bissery Brun (1934) Contribution à l´étude des craniopharyngiomes : perturbations morphologiques, génitales, psychiques (syndromes dissociés). Rev Neurol 41:385–394
Delay J, Brion S, Derouesné C (1964) Syndrome de Korsakoff et étiologie tumorale. Rev Neurol 111:97–133
Gouriou, Guiraud, Scherrer (1936) Tumeur du 3º ventricule à troubles mentaux prédominants. Ann Med Psychol (Paris) 94:842–848
Claude H, Cuel J (1939–1941) Un cas de tumeur d´ Erdheim sans symptômes localisateurs. Considérations médico-légales. Encéphale 34: 391–398
Vincent C, Thiébaut F (1941) Diagnostic des craniopharyngiomes. Paris Med 40:191–200
Manceaux A, Sutter J, Susini R (1941) Craniopharyngiome - Considérations anatomiques et cliniques. Alger Med 146:80–86
Klein R (1944) Craniopharyngiome et tumeur du IIIème ventricule. Ablation des deux tumeurs. Rev Neurol 76:21
Launay C, Ribadeau-Dumas C, Maillard L, Chadoutaud L (1946) La forme cachectisante du craniopharyngiome. Arch Fr Pediatr 3:381–384
Coste F, Feld M (1947) Craniopharyngiome du 3º ventricule, ablation, guérison. Rev Neurol 79:449–452
Laine ME (1949) Exerèse d´un craniopharyngiome à développement postérieur par voie transventriculaire à la faveur de l´ouverture de l´espace perforé postérieur. Rev Neurol 81:391–395
Riese W (1950) Craniopharyngiome chez une femme agée de 57 ans. Hallucinations visuelles et auditives. Deuxième note sur la genèse des hallucinations survenant chez les malades atteints de lesions cérébrales. Rev Neurol 82:137–139
Boitelle G, Fruhling L, Boitelle-Lentulo C (1952) Syndrome de Cushing avec confuso-démence symptomatique d´un craniopharyngiome. Ann Med Psychol (Paris) 110:179–182
Dejean Ch, Cl Gros, Boudet Ch (1952) Craniopharyngiome atypique méconnu avec atrophie totale du chiasma et des nerfs optiques. Rev Otoneuroophtalmol 24:290–293
Ajuriaguerra J, Hecaen H, Sadoun R (1954) Les troubles mentaux au cours des tumeurs de la région méso-diencéphalique. Encéphale 43:406–478
Kammerer T, Ebtinger R, Philippides D, Steimle R, Israel L (1955) Épisodes psychotiques, rigidité de décérébration, alteration EEG et troubles métaboliques dans un cas de craniopharyngiome du troisième ventricule. Cah Psychiatr 10:52–81
Thiébaut F, Philippiodès D, Matavuli N, Helle J (1956) Syndrome chiasmatique par craniopharyngiome. A propos de deux cas. Rev Otoneuroophtalmol 28:104–105
Riser M, Lazhortes G, Gleizes L, Garrigues P (1956) Névrite optique d´abord rétrobulbaire et craniopharyngiome. Rev Otoneuroophtalmol 28:289–292
Decourt J, Bernard-Weil E, Michard JP (1957) Sur le diabète insipide associé à une insuffisance antéhypophysaire. Action de la cortisone sur la soif, la polyurie et l´équilibre hydroélectrolytique. Ann Endocrinol 18:880–890
Wertheimer P, Corradi M (1957) Les craniopharyngiomes après 40 ans. Étude anatomo-clinique et resultants opératoires. Neurochirurgie 3:3–21
Sivadon P, Ajurriaguerra I, Baranda R (1957) Syndrome korsakowien d´étiologie tumorale par compression du troisième ventricule. Ann Med Psychol (Paris) 115(3):484–486
Guinet P, Putelat R, Tomáis M, Descours C, Franchet G (1961) Un cas de craniopharyngiome avec galactorrhée et aménorrhée. Ann Endocrinol 22:385–389
Pertuisset B, Janny P, Allègre G, Olivier L (1962) Cranio-pharyngiomes simulant une tumeur antérieure du 3 ventricule. Presse Med 70:1846–1848
Maspes PE, Cassinari V, Bernasconi V (1962) Craniopharyngiomes à physionomie anatomo-clinique inhabituelle. Neurochirurgie 8:315–323
Rougerie J, Fardeau M (1962) Les Craniopharyngiomes. Masson et Cie éditeurs, Paris
Pecker J, Ferrand B, Javalet A (1966) Tumeurs du troisième ventricule. Neurochirurgie 12:7–136
Violon A, Brihaye J, Crahay J, Jancu H (1973) Les troubles mentaux au cours des tumeurs du troisiéme ventricule. Neurochirurgie 19:317–320
Braunstein DG (2011) The Hypothalamus. In: Melmed S (ed) The Pituitary, 3rd edn. Academic Press, Elsevier, Amsterdan, pp 303–341
Swaab D (2004) The Human Hypothalamus. Basic and clinical aspects. In: Handbook of clinical neurology, vol 79 part 1, 3rd series, Elsevier, Amsterdam, vol 1, pp 243–248
Pascual JM, Prieto R, Carrasco R, Castro-Dufourny I, Stauss S, Gil-Simoes R, Barrios L (2014) The 2013 Sixto Obrador Award. A triple-axis topographical model for surgical planning of craniopharyngiomas. Part I: historical review of the topographic diagnosis and classification schemes of craniopharyngiomas. Neurocirugia (Astur) 25:154–169
Pascual JM, Prieto R, Carrasco R, Castro-Dufourny I, Stauss S, Gil-Simoes R, Barrios L (2014) 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. Neurocirugia (Astur) 25:211–239
Cushing H (1912) The pituitary body and its disorders. JB Lippincott, Philadelphia, pp 289–291
Cushing H (1922) Les syndromes hypophysaires au point de vue chirurgical. Rev Neurol 38:779–808
Young JK, Polston EK (2013) Specialized features of the Arcuate Nucleus of the hypothalamus. In: Dudás B (ed) The human hypothalamus: anatomy, functions and disorders. Nova Science Publishers, New York, pp 31–66
Northfield DWC (1957) Rathke-Pouch tumours. Brain 80:293–312
Steno J (1985) Microsurgical topography of craniopharyngiomas. Acta Neurochir (Suppl) 35:94–100
Pascual JM, Carrasco R, Prieto R, Gonzalez-Llanos F, Alvarez F, Roda JM (2008) Craniopharyngiomas classification. J Neurosurg 109:1180–1183
Pascual JM, Prieto R, Carrasco R, Barrios L (2013) Displacement of mammillary bodies by craniopharyngiomas involving the third ventricle: surgical-MRI correlations and use in topographical diagnosis. J Neurosurg 119:381–405
Morton GJ, Meek TH, Schwartz MW (2014) Neurobiology of food intake in health and disease. Nat Rev Neurosci 15:367–378
Lusting RH (2011) Hypothalamic obesity after craniopharyngioma : mechanisms, diagnosis and treatment. Front Endocrinol 2:60
Reeves AG, Plum F (1969) Hyperphagia, rage and dementia accompanying a ventromedial hypothalamic neoplasm. Arch Neurol 20:616–624
Clarke IJ, Campbell R, Smith JT, Prevot V, Wray S (2012) Neuroendocrine control of reproduction. In: Fink G, Pfaff DW, Levine JE (eds) Handbook of neuroendocrinology. Academic Press, Elsevier Inc, London, pp 197–236
Hoffman GE (2013) The hypothalamus and sleep. In: Dudás B (ed) The human hypothalamus: Anatomy, functions and disorders. Nova Science Publishers, New York, pp 343–369
Williams M, Pennybacker J (1954) Memory disturbances in the third ventricle tumours. J Neurol Neurosurg Psychiat 17:115–123
Izci Y, Karlidere T, Caliskan U, Akay KM (2003) Diencephalic tumours presenting as psychosis. Acta Neuropsychiatrica 15:97–101
Kanh EA, Crosby EC (1972) Korsakoff´s syndrome associated with surgical lesions involving the mammillary bodies. Neurology 22:117–125
Vann SD, Aggleton JP (2004) The mammillary bodies: two memory systems in one? Nat Rev Neurosci 5:35–44
Pascual JM, Prieto R, Castro-Dufourny I, Gil-Simoes R, Carrasco R (2012) Classification systems of adult craniopharyngiomas: the need for an accurate definition of the hypothalamus—tumor relationships (Letter). Arch Med Res 43:588–590
Pascual JM, Prieto R, Castro-Dufourny I, Gil-Simoes R, Carrasco R (2013) Hypothalamus-referenced classification for craniopharyngiomas: evidence provided by the endoscopic endonasal approach. Neurosurg Rev 36:337–340
Pascual JM, Prieto R, Carrasco R, Castro-Dufourny I, Barrios L (2014) Development of intracranial approaches for craniopharingiomas: an analysis of the first 160 historical procedures. Neurosurg Focus 36(4):E13
Acknowledgments
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, and 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 George Hamilton for his critical review of the language and style of the manuscript.
Conflict of interest
The authors declare that they have no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Castro-Dufourny, I., Carrasco, R., Prieto, R. et al. The infundibulo-tuberal syndrome caused by craniopharyngiomas: clinicopathological evidence from an historical French cohort (1705–1973). Pituitary 18, 642–657 (2015). https://doi.org/10.1007/s11102-014-0623-4
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11102-014-0623-4