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Review
. 2020;110(9-10):767-779.
doi: 10.1159/000509616. Epub 2020 Jun 24.

Diagnosis, Background, and Treatment of Hypothalamic Damage in Craniopharyngioma

Affiliations
Review

Diagnosis, Background, and Treatment of Hypothalamic Damage in Craniopharyngioma

Eva-Marie Erfurth. Neuroendocrinology. 2020.

Abstract

Craniopharyngiomas (CP) are rare brain tumors managed primarily with surgery and radiotherapy. There are 2 phenotypes of CP, i.e., one with a rather good outcome without hypothalamic damage and another with hypothalamic damage. With hypothalamic damage, progressive disease with recurrent operations and additional cranial radiotherapy often result in hypothalamic obesity, an affected psychosocial life, and cognitive dysfunction. The morbidity and mortality are increased for particularly cerebrovascular diseases. Preoperative hypothalamic involvement to predict hypothalamic damage is important for decision making for hypothalamus-sparing surgery. Also a postoperative hypothalamic damage evaluation with the use of hypothalamus volume measurement can predict hypothalamic obesity, which is important for early treatment options. The morbidity of CP includes cognitive dysfunction with attention deficits and impaired episodic memory and processing speed. Again patients with hypothalamic damage are more affected. Treatment options of hypothalamic obesity in the chronic phase are scarce and not convincingly successful. The most optimal situation is to try to hinder or stop the evolution of hypothalamic obesity. Prevention of hypothalamic damage is recommended, with special regard to hypothalamus-sparing therapeutic approaches that respect the integrity of essential nuclei located in both the medial and the posterior hypothalamic areas.

Keywords: Craniopharyngioma; Hypothalamic obesity; Hypothalamus; Neurosurgery.

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Conflict of interest statement

The authors have no conflict of interests to declare.

Figures

Fig. 1
Fig. 1
Delineation of the hypothalamic region in 3-T MRI. Representative overview of the delineation principles of the HT in T1-weighted MRI acquired at 3 T according to Gabery et al. [22]. The anatomical landmarks were based on histologically processed postmortem hypothalamic tissue derived from patients with Huntington disease [22]. Between 14 and 16 images were used for per case analysis. Images were preprocessed with cubic spine interpolation of the original voxel size to 0.5 × 0.5 × 0.5 mm. The images were taken from slices 1, 4, 8, 12, and 16 with 2-mm spacing. a, d, g, j, m In a rostral-to-caudal direction, the images are representative 3 T MR images on a coronal plane. b, e, h, k, n Schematic overview of the corresponding level modified from Tang-Christensen et al. [41]. c, f, i, l, o Overlap of the schematic overview on the MR image. The light blue lines illustrate how the HT region was delineated. Landmarks such as the HT sulcus (represented by a white asterisk) and the lateral edge of the optical tract (represented by a red asterisk) were identified for delineation and a straight line between these 2 points was drawn to set the superior/lateral border of the area in a reproducible fashion. The optical tract was excluded in all slides. d, dorsomedial hypothalamic nucleus; F, fornix; i, infundibular nucleus; l, lateral HT; MB, mammillary body; OT, optical tract; P, paraventricular nucleus; s, supra optic nucleus; v, ventromedial hypothalamic nucleus. Reproduced from Gabery et al. [22] with permission from Springer Nature.
Fig. 2
Fig. 2
a Correlation between leptin (ng/mL) and hypothalamic volume (mm3) among 35 CP patients and 31 controls (n = 35, rs = −0.53; p = 0.001). b Correlation between fat mass (kg) and hypothalamic volume (mm3) among 35 CP patients and 31 controls (n = 35, rs = −0.67; p < 0.001). TGTV, tumor growth into the third ventricle. Reproduced from Fjalldal et al. [21] with permission from Springer Nature.
Fig. 3
Fig. 3
Schematic overview of the arcuate nucleus, the ventromedial hypothalamus, and the lateral hypothalamus with its main nucleus and interrelations. The arcuate nucleus has 2 sets of neurons – one generates the orexigenic AgRP and NPY and the other generates the anorexigenic POMC and CART. POMC is a precursor of α-MSH, whose main effect on weight regulation is through MC4R.
Fig. 4
Fig. 4
Linear association between total body weight (kg) and BMR (kcal/day) among patients and controls. Patients had a significantly lower BMR compared to controls after adjustment for sex and total body weight in the linear regression analysis (mean difference: −90 kcal/24 h; 95% CI −160 to −10; p = 0.02). Reproduced from Holmer et al. [31] with permission from Oxford University Press.
Fig. 5
Fig. 5
Graphical renderings of directionally color-coded (red = right-left, green = anterior-posterior, and blue = superior-inferior) segmented tractographys of dorsal cingulum (a), ventral cingulum (b), fornix (c), and uncinate fasciculus (d) superimposed on a mid-sagittal FA map in a representative subject. Reproduced from Fjalldal et al. [81] with permission from Bio Scientifica.
Fig. 6
Fig. 6
Episodic visual memory (Rey complex figure test) with immediate recall (a) and delayed recall (b) in association with DTI in the left ventral cingulum (MD; mean diffusivity) among 32 CP patients. Reproduced from Fjalldal et al. [81] with permission from Bio Scientifica.

References

    1. Müller HL, Merchant TE, Warmuth-Metz M, Martinez-Barbera JP, Puget S. Craniopharyngioma. Nat Rev Dis Primers. 2019 Nov;5((1)):75. - PubMed
    1. Müller HL, Merchant TE, Puget S, Martinez-Barbera JP. New outlook on the diagnosis, treatment and follow-up of childhood-onset craniopharyngioma. Nat Rev Endocrinol. 2017 May;13((5)):299–312. - PubMed
    1. Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM. The descriptive epidemiology of craniopharyngioma. J Neurosurg. 1998 Oct;89((4)):547–51. - PubMed
    1. Olsson DS, Andersson E, Bryngelsson IL, Nilsson AG, Johannsson G. Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood onset: a population-based study in Sweden. J Clin Endocrinol Metab. 2015 Feb;100((2)):467–74. - PubMed
    1. Apps JR, Carreno G, Gonzalez-Meljem JM, Haston S, Guiho R, Cooper JE, et al. Tumour compartment transcriptomics demonstrates the activation of inflammatory and odontogenic programmes in human adamantinomatous craniopharyngioma and identifies the MAPK/ERK pathway as a novel therapeutic target. Acta Neuropathol. 2018 May;135((5)):757–77. - PMC - PubMed

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