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Craniopharyngioma Classification

An appropriate classification system with which to individualize treatment is absent. The objectives of this study were to identify preoperative prognostic factors in patients with craniopharyngiomas and to develop a risk-based treatment algorithm.

The authors reviewed data obtained in a retrospective cohort of 66 children (mean age 7.4 years, mean follow-up period 7 years) who underwent resection between 1984 and 2001. Postoperative recurrence rates, vision status, and endocrine function were consistent with those reported in the literature. The postoperative morbidity was related to hypothalamic dysfunction. The preoperative magnetic resonance imaging grade, clinically assessed hypothalamic function, and the sugeon's operative experience (p = 0.007, p = 0.047, p = 0.035, respectively) significantly predicted poor outcome. Preoperative hypothalamic grading was used in a prospective cohort of 22 children (mean age 8 years, mean follow-up period 1.2 years) treated between 2002 and 2004 to stratify patients according to whether they underwent gross-total resection (GTR) (20%), complete resection avoiding the hypothalamus (40%), or subtotal resection (STR) (40%). In cases in which residual disease was present, the patient underwent radiotherapy. There have been no new cases of postoperative hyperphagia, morbid obesity, or behavioral dysfunction in this prospective cohort.

For many children with craniopharyngiomas, the cost of resection is hypothalamic dysfunction and a poor QOL. By using a preoperative classification system to grade hypothalamic involvement and stratify treatment, the authors were able to minimize devastating morbidity. This was achieved by identifying subgroups in which complete resection or STR, performed by an experienced craniopharyngioma surgeon and with postoperative radiotherapy when necessary, yielded better overall results than the traditional GTR 1).

To date, however, the Puget system has not been externally validated.

A panel of 6 experts, consisting of pediatric neurosurgeons and pediatric neuroradiologists, graded 30 preoperative and postoperative MRI scans according to the Puget system. Interrater reliability was calculated using Fleiss' κ and Krippendorff's α statistics.

Interrater reliability in the preoperative context demonstrated moderate agreement (κ = 0.50, α = 0.51). Interrater reliability in the postoperative context was 0.27 for both methods of statistical evaluation.

Interrater reliability for the system as defined is moderate. Slight refinements of the Puget MRI grading system, such as collapsing the 3 grades into 2, may improve its reliability, making the system more generalizable 2)

Adamantinomatous craniopharyngioma, the most frequent histological variety in children.

Papillary craniopharyngioma.

see Anaplastic Craniopharyngioma.


Most craniopharyngiomas can be classified as either “prechiasmatic” or “retrochiasmatic” according to their growth patterns.

Retrochiasmatic craniopharyngioma

Subdiaphragmatic craniopharyngioma

Supradiaphragmatic craniopharyngioma

Intraventricular craniopharyngioma


Cystic craniopharyngioma

Puget S, Garnett M, Wray A, Grill J, Habrand JL, Bodaert N, Zerah M, Bezerra M, Renier D, Pierre-Kahn A, Sainte-Rose C. Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J Neurosurg. 2007 Jan;106(1 Suppl):3-12. Review. PubMed PMID: 17233305.
Whelan R, Prince E, Mirsky DM, Naftel R, Bhatia A, Pettorini B, Avula S, Staulcup S, Alexander AL, Meier M, Hankinson TC. Interrater reliability of a method to assess hypothalamic involvement in pediatric adamantinomatous craniopharyngioma. J Neurosurg Pediatr. 2019 Oct 11:1-6. doi: 10.3171/2019.8.PEDS19295. [Epub ahead of print] PubMed PMID: 31604324.
craniopharyngioma_classification.txt · Last modified: 2019/10/12 14:08 by administrador