<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_raon-2019-0036_s_011_w2aab3b8c17b1b7b1aab1c16b1Aa"><h3>Background</h3><p>Childhood and adult-onset craniopharyngioma is a rare embryogenic tumor of the sellar, suprasellar, and parasellar region. Survival rates are high; however, tumor location and treatment sequalae including endocrine deficits, visual impairment, metabolic complications, cognitive and psychosocial deficits can significantly impair patient’s quality of life. There is considerable controversy regarding the optimal management of craniopharyngiomas. Subtotal resection of the tumor followed by targeted irradiation to avoid further hypothalamic damage is currently indicated. Novel insights in the tumor’s molecular pathology present the possibility for targeted therapy possibly decreasing the rate and severity of treatment-associated morbidity.</p></sec><sec id="j_raon-2019-0036_s_012_w2aab3b8c17b1b7b1aab1c16b2Aa"><h3>Conclusions</h3><p>Craniopharyngioma should be seen as a chronic disease. To achieve optimal outcomes a multidisciplinary team of specialized neurosurgeons, neuro-radiologists, neuro-oncologists, pathologists and endocrinologists should be involved in the diagnosis, planning of the surgery, irradiation and long-term follow-up.</p></sec></abstract>

BackgroundChildhood and adult-onset craniopharyngioma is a rare embryogenic tumor of the sellar, suprasellar, and parasellar region. Survival rates are high; however, tumor location and treatment sequalae including endocrine deficits, visual impairment, metabolic complications, cognitive and psychosocial deficits can significantly impair patient’s quality of life. There is considerable controversy regarding the optimal management of craniopharyngiomas. Subtotal resection of the tumor followed by targeted irradiation to avoid further hypothalamic damage is currently indicated. Novel insights in the tumor’s molecular pathology present the possibility for targeted therapy possibly decreasing the rate and severity of treatment-associated morbidity.ConclusionsCraniopharyngioma should be seen as a chronic disease. To achieve optimal outcomes a multidisciplinary team of specialized neurosurgeons, neuro-radiologists, neuro-oncologists, pathologists and endocrinologists should be involved in the diagnosis, planning of the surgery, irradiation and long-term follow-up.

Advances in the management of craniopharyngioma in children and adults

Radiology and Oncology

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

{"article-title":"Advances in the management of craniopharyngioma in children and adults"}

BackgroundChildhood and adult-onset craniopharyngioma is a rare embryogenic tumor of the sellar, suprasellar, and parasellar region. Survival rates are high;...

	Pediatric-onset	Adult-onset
	30–50 % of all CPs
Age at presentation	Peak at 5–14 years¹	Peak at 40–44 years²

Gender distribution (m/f)	Equal^8,21	Equal^8,27

Most frequent presentation	Headache (68–85%)	Visual impairment (40–84%)
	Visual impairment (36– 55%)	Menstrual irregularities (57%)
	Growth failure (7–36%)^9,35,66	Headache (42–56%)^13,26,27

Pathohistological type	Adamantinomatous 99%	³³
Pathohistological type	Papillary extremely rare*	Papillary 14–50%

Initial hypothalamic involvement	42–66%^8,9,35	42%¹⁸

Endocrine deficits at diagnosis

Any	40–87%^{8,13,21,35,65,66}	41–73%⁸
GH	41–75%^{8,13,21,35,65,66}	18–86%^8,13
FSH/LH	20–56%^{8,13,21,35,65,66}	29–74%^8,13
ACTH	8–68%^{8,13,21,35,65,66}	35–58%^8,13
TSH	15–32%^{8,13,21,35,65,66}	35–56%^8,13
ADH	7–17%^{8,13,21,35,65,66}	6–17%^8,13

Pituitary hormone deficiencies after treatment

Any	64–100%^8,64	48–97%^8,64
GH	93–96%^8,18,21	52–68%^8,18
FSH/LH	59–95%^8,18,21	70–94%^8,18
ACTH	78–100%^8,18,21	74–88%^8,18
TSH	86–100%^8,18,21	81–92%^8,18
ADH	65–96%^8,18,21	43–70%^8,18
Panhypopituitarism***	43–100%^8,18,64	59–74%^8,18,64

Obesity**	44–64%^8,9,19,64	41–47%^8,19,64

Advances in the management of craniopharyngioma in children and adults

Article Category: Review

Published Online: Oct 25, 2019

Page range: 388 - 396

Received: Jun 21, 2019

Accepted: Jul 11, 2019

DOI: https://doi.org/10.2478/raon-2019-0036

© 2019 Mojca Jensterle, Soncka Jazbinsek, Roman Bosnjak, Mara Popovic, Lorna Zadravec Zaletel, Tina Vipotnik Vesnaver, Barbara Faganel Kotnik, Primoz Kotnik, published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Figure 1

Comparison of pediatric and adult-onset craniopharyngioma characteristics