Pediatric Craniopharyngioma  

Azzoug S. , Belhadj Aissa N. , Chentli F.
Endocrine and Metabolic diseases Department Bab El Oued Hospital Algiers, Algeria
Author    Correspondence author
International Journal of Clinical Case Reports, 2015, Vol. 5, No. 48   doi: 10.5376/ijccr.2015.05.0048
Received: 20 Jul., 2015    Accepted: 22 Aug., 2015    Published: 08 Dec., 2015
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Azzoug S., Belhadj Aissa N., Chentli F., 2015, Pediatric Craniopharyngioma, International Journal of Clinical Case Reports, 5(48) 1-2 (doi: 10.5376/ijccr.2015.05.0048)

Abstract

Craniopharyngioma are epithelial tumors of the sellar and suprasellar region derived from Rathke cleft. They are among the most common forms of cerebral tumors in children. Although they are histologically benign they may have severe complications due to their proximity to vital structures. Furthermore they often recur after surgical resection. The aim of our study was to analyze the clinical profile, radiological aspect and complications of pediatric craniopharyngioma. The medical records of 47 patients (28M/19F) harboring a craniopharyngioma were reviewed. Mean age at diagnosis was 11.2 ± 5.4 years. Mean size of the tumor was 37 ± 10.7 mm. Appealing symptoms were ophthalmological complaints in 34.6%, neurological signs mainly headaches in 28.2%, intracranial hypertension in 28.2%, endocrine symptoms in 9%. Endocrine repercussions were anterior pituitary deficiency in 72.4% (global in 32% and partial in 40.4%) and diabetes insipidus in 46.8%. 68% of patients have visual acuity impairment among which 21.2% were blind (12.7% bilateral and 8.5% unilateral blindness). 12.7% of patients have ocular motor palsy. All patients were operated on sometimes more than once; radiotherapy was used in 20.5%. Craniopharyngioma are rare tumors which may have severe endocrine, neurological and visual repercussions; it often needs iterative surgery sometimes combined with radiotherapy.

Keywords
Pediatric craniopharyngioma; Visual impairment; Endocrine dysfunction

Introduction
Pediatric craniopharyngioma are rare non glial intracranial tumors of the suprasellar and sellar area deriving from a malformation of embryonic tissue. Although craniopharyngioma are histologically benign, they may have a dire prognosis due to their ophthalmological, endocrine and neurological repercussions and their high rate of recurrence. The aim of our study is to analyze the clinical profile, radiological aspect and complications of pediatric craniopharyngioma in our institution.
 
Material and Methods
This is a retrospective study concerning 47 children (28M/19F) harboring a craniopharyngioma.

Their mean age was of 11.2 ± 5.4 years. Medical records of the patients were reviewed in order to analyze their presenting symptoms, clinical and radiological profile, ophthalmological and endocrine repercussions and therapeutic modalities of these tumors.

Results
The presenting symptoms were ophthalmological complaints in 34.6%, neurological signs mainly headaches in 28.2%, intracranial hypertension in 28.2%, endocrine symptoms (growth retardation, obesity, diabetes insipidus) in 9%.
Endocrine repercussions were anterior pituitary deficiency in 72.4% (global in 32% and partial in 40.4%) and diabetes insipidus in 46.8%.

68% of patients have visual acuity impairment among which 21.2% were blind (12.7% bilateral and 8.5% unilateral blindness). 12.7% of patients have ocular motor palsy.

The mean tumoral diameter was of 37 ± 10.7 mm. Craniopharyngioma were mainly cystic with most of tumors presenting the association of cystic, solid portions and calcifications.

Treatment
All patients were operated on. 70% of the patients underwent partial resection and 30% total resection. Recurrences were noted in 44% requiring surgical reoperation. After surgery, visual improvement was observed in 16%, diabetes insipidus appeared in 25% post surgery and anterior pituitary deficits worsen in 8%. Conventional radiotherapy was used in 8.5% in complement to partial surgical resection. Patients with a predominant cystic portion underwent an aspiration of the fluid with instillation of sclerosing substances in six patients (Yttrium in five patients and Bleomycine in one patient). Evolution was characterized by tumour shrinkage in 50%, tumour stabilization in 33% and tumour progression in 17%.

 
Figure 1

 
Discussion
Craniopharyngioma represent 5-10% of all childhood intracranial tumors (Liubinas., 2011). They derived either from the remnants of the incompletely waned Rathke’s pouch or a metaplasia of the residual squamous epithelial rests (Rushing., 2007). There are two subtypes of craniopharyngioma, the adamantinomatous subtype which predominates in children and derive from neoplastic transformation of epithelial remnants of the craniopharyngeal duct and the papillary subtype, which predominates in adults and result from metaplasia of adenohypophyseal cells. Adamantinomatous craniopharyngioma is characterized by keratinized squamous epithelium with calcifications and cystic degeneration. Papillary subtype is characterized by stratified squamous epithelium usually without calcifications or cystic degeneration (Pettorini., 2010). 30-50% of craniopharyngioma manifest during childhood and adolescence. Initial symptoms of craniopharyngioma often occur many years before diagnosis is made. The difficulty to recognize decreased visual acuity in children may account for the frequency of visual impairment in our patients and as reported by others (Müller., 2011).

Craniopharyngioma frequently involve impairment of the hypothalamic pituitary axis with anterior pituitary deficits and/or diabetes insipidus (Müller., 2010). The association of solid, cystic and calcified tumors components is an important radiological clue to diagnosis of craniopharyngioma.

The close proximity of craniopharyngioma to vital structures makes their complete resection very challenging to the neurosurgeons with a risk of complications following surgery. Partial resection followed by radiotherapy seems more favorable (Müller., 2010).

Recurrences are frequent in pediatric craniopharyngioma as we observed in our series. Treatment options for recurrence include repeat surgery, radiotherapy or instillation of sclerosing agents for predominantly cystic tumors.
 
References
Liubinas S.V., Munshey A.S., and Kaye A.H., 2011, Management of recurrent craniopharyngioma, J. Clin. Neurosci., 18(4): 451-457
http://dx.doi.org/10.1016/j.jocn.2010.10.004

Müller H.L., 2011, Diagnostics treatment and follow-up in craniopharyngioma, Front Endocrinol (Lausanne), 2: 70
http://dx.doi.org/10.3389/fendo.2011.00070

Müller H.L., 2010, Childhood craniopharyngiom a current concepts in diagnosis, therapy and follow-up,  Nat. Rev. Endocrinol., 6: 609-618
http://dx.doi.org/10.1038/nrendo.2010.168

Müller H.L., Ebhardt U., Schröder S., Pohl F., Kortmann RD., Faldum A., et al., 2010, Analyses of treatment variables for patients with childhood craniopharyngioma results of the multicenter prospective study trial KRANIOPHARYNGEOM 2000 after three years of follow up, Horm. Res. Paediatr., 73: 175-180
http://dx.doi.org/10.1159/000284358

Pettorini B.L., Frassanito P., Caldarelli M., Tamburrini G., Massimi L., and Di Rocco C., 2010, Molecular pathogenesis of craniopharyngioma: switching from a surgical approach to a biological one, Neurosurg Focus, 28: E1
http://dx.doi.org/10.3171/2010.1.FOCUS09300

Rushing E., Giangaspero F., Paulus W., et al., Craniopharyngioma, In: Louis D., Ohgaki H., Wiestler O., Cavenee WK., editors, 2007, WHO classification of tumours of the central nervous system Geneva: WHO Press, pp.238-40

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