Endocrine Abstracts

Introduction: Craniopharyngioma (CP) is a rare epithelial tumor of the sellar and suprasellar region, histologically of low grade (WHO grade I). Significant locoregional invasion explains the frequency of recurrence, mainly due to incomplete surgical resection. We report two cases of craniopharyngioma in children, in order to outline the therapeutic difficulties encountered during management.

Case 1: Eleven years-old patient reported frontal headache with asthenia and chills, followed by vomiting. Examination BP 11/6 cmhg, HR 95 bpm, Tanner S2P3. Workup: TSH 3.3 mIU/l, fT4 10 pmol/l, fT3: 3.24 pmol/l, FSH 5.5 IU/l, LH: 2.1 IU/l, PRL 12.58 ng/ml, Cortisolaemia 12.64 ug/dl, Na+ 133, K+ 5.3. MRI: median intra- and suprasellar mass 16*19*20 mm, polymicrocystic. Fundus: no abnormalities. Patient underwent surgery, histology: craniopharyngioma. Post-operative MRI showed persistent lesional process of 16×17×12 mm, indicating incomplete surgical resection.

Case 2: Patient aged 21, followed for sellar and suprasellar lesional process of 22.4×30×37 mm. Examination BP 126/72 mmHg, HR 76 bpm, Tanner S2P2. Work-up: Cortisol 14.61 µg/dl; TSH 1.71 mIU/l; fT4 17.53 pmol/l, Prolactin 8.8 ng/ml; estradiol <5 ng/l; FSH 4.86 IU/l; LH 0.98 IU/l. Tumour resected, histology showed a craniopharyngioma. At 18 months post-op recurrence with signs of compression of the anterior quadrigeminal tubercles, visual field: OD concentric narrowing and OG temporal superior quadranopsia. Several repeat operations performed (6 times), with installation of bilateral blindness. At last checkup, intra- and suprasellar process with polylobed contours and dual cystic and fleshy components, 49×46×48 mm.

Discussion: The most common symptoms in children prior to CP diagnosis are headache (68%), visual disturbances (55%), growth retardation (36%), nausea (34%), neurological deficits (23%), polydipsia/polyuria (19%) and weight gain (16%). Hypothalamic-pituitary MRI is the gold-standard imaging modality. The type of surgical resection depends on locoregional invasion and determination of the primary origin of the prostate cancer (ventricular, suprasellar, etc.). Over two-thirds of patients treated with surgery and radiotherapy (RTH) in childhood have a satisfactory outcome. Current radiotherapy techniques include 3D fractionated radiotherapy, intensity-modulated HBRT, steriotactic HBRT and, more recently, proton therapy.

Conclusion: Le CP is a locally aggressive, low-grade tumor with high morbidity in children and adults.

Keywords: craniopharyngioma, children, surgery, invasion, recurrence.