Introduction: Chordomas are rare tumours that usually occur in adults, in children since 1923, <100 cases has been reported. It represent few than 5% of these tumours and most frequently develop in the skull base.
Para cellar localisation is uncommon, cause hypopituitarism and oculomotor nerves palsy.
Chordomas are believed to behave more aggressively than chordomas in adults.
Case report: A 14-year-old boy presented in December 2006 with polyuropolydipsia syndrome and headache.
1-On examination: he had
Short stature (<−2S.D).
Pubertal stage G1P1 of Tanner.
Left oculomotor nerves palsy.
Ophthalmologic evaluation: normal visual field.
2-Serum hormonal investigation: found hypopiytuitarism and insipidus diabetes.
3-MR imaging demonstrated a mass developing in the clivial region invades the cavernous sinus, the para sellar and the temporal regions. A trans temporal surgery removed apart of the tumour.
4-Pathological findings: physalliphorus cells arranged in lobules and embedded in a mucoid stroma
5-Post operative course was uneventful, two months later the tumour had growth explosively, became exophytic behind the temporal region, and the patient died.
Conclusion: Chordomas is uncommon tumours in child in the case report the insipidus diabetes have revealed them. The para sellar localisation explains the hypopituitarism. The explosively evolution may be explained by a sarcomatous transformation or the expression of growth factors: TGF alpha, BFGF and the strong fibronectine.
03 - 07 May 2008
European Society of Endocrinology