Endocrine Abstracts

Introduction

Craniopharyngioma is a rare type of benign brain neoplasm, arising from the pituitary stalk or gland and found most commonly in children. The clinical presentation is variable and may include endocrine or ophtalmogical disorders, intracranial hypertension syndrome and other neurological symptoms.

Observation

We report the case of a 16 year-old girl with a normal staturo-ponderal development and no medical history, who consulted for a nine months secondary amenorrhea associated to a polyuric polydipsic syndrome ( volume of urines > 5L/day and urine density 1005 ), with no other symptoms . The biology showed an hypotonic urine and an hypogonadotropic hypogonadism ; low gonadotropin levels ( FSH at 3.7 mU/ml, LH at 1.51 mU/ml ) associated to oestradiol deficiency at 12.51 pg/ml . We completed the evaluation of the hypophyseal function and found a corticotropic and a thyrotropic insufficiency . In front of this hypopituitarism, a pituitary MRI was performed, revealing an heterogeneous tumor in the sella and supra sellar space, measuring 18 × 16 × 12 mm in diameter, with disappearance of the posterior pituitary signal. Taking in consideration the young age of the patient, the diagnosis of a pituitary macroadenoma is unlikely, and the cerebral CT-scan showed multiple calcifications in the periphery of the pituitary mass in favor of the diagnosis of craniopharyngioma . The clinical presentation of the tumor was in this case the association of secondary amenorrhea to a diabetes insipidus. The procedure to follow was to put the patient under hydrocortisone, levothyroxine and desmopressin treatement and preparing her for an endonasal transsphenoidal resection of the pituitary tumor .

Conclusion

Even though it is a benign tumor, craniopharyngioma is a serious pathology with severe repercussions if not early diagnosed and treated, and post surgical surveillance is a must, to detect any recurrence which is frequent .