Background: Craneopharygioma is a rare solid or mixed tumor, that arise from remnants of Rathkes pounch. Usually they are in the suprasellar region and very few of them arise from the sella. Bimodal (514 years old and 5075) and slow growth are typical characteristics as well.
Clinical case: A 20 years old boy was refered to endocrine clinic by hypothyroidism, hypotension, and sexual dysfunction. Central hypothyroidism, hypogonadotropic hypogonadism and secundary adrenal insufficiency due to sellar mass was diagnosed. Sellar and suprasellar mixed mass with solid and cystic changes were found and due to its stability for 5 years and because of the clinical presentation was interpreted as a macroadenoma. Sustitutive hormone treatment is prescribed and regular clinical and radiologic follow up is made. For 5 years the mass experimented no changes but in the next evaluation mass enlargament with significant suprasellar extension was seen. Pterional craneotomy are made and a cranepharygioma is diagnosed. Treatment is completed with stereotactic radiotherapy and the patient has a good response so far.
Discussion: Usually calcification in the suprasellar region is seen up to 80% and cyst are present very often (up to 75%). If calcification is not seen, suspection is more difficult. Bimodal age distribution, typical diabetes insipidus (if pituitary stalk is envolved) visual symtomps and sexual dysfuction are typical clinical presentation in craneopharygioma. Diagnosis is confirmed by pathologic report. In our patient stability on time on MRI, his age and no visual disturbance made us to manage as a macroadenoma with panhypopituitarism. We insited on our diagnosis for years of follow-up with clinical and radiologic stability. We decided to surgery because of quick growth and our misdiagnosis was seen. Craneopharygioma must be distinguished from others sellar mass, but: When must we send to surgery a non typical mass thinking about craneopharyingioma?
27 Apr - 01 May 2013
European Society of Endocrinology