Endocrine Abstracts

Teratomas comprise 0.5% of all intracranial tumors. 15 cases of sellar teratoma have been described in the last 24 years (yrs). A 29-yrs female pan-hypopituitaric patient (pt) was admitted to our Pituitary Unit. At 6-yrs age, for polyuria and growth delay, an endocrine evaluation revealed diabetes insipidus and hypopituitarism. Cranial contrasted-MRI (cc-MRI) showed pituitary stalk thickening and a low intensity suprasellar lesion. Histological examination (HE) was suggestive for germinoma. Conventional radiotherapy was performed. After 6 months (mts) for disease-progression, a second biopsy was performed and HE was suggestive of 3th ventricle choroid plexus papilloma. Pt underwent radical neurosurgical resection. HE was conclusive for mature teratoma. At 10-yrs age, pt weight was 38 Kg (90th percentile), height was 124 cm (3rd percentile) and growth velocity was 3 cm/yr (<3rd percentile). Clonidine test suggested an impaired GH secretion. cc-MRI didn’t documented teratoma recurrence (rec). After 3 disease-free survival yrs, rhGH replacement therapy (rhGH-RT) was started. Every 6 mts, endocrinological and neuroradiological evaluation were scheduled. At 18-yrs age, cc-MRI evidenced a falx cerebri lesion. rhGH-RT was discontinued. Pt underwent neurosurgery. HE documented an atypical meningioma. 1-yr post-surgery cc-MRI confirmed tumor radical resection. rhGH-RT was re-started. Every 6 mts, endocrinological and neuroradiological evaluation was scheduled. At 27-yrs age, cc-MRI showed meningioma rec. Pt underwent second radical neurosurgery. HE documented transitional meningioma with strong immunohistochemical positivity for GH receptor. rhGH-RT was discontinued. At admission in our department, laboratory tests confirmed panhypopituitarism, cc-MRI was negative for teratoma and meningioma rec. We confirmed the rhGH-RT discontinuation. To our knowledge, our case is the first describing rhGH-RT safety in a pt with teratoma history. Teratomas should be taken into account in the diagnosis of pituitary region tumors for allowing patients to benefit of correct treatment and later of a complete hormonal replacement therapy.