Endocrine Abstracts

Aggressive pituitary tumors (APT) causing Cushing’s Disease are very rare, difficult to treat. The majority of Crooke’s corticotropinomas are macroadenomas, exhibiting rapid growth, resistance to conventional treatments, a high recurrence rate. To date, there is no fully effective method of treatment for these tumors. Pituitary carcinomas (PC) are defined by distant metastasis. PC is exceedingly rare, comprising only 0.1–0.2% of all pituitary neoplasms but APT may account for up to 15% of all pituitary neoplasms. We report the case of a young female patient with Cushing’s disease (macroadenoma, right cavernous sinus invading, nerve VI palsy) who underwent three transsphenoidal surgeries and two γ-knife radiosurgery (GKR). Pathological findings: ‘hyalin Crooke’ cell type; ACTH positive immunohistochemistry and a very high Ki67 labeling index. She achieved hypocortisolism and healed the palsy after the 1^st surgery but early tumor recurrence occurred thereafter. One year and a half after first GKR she returned with rapidly progressive recurrence: headache, right orbital pain, palpebral ptosis and diplopia; hypercortisolism and tumoral rest progression (MRI showed tumoral rest progression 41/31/36 mm). Ophtalmologic exam: nerve III palsy. She underwent urgent endonasal endoscopic surgery for cavernous sinus decompression and short time after, double approach: transsphenoidal and pterional craniotomy was done. The resection was limited by fibrous tumor that invade the cavernous sinus and cause internal carotid artery compression. Histopathological analysis did not show substantial change compared with the first examination. Hypercortisolism persisted and Pasireotide treatment and CBG was started and also she underwent the second GKR. For 6 months she had hormonal response under Pasireotide treatment but fasting blood glucose and Hba1c increased during therapy. And, a new lession with extension in left nasopharynx rapidly increased (40/20 mm). Aggressive corticotroph tumors is a ‘high risk pituitary adenomas’ and should be followed carefully for early recurrence and clinical aggressive behavior and expand treatment intensity; these patients often require medical therapy to reduce hypercortisolism.